-
1
-
-
0031917466
-
Toward understanding the assembly and structure of KATP channels
-
L. Aguilar-Bryan, J.P. Clement J.P.t., G. Gonzalez, K. Kunjilwar, A. Babenko and J. Bryan (1998) Toward understanding the assembly and structure of KATP channels. Physiol. Rev. 78(1), 227-245.
-
(1998)
Physiol. Rev.
, vol.78
, Issue.1
, pp. 227-245
-
-
Aguilar-Bryan, L.1
Clement, J.P.2
Gonzalez, G.3
Kunjilwar, K.4
Babenko, A.5
Bryan, J.6
-
2
-
-
0034635458
-
Cystic fibrosis transmembrane conductance regulator. Structure and function of an epithelial chloride channel
-
M.H. Akabas (2000) Cystic fibrosis transmembrane conductance regulator. Structure and function of an epithelial chloride channel. J. Biol. Chem. 275(6), 3729-3732.
-
(2000)
J. Biol. Chem.
, vol.275
, Issue.6
, pp. 3729-3732
-
-
Akabas, M.H.1
-
3
-
-
0028264188
-
Amino acid residues lining the chloride channel of the cystic fibrosis transmembrane conductance regulator
-
M.H. Akabas, C. Kaufmann, T.A. Cook and P. Archdeacon (1994) Amino acid residues lining the chloride channel of the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 269(21), 14865-14868.
-
(1994)
J. Biol. Chem.
, vol.269
, Issue.21
, pp. 14865-14868
-
-
Akabas, M.H.1
Kaufmann, C.2
Cook, T.A.3
Archdeacon, P.4
-
4
-
-
0032503962
-
Regulation of CFTR ion channel gating by MgATP
-
A.A. Aleksandrov and J.R. Riordan (1998) Regulation of CFTR ion channel gating by MgATP. FEBS Lett. 431(1), 97-101.
-
(1998)
FEBS Lett.
, vol.431
, Issue.1
, pp. 97-101
-
-
Aleksandrov, A.A.1
Riordan, J.R.2
-
5
-
-
0033759953
-
The non-hydrolytic pathway of cystic fibrosis transmembrane conductance regulator ion channel gating
-
A.A. Aleksandrov, X. Chang, L. Aleksandrov and J.R. Riordan (2000) The non-hydrolytic pathway of cystic fibrosis transmembrane conductance regulator ion channel gating. J. Physiol. 528(Pt 2), 259-265.
-
(2000)
J. Physiol.
, vol.528
, pp. 259-265
-
-
Aleksandrov, A.A.1
Chang, X.2
Aleksandrov, L.3
Riordan, J.R.4
-
6
-
-
0035918147
-
Differential interactions of nucleotides at the two nucleotide binding domains of the cystic fibrosis transmembrane conductance regulator
-
L. Aleksandrov, A. Mengos, X. Chang, A. Aleksandrov and J.R. Riordan (2001) Differential interactions of nucleotides at the two nucleotide binding domains of the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 276(16), 12918-12923.
-
(2001)
J. Biol. Chem.
, vol.276
, Issue.16
, pp. 12918-12923
-
-
Aleksandrov, L.1
Mengos, A.2
Chang, X.3
Aleksandrov, A.4
Riordan, J.R.5
-
7
-
-
0037013262
-
The first nucleotide binding domain of CFTR is a site of stable nucleotide binding domain of CFTR is a site of stable nucleotide interaction whereas the second is a site of rapid turnover
-
L. Aleksandrov, A.A. Aleksandrov, X.-B. Chang and J.R. Riordan (2002) The first nucleotide binding domain of CFTR is a site of stable nucleotide binding domain of CFTR is a site of stable nucleotide interaction whereas the second is a site of rapid turnover. J. Biol. Chem. 3 15419-15425.
-
(2002)
J. Biol. Chem.
, vol.3
, pp. 15419-15425
-
-
Aleksandrov, L.1
Aleksandrov, A.A.2
Chang, X.-B.3
Riordan, J.R.4
-
8
-
-
0025931429
-
Nucleoside triphosphates are required to open the CFTR chloride channel
-
M.P. Anderson, H.A. Berger, D.P. Rich, R.J. Gregory, A.E. Smith and M.J. Welsh (1991) Nucleoside triphosphates are required to open the CFTR chloride channel. Cell 67 775-784.
-
(1991)
Cell
, vol.67
, pp. 775-784
-
-
Anderson, M.P.1
Berger, H.A.2
Rich, D.P.3
Gregory, R.J.4
Smith, A.E.5
Welsh, M.J.6
-
9
-
-
0025868103
-
Demonstration that CFTR is a chloride channel by alteration of its anion selectivity
-
M.P. Anderson, R.J. Gregory, S. Thompson, D.W. Souza, S. Paul, R.C. Mulligan, A.E. Smith and M.J. Welsh (1991) Demonstration that CFTR is a chloride channel by alteration of its anion selectivity. Science 253 202-205.
-
(1991)
Science
, vol.253
, pp. 202-205
-
-
Anderson, M.P.1
Gregory, R.J.2
Thompson, S.3
Souza, D.W.4
Paul, S.5
Mulligan, R.C.6
Smith, A.E.7
Welsh, M.J.8
-
10
-
-
0025863209
-
Generation of cAMP-activated chloride currents by expression of CFTR
-
M.P. Anderson, D.P. Rich, R.J. Gregory, A.E. Smith and M.J. Welsh (1991) Generation of cAMP-activated chloride currents by expression of CFTR. Science 251 679-682.
-
(1991)
Science
, vol.251
, pp. 679-682
-
-
Anderson, M.P.1
Rich, D.P.2
Gregory, R.J.3
Smith, A.E.4
Welsh, M.J.5
-
11
-
-
0024290913
-
Altered plasma membrane ultrastructure in multidrug-resistant cells
-
A.L. Arsenault, V. Ling and N. Kartner (1988) Altered plasma membrane ultrastructure in multidrug-resistant cells. Biochim. Biophys. Acta. 938(2), 315-321.
-
(1988)
Biochim. Biophys. Acta.
, vol.938
, Issue.2
, pp. 315-321
-
-
Arsenault, A.L.1
Ling, V.2
Kartner, N.3
-
12
-
-
0027366132
-
Synergistic activation of non-rectifying small-conductance chloride channels by forskolin and phorbol esters in cell-attached patches of the human colon carcinoma cell line HT-29cl.19A
-
R.B. Bajnath, J.A. Groot, H.R. De Jonge, M. Kansen and J. Bijman (1993) Synergistic activation of non-rectifying small-conductance chloride channels by forskolin and phorbol esters in cell-attached patches of the human colon carcinoma cell line HT-29cl.19A. Pflugers Arch. 425(1–2), 100-108.
-
(1993)
Pflugers Arch.
, vol.425
, Issue.1-2
, pp. 100-108
-
-
Bajnath, R.B.1
Groot, J.A.2
De Jonge, H.R.3
Kansen, M.4
Bijman, J.5
-
13
-
-
0034328820
-
Traffic pattern of cystic fibrosis transmembrane regulator through the early exocytic pathway
-
S.I. Bannykh, G.I. Bannykh, K.N. Fish, B.D. Moyer, J.R. Riordan and W.E. Balch (2000) Traffic pattern of cystic fibrosis transmembrane regulator through the early exocytic pathway. Traffic 1(11), 852-870.
-
(2000)
Traffic
, vol.1
, Issue.11
, pp. 852-870
-
-
Bannykh, S.I.1
Bannykh, G.I.2
Fish, K.N.3
Moyer, B.D.4
Riordan, J.R.5
Balch, W.E.6
-
14
-
-
0028174962
-
− channel gating to an ATP hydrolysis cycle
-
T. Baukrowitz, T.C. Hwang, A.C. Nairn and D.C. Gadsby (1994) Coupling of CFTR Cl− channel gating to an ATP hydrolysis cycle. Neuron 12(3), 473-482.
-
(1994)
Neuron
, vol.12
, Issue.3
, pp. 473-482
-
-
Baukrowitz, T.1
Hwang, T.C.2
Nairn, A.C.3
Gadsby, D.C.4
-
15
-
-
0026506969
-
cAMP-activated chloride conductance in the colonic cell line, Caco-2
-
C.E. Bear and E.F. Reyes (1992) cAMP-activated chloride conductance in the colonic cell line, Caco-2. Am. J. Physiol. 262(1 Pt 1), C251-C256.
-
(1992)
Am. J. Physiol.
, vol.262
, Issue.1
, pp. C251-C256
-
-
Bear, C.E.1
Reyes, E.F.2
-
16
-
-
0026054703
-
− channel activity in Xenopus oocytes expressing the cystic fibrosis gene
-
C.E. Bear, F. Duguay, A.L. Naismith, N. Kartner, J.W. Hanrahan and J.R. Riordan (1991) Cl− channel activity in Xenopus oocytes expressing the cystic fibrosis gene. J. Biol. Chem. 266(29), 19142-19145.
-
(1991)
J. Biol. Chem.
, vol.266
, Issue.29
, pp. 19142-19145
-
-
Bear, C.E.1
Duguay, F.2
Naismith, A.L.3
Kartner, N.4
Hanrahan, J.W.5
Riordan, J.R.6
-
17
-
-
0026532895
-
Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR)
-
C.E. Bear, C. Li, N. Kartner, R.J. Bridges, T.J. Jensen, M. Ramjeesingh and J.R. Riordan (1992) Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR). Cell 68 809-818.
-
(1992)
Cell
, vol.68
, pp. 809-818
-
-
Bear, C.E.1
Li, C.2
Kartner, N.3
Bridges, R.J.4
Jensen, T.J.5
Ramjeesingh, M.6
Riordan, J.R.7
-
18
-
-
0028577602
-
Phosphatase inhibitors activate normal and defective CFTR chloride channels
-
F. Becq, T.J. Jensen, X.B. Chang, A. Savoia, J.M. Rommens, L.C. Tsui, M. Buchwald, J.R. Riordan and J.W. Hanrahan (1994) Phosphatase inhibitors activate normal and defective CFTR chloride channels. Proc. Natl Acad. Sci. USA 91(19), 9160-9164.
-
(1994)
Proc. Natl Acad. Sci. USA
, vol.91
, Issue.19
, pp. 9160-9164
-
-
Becq, F.1
Jensen, T.J.2
Chang, X.B.3
Savoia, A.4
Rommens, J.M.5
Tsui, L.C.6
Buchwald, M.7
Riordan, J.R.8
Hanrahan, J.W.9
-
19
-
-
0034703056
-
Differences between cystic fibrosis transmembrane conductance regulator and HisP in the interaction with the adenine ring of ATP
-
A.L. Berger and M.J. Welsh (2000) Differences between cystic fibrosis transmembrane conductance regulator and HisP in the interaction with the adenine ring of ATP. J. Biol. Chem. 275(38), 29407-29412.
-
(2000)
J. Biol. Chem.
, vol.275
, Issue.38
, pp. 29407-29412
-
-
Berger, A.L.1
Welsh, M.J.2
-
20
-
-
0037127222
-
Mutations that change the position of the putative gammaphosphate linker in the nucleotide binding domains of CFTR alter channel gating
-
A.L. Berger, M. Ikuma, J.F. Hunt, P.J. Thomas and M.J. Welsh (2002) Mutations that change the position of the putative gammaphosphate linker in the nucleotide binding domains of CFTR alter channel gating. J. Biol. Chem. 277(3), 2125-2131.
-
(2002)
J. Biol. Chem.
, vol.277
, Issue.3
, pp. 2125-2131
-
-
Berger, A.L.1
Ikuma, M.2
Hunt, J.F.3
Thomas, P.J.4
Welsh, M.J.5
-
21
-
-
0026077676
-
Identification and regulation of the CFTR-generated chloride channel
-
H.A. Berger, M.P. Anderson, R.J. Gregory, S. Thompson, P.W. Howard, R.R. Maurer, R. Mulligan, A.E. Smith and M.J. Welsh (1991) Identification and regulation of the CFTR-generated chloride channel. J. Clin. Invest. 88 1422-1431.
-
(1991)
J. Clin. Invest.
, vol.88
, pp. 1422-1431
-
-
Berger, H.A.1
Anderson, M.P.2
Gregory, R.J.3
Thompson, S.4
Howard, P.W.5
Maurer, R.R.6
Mulligan, R.7
Smith, A.E.8
Welsh, M.J.9
-
22
-
-
0006663253
-
Phosphorylation of CFTR Ser-737 by protein kinase A
-
(Abstract)
-
J. Borchardt, J. Kole and J. Cohn (1996) Phosphorylation of CFTR Ser-737 by protein kinase A. Pediatr. Pulm. (Suppl. 13), 212. (Abstract)
-
(1996)
Pediatr. Pulm.
, pp. 212
-
-
Borchardt, J.1
Kole, J.2
Cohn, J.3
-
23
-
-
0027998618
-
Human airway ion transport. Part two
-
R.C. Boucher (1994) Human airway ion transport. Part two. Am. J. Respir. Crit. Care Med. 150(2), 581-593.
-
(1994)
Am. J. Respir. Crit. Care Med.
, vol.150
, Issue.2
, pp. 581-593
-
-
Boucher, R.C.1
-
24
-
-
0035213684
-
Chloride conductance and genetic background modulate the cystic fibrosis phenotype of Delta F508 homozygous twins and siblings
-
I. Bronsveld, F. Mekus, J. Bijman, M. Ballmann, H.R. de Jonge, U. Laabs, et al. (2001) Chloride conductance and genetic background modulate the cystic fibrosis phenotype of Delta F508 homozygous twins and siblings. J. Clin. Invest. 108(11), 1705-1715.
-
(2001)
J. Clin. Invest.
, vol.108
, Issue.11
, pp. 1705-1715
-
-
Bronsveld, I.1
Mekus, F.2
Bijman, J.3
Ballmann, M.4
de Jonge, H.R.5
Laabs, U.6
-
25
-
-
0032813808
-
Mutations in ABC1 in Tangier disease and familial high-density lipoprotein deficiency
-
A. Brooks-Wilson, M. Marcil, S.M. Clee, L.H. Zhang, K. Roomp, M. van Dam, et al. (1999) Mutations in ABC1 in Tangier disease and familial high-density lipoprotein deficiency. Nat. Genet. 22(4), 336-345.
-
(1999)
Nat. Genet.
, vol.22
, Issue.4
, pp. 336-345
-
-
Brooks-Wilson, A.1
Marcil, M.2
Clee, S.M.3
Zhang, L.H.4
Roomp, K.5
van Dam, M.6
-
26
-
-
0030154620
-
Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein
-
C.R. Brown, L.Q. Hong-Brown, J. Biwersi, A.S. Verkman and W.J. Welch (1996) Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein. Cell Stress Chaperon 1(2), 117-125.
-
(1996)
Cell Stress Chaperon
, vol.1
, Issue.2
, pp. 117-125
-
-
Brown, C.R.1
Hong-Brown, L.Q.2
Biwersi, J.3
Verkman, A.S.4
Welch, W.J.5
-
27
-
-
0035034208
-
PCK-mediated stimulation of amphibian CFTR depends on a single phosphorylation consensus site: insertion of this site confers PKC sensitivity to human CFTR
-
B. Button, L. Reuss and G.A. Altenberg (2001) PCK-mediated stimulation of amphibian CFTR depends on a single phosphorylation consensus site: insertion of this site confers PKC sensitivity to human CFTR. J. Gen. Physiol. 117(5), 457-468.
-
(2001)
J. Gen. Physiol.
, vol.117
, Issue.5
, pp. 457-468
-
-
Button, B.1
Reuss, L.2
Altenberg, G.A.3
-
28
-
-
0029063956
-
Alternate translation initiation codons can create functional forms of cystic fibrosis transmembrane conductance regulator
-
T.P. Carroll, M.M. Morales, S.B. Fulmer, S.S. Allen, T.R. Flotte, G.R. Cutting and W.B. Guggino (1995) Alternate translation initiation codons can create functional forms of cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 270(20), 11941-11946.
-
(1995)
J. Biol. Chem.
, vol.270
, Issue.20
, pp. 11941-11946
-
-
Carroll, T.P.1
Morales, M.M.2
Fulmer, S.B.3
Allen, S.S.4
Flotte, T.R.5
Cutting, G.R.6
Guggino, W.B.7
-
29
-
-
0027255319
-
5′-adenylylimidodiphosphate does not activate CFTR chloride channels in cell-free patches of membrane
-
M.R. Carson and M.J. Welsh (1993) 5′-adenylylimidodiphosphate does not activate CFTR chloride channels in cell-free patches of membrane. Am. J. Physiol. 265(1 Pt 1), L27-L32.
-
(1993)
Am. J. Physiol.
, vol.265
, Issue.1
, pp. L27-L32
-
-
Carson, M.R.1
Welsh, M.J.2
-
30
-
-
0028906612
-
The two nucleotide-binding domains of cystic fibrosis transmembrane conductance regulator (CFTR) have distinct functions in controlling channel activity
-
M.R. Carson, S.M. Travis and M.J. Welsh (1995) The two nucleotide-binding domains of cystic fibrosis transmembrane conductance regulator (CFTR) have distinct functions in controlling channel activity. J. Biol. Chem. 270(4), 1711-1717.
-
(1995)
J. Biol. Chem.
, vol.270
, Issue.4
, pp. 1711-1717
-
-
Carson, M.R.1
Travis, S.M.2
Welsh, M.J.3
-
31
-
-
0035956875
-
Defective trafficking and function of KATP channels caused by a sulfonylurea receptor 1 mutation associated with persistent hyperinsulinemic hypoglycemia of infancy
-
E.A. Cartier, L.R. Conti, C.A. Vandenberg and S.L. Shyng (2001) Defective trafficking and function of KATP channels caused by a sulfonylurea receptor 1 mutation associated with persistent hyperinsulinemic hypoglycemia of infancy. Proc. Natl Acad. Sci. USA 98(5), 2882-2887.
-
(2001)
Proc. Natl Acad. Sci. USA
, vol.98
, Issue.5
, pp. 2882-2887
-
-
Cartier, E.A.1
Conti, L.R.2
Vandenberg, C.A.3
Shyng, S.L.4
-
32
-
-
0033694833
-
From ionic currents to molecular mechanisms: the structure and function of voltage-gated sodium channels
-
W.A. Catterall (2000) From ionic currents to molecular mechanisms: the structure and function of voltage-gated sodium channels. Neuron 26(1), 13-25.
-
(2000)
Neuron
, vol.26
, Issue.1
, pp. 13-25
-
-
Catterall, W.A.1
-
33
-
-
0025014729
-
Small conductance chloride channels in the apical membrane of thyroid cells
-
G. Champigny, B. Verrier, C. Gerard, J. Mauchamp and M. Lazdunski (1990) Small conductance chloride channels in the apical membrane of thyroid cells. FEBS Lett. 259(2), 263-268.
-
(1990)
FEBS Lett.
, vol.259
, Issue.2
, pp. 263-268
-
-
Champigny, G.1
Verrier, B.2
Gerard, C.3
Mauchamp, J.4
Lazdunski, M.5
-
34
-
-
0027311276
-
Protein kinase A (PKA) still activates CFTR chloride channel after mutagenesis of all ten PKA consensus phosphorylation sites
-
X.-B. Chang, J.A. Tabcharani, Y.-X. Hou, T.J. Jensen, N. Kartner, N. Alon, J.W. Hanrahan and J.R. Riordan (1993) Protein kinase A (PKA) still activates CFTR chloride channel after mutagenesis of all ten PKA consensus phosphorylation sites. J. Biol. Chem. 268(15), 11304-11311.
-
(1993)
J. Biol. Chem.
, vol.268
, Issue.15
, pp. 11304-11311
-
-
Chang, X.-B.1
Tabcharani, J.A.2
Hou, Y.-X.3
Jensen, T.J.4
Kartner, N.5
Alon, N.6
Hanrahan, J.W.7
Riordan, J.R.8
-
35
-
-
0028241858
-
Mapping of the cystic fibrosis transmembrane conductance regulator membrane topology by glycosylation site insertion
-
X.-B. Chang, Y.-X. Hou, T. Jensen and J.R. Riordan (1994) Mapping of the cystic fibrosis transmembrane conductance regulator membrane topology by glycosylation site insertion. J. Biol. Chem. 269(28), 18572-18575.
-
(1994)
J. Biol. Chem.
, vol.269
, Issue.28
, pp. 18572-18575
-
-
Chang, X.-B.1
Hou, Y.-X.2
Jensen, T.3
Riordan, J.R.4
-
36
-
-
0033166350
-
Removal of multiple arginine-framed trafficking signals overcomes misprocessing of delta F508 CFTR present in most patients with cystic fibrosis
-
X.-B. Chang, L. Cui, Y. Hou, T.J. Jensen, A.A. Aleksandrov, A. Mengos and J.R. Riordan (1999) Removal of multiple arginine-framed trafficking signals overcomes misprocessing of delta F508 CFTR present in most patients with cystic fibrosis. Mol. Cell 4(1), 137-142.
-
(1999)
Mol. Cell
, vol.4
, Issue.1
, pp. 137-142
-
-
Chang, X.-B.1
Cui, L.2
Hou, Y.3
Jensen, T.J.4
Aleksandrov, A.A.5
Mengos, A.6
Riordan, J.R.7
-
37
-
-
85163445282
-
Phosphorylation of protein kinase C consensus sites in NBD1 and the R domain control CFTR channel activation by PKA
-
(in press)
-
V. Chappe, D.A.R. Hinkson, X.-B. Chang, J.R. Riordan and J.W. Hanrahan (2002) Phosphorylation of protein kinase C consensus sites in NBD1 and the R domain control CFTR channel activation by PKA. J. Physiol. (in press)
-
(2002)
J. Physiol.
-
-
Chappe, V.1
Hinkson, D.A.R.2
Chang, X.-B.3
Riordan, J.R.4
Hanrahan, J.W.5
-
38
-
-
85163451528
-
Chemical cross-linking reveals different CFTR associations when the protein is heterologously and endogenously expressed
-
(submitted)
-
J.-H. Chen and J.R. Riordan (2002) Chemical cross-linking reveals different CFTR associations when the protein is heterologously and endogenously expressed. Arch. Biochem. Biophys. (submitted)
-
(2002)
Arch. Biochem. Biophys.
-
-
Chen, J.-H.1
Riordan, J.R.2
-
40
-
-
0034893723
-
A combined analysis of the cystic fibrosis transmembrane conductance regulator: implications for structure and disease models
-
J.M. Chen, C. Cutler, C. Jacques, G. Boeuf, E. Denamur, G. Lecointre, B. Mercier, G. Cramb and C. Ferec (2001) A combined analysis of the cystic fibrosis transmembrane conductance regulator: implications for structure and disease models. Mol. Biol. Evol. 18(9), 1771-1788.
-
(2001)
Mol. Biol. Evol.
, vol.18
, Issue.9
, pp. 1771-1788
-
-
Chen, J.M.1
Cutler, C.2
Jacques, C.3
Boeuf, G.4
Denamur, E.5
Lecointre, G.6
Mercier, B.7
Cramb, G.8
Ferec, C.9
-
41
-
-
0036479131
-
A Golgi-associated PDZ domain protein modulates cystic fibrosis transmembrane regulator plasma membrane expression
-
J. Cheng, B.D. Moyer, M. Milewski, J. Loffing, M. Ikeda, J.E. Mickle, G.R. Cutting, M. Li, R.A. Stanton and W.B. Gugino (2002) A Golgi-associated PDZ domain protein modulates cystic fibrosis transmembrane regulator plasma membrane expression. J. Biol. Chem. 277(5), 3520-3529.
-
(2002)
J. Biol. Chem.
, vol.277
, Issue.5
, pp. 3520-3529
-
-
Cheng, J.1
Moyer, B.D.2
Milewski, M.3
Loffing, J.4
Ikeda, M.5
Mickle, J.E.6
Cutting, G.R.7
Li, M.8
Stanton, R.A.9
Gugino, W.B.10
-
42
-
-
0025242929
-
Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
-
S.H. Cheng, R.J. Gregory, J. Marshall, S. Paul, D.W. Souza, G.A. White, C.R. O'Riordan and A.E. Smith (1990) Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell 63 827-834.
-
(1990)
Cell
, vol.63
, pp. 827-834
-
-
Cheng, S.H.1
Gregory, R.J.2
Marshall, J.3
Paul, S.4
Souza, D.W.5
White, G.A.6
O'Riordan, C.R.7
Smith, A.E.8
-
43
-
-
0025987020
-
Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel
-
S.H. Cheng, D.P. Rich, J. Marshall, R.J. Gregory, M.J. Welsh and A.E. Smith (1991) Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel. Cell 66 1027-1036.
-
(1991)
Cell
, vol.66
, pp. 1027-1036
-
-
Cheng, S.H.1
Rich, D.P.2
Marshall, J.3
Gregory, R.J.4
Welsh, M.J.5
Smith, A.E.6
-
44
-
-
0030893916
-
Locating the anion-selectivity filter of the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel
-
M. Cheung and M.H. Akabas (1997) Locating the anion-selectivity filter of the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel. J. Gen. Physiol. 109(3), 289-299.
-
(1997)
J. Gen. Physiol.
, vol.109
, Issue.3
, pp. 289-299
-
-
Cheung, M.1
Akabas, M.H.2
-
45
-
-
0033605158
-
Cystic fibrosis-associated mutations at arginine 347 alter the pore architecture of CFTR. Evidence for disruption of a salt bridge
-
J.F. Cotten and M.J. Welsh (1999) Cystic fibrosis-associated mutations at arginine 347 alter the pore architecture of CFTR. Evidence for disruption of a salt bridge. J. Biol. Chem. 274(9), 5429-5435.
-
(1999)
J. Biol. Chem.
, vol.274
, Issue.9
, pp. 5429-5435
-
-
Cotten, J.F.1
Welsh, M.J.2
-
46
-
-
0028878728
-
The genetic advantage hypothesis in cystic fibrosis heterozygotes: a murine study
-
A.W. Cuthbert, J. Halstead, R. Ratcliff, W.H. Colledge and M.J. Evans (1995) The genetic advantage hypothesis in cystic fibrosis heterozygotes: a murine study. J. Physiol. 482(Pt 2), 449-454.
-
(1995)
J. Physiol.
, vol.482
, pp. 449-454
-
-
Cuthbert, A.W.1
Halstead, J.2
Ratcliff, R.3
Colledge, W.H.4
Evans, M.J.5
-
47
-
-
25944453126
-
CFTR-protein phosphatase 2C (PP2C) association is independent of the carboxyl terminus PDZ binding motif
-
(Abstract)
-
D. Dahan, T. Zhu, A. Evagleidis and J.W. Hanrahan (1999) CFTR-protein phosphatase 2C (PP2C) association is independent of the carboxyl terminus PDZ binding motif. FASEB J. 13 A71. (Abstract)
-
(1999)
FASEB J.
, vol.13
, pp. A71
-
-
Dahan, D.1
Zhu, T.2
Evagleidis, A.3
Hanrahan, J.W.4
-
48
-
-
0032934694
-
CFTR: mechanism of anion conduction
-
D.C. Dawson, S.S. Smith and M.K. Mansoura (1999) CFTR: mechanism of anion conduction. Physiol. Rev. 79(1 Suppl), S47-S75.
-
(1999)
Physiol. Rev.
, vol.79
, Issue.1
, pp. S47-S75
-
-
Dawson, D.C.1
Smith, S.S.2
Mansoura, M.K.3
-
49
-
-
0026727675
-
Effect of modulation of protein kinase C on the cAMP-dependent chloride conductance in T84 cells
-
M.C. Dechecchi, R. Rolfini, A. Tamanini, C. Gamberi, G. Berton and G. Cabrini (1992) Effect of modulation of protein kinase C on the cAMP-dependent chloride conductance in T84 cells. FEBS Lett. 311(1), 25-28.
-
(1992)
FEBS Lett.
, vol.311
, Issue.1
, pp. 25-28
-
-
Dechecchi, M.C.1
Rolfini, R.2
Tamanini, A.3
Gamberi, C.4
Berton, G.5
Cabrini, G.6
-
50
-
-
0031156379
-
Function, evolution and structure of multidrug resistance protein (MRP)
-
R.G. Deeley and S.P. Cole (1997) Function, evolution and structure of multidrug resistance protein (MRP). Semin. Cancer Biol. 8(3), 193-204.
-
(1997)
Semin. Cancer Biol.
, vol.8
, Issue.3
, pp. 193-204
-
-
Deeley, R.G.1
Cole, S.P.2
-
51
-
-
0026781952
-
Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive
-
G.M. Denning, M.P. Anderson, J.F. Amara, J. Marshall, A.E. Smith and M.J. Welsh (1992) Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive. Nature 358(6389), 761-764.
-
(1992)
Nature
, vol.358
, Issue.6389
, pp. 761-764
-
-
Denning, G.M.1
Anderson, M.P.2
Amara, J.F.3
Marshall, J.4
Smith, A.E.5
Welsh, M.J.6
-
52
-
-
17644443114
-
Heterozygous MDR3 missense mutation associated with intrahepatic cholestasis of pregnancy: evidence for a defect in protein trafficking
-
P.H. Dixon, N. Weerasekera, K.J. Linton, O. Donaldson, J. Chambers, E. Egginton, et al. (2000) Heterozygous MDR3 missense mutation associated with intrahepatic cholestasis of pregnancy: evidence for a defect in protein trafficking. Hum. Mol. Genet. 9(8), 1209-1217.
-
(2000)
Hum. Mol. Genet.
, vol.9
, Issue.8
, pp. 1209-1217
-
-
Dixon, P.H.1
Weerasekera, N.2
Linton, K.J.3
Donaldson, O.4
Chambers, J.5
Egginton, E.6
-
53
-
-
0035203949
-
Correction of delF508-CFTR activity with benzo(c)quinolizinium compounds through facilitation of its processing in cystic fibrosis airway cells
-
R.L. Dormer, R. Derand, C.M. McNeilly, Y. Mettey, L. Bulteau-Pignoux, T. Metaye, et al. (2001) Correction of delF508-CFTR activity with benzo(c)quinolizinium compounds through facilitation of its processing in cystic fibrosis airway cells. J. Cell Sci. 114(Pt 22), 4073-4081.
-
(2001)
J. Cell Sci.
, vol.114
, pp. 4073-4081
-
-
Dormer, R.L.1
Derand, R.2
McNeilly, C.M.3
Mettey, Y.4
Bulteau-Pignoux, L.5
Metaye, T.6
-
54
-
-
0032478818
-
+ conduction and selectivity
-
D.A. Doyle, J. Morais Cabral, R.A. Pfuetzner, A. Kuo, J.M. Gulbis, S.L. Cohen, B.T. Chait and R. MacKinnon (1998) The structure of the potassium channel: molecular basis of K+ conduction and selectivity. Science 280(5360), 69-77.
-
(1998)
Science
, vol.280
, Issue.5360
, pp. 69-77
-
-
Doyle, D.A.1
Morais Cabral, J.2
Pfuetzner, R.A.3
Kuo, A.4
Gulbis, J.M.5
Cohen, S.L.6
Chait, B.T.7
MacKinnon, R.8
-
55
-
-
0026337305
-
Chloride conductance expressed by DF508 and other mutant CFTRs in Xenopus oocytes
-
M.L. Drumm, D.J. Wilkinson, L.S. Smit, R.T. Worrell, T.V. Strong, R.A. Frizzell, D.C. Dawson and F.S. Collins (1991) Chloride conductance expressed by DF508 and other mutant CFTRs in Xenopus oocytes. Science 254 1797-1799.
-
(1991)
Science
, vol.254
, pp. 1797-1799
-
-
Drumm, M.L.1
Wilkinson, D.J.2
Smit, L.S.3
Worrell, R.T.4
Strong, T.V.5
Frizzell, R.A.6
Dawson, D.C.7
Collins, F.S.8
-
56
-
-
0033851783
-
Nucleotide-binding domain 1 of cystic fibrosis transmembrane conductance regulator production of a suitable protein for structural studies
-
F. Duffieux, J.P. Annereau, J. Boucher, E. Miclet, O. Pamlard, M. Schneider, V. Stoven and J.Y. Lallemand (2000) Nucleotide-binding domain 1 of cystic fibrosis transmembrane conductance regulator production of a suitable protein for structural studies. Eur. J. Biochem. 267(17), 5306-5312.
-
(2000)
Eur. J. Biochem.
, vol.267
, Issue.17
, pp. 5306-5312
-
-
Duffieux, F.1
Annereau, J.P.2
Boucher, J.3
Miclet, E.4
Pamlard, O.5
Schneider, M.6
Stoven, V.7
Lallemand, J.Y.8
-
57
-
-
0028328645
-
Phosphorylation by cAMP-dependent protein kinase causes a conformational change in the R domain of the cystic fibrosis transmembrane conductance regulator
-
A.M. Dulhanty and J.R. Riordan (1994) Phosphorylation by cAMP-dependent protein kinase causes a conformational change in the R domain of the cystic fibrosis transmembrane conductance regulator. Biochemistry 33(13), 4072-4079.
-
(1994)
Biochemistry
, vol.33
, Issue.13
, pp. 4072-4079
-
-
Dulhanty, A.M.1
Riordan, J.R.2
-
58
-
-
0037122805
-
X-ray structure of a ClC chloride channel at 3.0 A reveals the molecular basis of anion selectivity
-
R. Dutzler, E.B. Campbell, M. Cadene, B.T. Chait and R. MacKinnon (2002) X-ray structure of a ClC chloride channel at 3.0 A reveals the molecular basis of anion selectivity. Nature 415(6869), 287-294.
-
(2002)
Nature
, vol.415
, Issue.6869
, pp. 287-294
-
-
Dutzler, R.1
Campbell, E.B.2
Cadene, M.3
Chait, B.T.4
MacKinnon, R.5
-
59
-
-
0021070747
-
Ionic selectivity revisited: the role of kinetic and equilibrium processes in ion permeation through channels
-
G. Eisenman and R. Horn (1983) Ionic selectivity revisited: the role of kinetic and equilibrium processes in ion permeation through channels. J. Membr. Biol. 76(3), 197-225.
-
(1983)
J. Membr. Biol.
, vol.76
, Issue.3
, pp. 197-225
-
-
Eisenman, G.1
Horn, R.2
-
60
-
-
0032530592
-
Structural analysis of cloned plasma membrane proteins by freeze-fracture electron microscopy
-
S. Eskandari, E.M. Wright, M. Kreman, D.M. Starace and G.A. Zampighi (1998) Structural analysis of cloned plasma membrane proteins by freeze-fracture electron microscopy. Proc. Natl. Acad. Sci. USA 95(19), 11235-11240.
-
(1998)
Proc. Natl. Acad. Sci. USA
, vol.95
, Issue.19
, pp. 11235-11240
-
-
Eskandari, S.1
Wright, E.M.2
Kreman, M.3
Starace, D.M.4
Zampighi, G.A.5
-
61
-
-
0033018496
-
Accelerated transport and maturation of lysosomal alpha-galactosidase A in Fabry lymphoblasts by an enzyme inhibitor
-
J.Q. Fan, S. Ishii, N. Asano and Y. Suzuki (1999) Accelerated transport and maturation of lysosomal alpha-galactosidase A in Fabry lymphoblasts by an enzyme inhibitor. Nat. Med. 5(1), 112-115.
-
(1999)
Nat. Med.
, vol.5
, Issue.1
, pp. 112-115
-
-
Fan, J.Q.1
Ishii, S.2
Asano, N.3
Suzuki, Y.4
-
62
-
-
0033601370
-
Pharmacological rescue of mutant p53 conformation and function
-
B.A. Foster, H.A. Coffey, M.J. Morin and F. Rastinejad (1999) Pharmacological rescue of mutant p53 conformation and function. Science 286(5449), 2507-2510.
-
(1999)
Science
, vol.286
, Issue.5449
, pp. 2507-2510
-
-
Foster, B.A.1
Coffey, H.A.2
Morin, M.J.3
Rastinejad, F.4
-
63
-
-
0022516747
-
Altered regulation of airway epithelial cell chloride channels in cystic fibrosis
-
R.A. Frizzell, G. Rechkemmer and R.L. Shoemaker (1986) Altered regulation of airway epithelial cell chloride channels in cystic fibrosis. Science 233 558-560.
-
(1986)
Science
, vol.233
, pp. 558-560
-
-
Frizzell, R.A.1
Rechkemmer, G.2
Shoemaker, R.L.3
-
64
-
-
0027191065
-
CFTR and outward rectifying chloride channels are distinct proteins with a regulatory relationship
-
S.E. Gabriel, L.L. Clarke, R.C. Boucher and M.J. Stutts (1993) CFTR and outward rectifying chloride channels are distinct proteins with a regulatory relationship. Nature 363 263-268.
-
(1993)
Nature
, vol.363
, pp. 263-268
-
-
Gabriel, S.E.1
Clarke, L.L.2
Boucher, R.C.3
Stutts, M.J.4
-
65
-
-
0028062781
-
Cystic fibrosis heterozygote resistance to cholera toxin in the cystic fibrosis mouse model
-
S.E. Gabriel, K.N. Brigman, B.H. Koller, R.C. Boucher and M.J. Stutts (1994) Cystic fibrosis heterozygote resistance to cholera toxin in the cystic fibrosis mouse model. Science 266(5282), 107-109.
-
(1994)
Science
, vol.266
, Issue.5282
, pp. 107-109
-
-
Gabriel, S.E.1
Brigman, K.N.2
Koller, B.H.3
Boucher, R.C.4
Stutts, M.J.5
-
66
-
-
0032936619
-
Control of CFTR channel gating by phosphorylation and nucleotide hydrolysis
-
D.C. Gadsby and A.C. Nairn (1999) Control of CFTR channel gating by phosphorylation and nucleotide hydrolysis. Physiol. Rev. 79(1 Suppl.), S77-S107.
-
(1999)
Physiol. Rev.
, vol.79
, Issue.1
, pp. S77-S107
-
-
Gadsby, D.C.1
Nairn, A.C.2
-
67
-
-
0032617252
-
− ion channels by phosphorylation and dephosphorylation
-
D.C. Gadsby and A.C. Nairn (1999) Regulation of CFTR Cl− ion channels by phosphorylation and dephosphorylation. Adv. Sec. Mess. Phosph. 33 79-106.
-
(1999)
Adv. Sec. Mess. Phosph.
, vol.33
, pp. 79-106
-
-
Gadsby, D.C.1
Nairn, A.C.2
-
68
-
-
0035827680
-
Novel CFTR chloride channel activators identified by screening of combinatorial libraries based on flavone and benzoquino-lizinium lead compounds
-
L.J. Galietta, M.F. Springsteel, M. Eda, E.J. Niedzinski, K. By, M.J. Haddadin, M.J. Kurth, M.H. Nantz and A.S. Verkman (2001) Novel CFTR chloride channel activators identified by screening of combinatorial libraries based on flavone and benzoquino-lizinium lead compounds. J. Biol. Chem. 276(23), 19723-19728.
-
(2001)
J. Biol. Chem.
, vol.276
, Issue.23
, pp. 19723-19728
-
-
Galietta, L.J.1
Springsteel, M.F.2
Eda, M.3
Niedzinski, E.J.4
By, K.5
Haddadin, M.J.6
Kurth, M.J.7
Nantz, M.H.8
Verkman, A.S.9
-
69
-
-
0034724680
-
Comparison of the functional characteristics of the nucleotide binding domains of multidrug resistance protein 1
-
M. Gao, H.R. Cui, D.W. Loe, C.E. Grant, K.C. Almquist, S.P. Cole and R.G. Deeley (2000) Comparison of the functional characteristics of the nucleotide binding domains of multidrug resistance protein 1. J. Biol. Chem. 275(17), 13098-13108.
-
(2000)
J. Biol. Chem.
, vol.275
, Issue.17
, pp. 13098-13108
-
-
Gao, M.1
Cui, H.R.2
Loe, D.W.3
Grant, C.E.4
Almquist, K.C.5
Cole, S.P.6
Deeley, R.G.7
-
70
-
-
0030945484
-
Epithelial sodium channels: function, structure, and regulation
-
H. Garty and L.G. Palmer (1997) Epithelial sodium channels: function, structure, and regulation. Physiol. Rev. 77(2), 359-396.
-
(1997)
Physiol. Rev.
, vol.77
, Issue.2
, pp. 359-396
-
-
Garty, H.1
Palmer, L.G.2
-
71
-
-
0037023764
-
A principal role for the proteasome in ER-associated degradation of misfolded intracellular CFTR
-
M.S. Gelman, E.S. Kannegaard and R.R. Kopito (2002) A principal role for the proteasome in ER-associated degradation of misfolded intracellular CFTR. J. Biol. Chem. 277 11709-11714.
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 11709-11714
-
-
Gelman, M.S.1
Kannegaard, E.S.2
Kopito, R.R.3
-
72
-
-
0035847015
-
Localization of sequences within the C-terminal domain of the cystic fibrosis transmembrane conductance regulator which impact maturation and stability
-
M. Gentzsch and J.R. Riordan (2001) Localization of sequences within the C-terminal domain of the cystic fibrosis transmembrane conductance regulator which impact maturation and stability. J. Biol. Chem. 276(2), 1291-1298.
-
(2001)
J. Biol. Chem.
, vol.276
, Issue.2
, pp. 1291-1298
-
-
Gentzsch, M.1
Riordan, J.R.2
-
73
-
-
0036714504
-
Functional analysis of the C-terminal boundary of the second nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator and structural implications
-
05/20/02 DOI: 10.1042/BJ20020511
-
M. Gentzsch, A. Aleksandrov, L. Aleksandrov and J.R. Riordan (2002) Functional analysis of the C-terminal boundary of the second nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator and structural implications. Biochem. J. 05/20/02 DOI: 10.1042/BJ20020511
-
(2002)
Biochem. J.
-
-
Gentzsch, M.1
Aleksandrov, A.2
Aleksandrov, L.3
Riordan, J.R.4
-
74
-
-
0023782863
-
Secretin-regulated chloride channel on the apical plasma membrane of pancreatic duct cells
-
M.A. Gray, J.R. Greenwell and B.E. Argent (1988) Secretin-regulated chloride channel on the apical plasma membrane of pancreatic duct cells. J. Mem. Biol. 105(2), 131-142.
-
(1988)
J. Mem. Biol.
, vol.105
, Issue.2
, pp. 131-142
-
-
Gray, M.A.1
Greenwell, J.R.2
Argent, B.E.3
-
75
-
-
0024426797
-
Two types of chloride channel on duct cells cultured from human fetal pancreas
-
M.A. Gray, A. Harris, L. Coleman, J.R. Greenwell and B.E. Argent (1989) Two types of chloride channel on duct cells cultured from human fetal pancreas. Am. J. Physiol. 257 C240-C251.
-
(1989)
Am. J. Physiol.
, vol.257
, pp. C240-C251
-
-
Gray, M.A.1
Harris, A.2
Coleman, L.3
Greenwell, J.R.4
Argent, B.E.5
-
76
-
-
0025092468
-
Anion selectivity and block of the small-conductance chloride channel on pancreatic duct cells
-
Pt 1
-
M.A. Gray, C.E. Pollard, A. Harris, L. Coleman, J.R. Greenwell and B.E. Argent (1990) Anion selectivity and block of the small-conductance chloride channel on pancreatic duct cells. Am. J. Physiol. 259(5), C752-C761. Pt 1
-
(1990)
Am. J. Physiol.
, vol.259
, Issue.5
, pp. C752-C761
-
-
Gray, M.A.1
Pollard, C.E.2
Harris, A.3
Coleman, L.4
Greenwell, J.R.5
Argent, B.E.6
-
77
-
-
0033608961
-
Arg352 is a major determinant of charge selectivity in the cystic fibrosis transmembrane conductance regulator chloride channel
-
R. Guinamard and M.H. Akabas (1999) Arg352 is a major determinant of charge selectivity in the cystic fibrosis transmembrane conductance regulator chloride channel. Biochemistry 38(17), 5528-5537.
-
(1999)
Biochemistry
, vol.38
, Issue.17
, pp. 5528-5537
-
-
Guinamard, R.1
Akabas, M.H.2
-
78
-
-
0028070453
-
Effects of pyrophosphate and nucleotide analogs suggest a role for ATP hydrolysis in cystic fibrosis transmembrane regulator channel gating
-
K.L. Gunderson and R.R. Kopito (1994) Effects of pyrophosphate and nucleotide analogs suggest a role for ATP hydrolysis in cystic fibrosis transmembrane regulator channel gating. J. Biol. Chem. 269(30), 19349-19353.
-
(1994)
J. Biol. Chem.
, vol.269
, Issue.30
, pp. 19349-19353
-
-
Gunderson, K.L.1
Kopito, R.R.2
-
79
-
-
0035811064
-
Asymmetric structure of the cystic fibrosis transmembrane conductance regulator chloride channel pore suggested by mutagenesis of the twelfth transmembrane region
-
J. Gupta, A. Evagelidis, J.W. Hanrahan and P. Linsdell (2001) Asymmetric structure of the cystic fibrosis transmembrane conductance regulator chloride channel pore suggested by mutagenesis of the twelfth transmembrane region. Biochemistry 40(22), 6620-6627.
-
(2001)
Biochemistry
, vol.40
, Issue.22
, pp. 6620-6627
-
-
Gupta, J.1
Evagelidis, A.2
Hanrahan, J.W.3
Linsdell, P.4
-
80
-
-
0033933777
-
Inhibition of cystic fibrosis transmembrane conductance regulator by novel interaction with the metabolic sensor AMP-activated protein kinase
-
K.R. Hallows, V. Raghuram, B.E. Kemp, L.A. Witters and J.K. Foskett (2000) Inhibition of cystic fibrosis transmembrane conductance regulator by novel interaction with the metabolic sensor AMP-activated protein kinase. J. Clin. Invest. 105(12), 1711-1721.
-
(2000)
J. Clin. Invest.
, vol.105
, Issue.12
, pp. 1711-1721
-
-
Hallows, K.R.1
Raghuram, V.2
Kemp, B.E.3
Witters, L.A.4
Foskett, J.K.5
-
81
-
-
0035805490
-
Disease-associated mutations in the extracytoplasmic loops of cystic fibrosis transmembrane conductance regulator do not impede biosynthetic processing but impair chloride channel stability
-
M.M. Hämmerle, A.A. Aleksandrov and J.R. Riordan (2001) Disease-associated mutations in the extracytoplasmic loops of cystic fibrosis transmembrane conductance regulator do not impede biosynthetic processing but impair chloride channel stability. J. Biol. Chem. 276(18), 14848-14854.
-
(2001)
J. Biol. Chem.
, vol.276
, Issue.18
, pp. 14848-14854
-
-
Hämmerle, M.M.1
Aleksandrov, A.A.2
Riordan, J.R.3
-
82
-
-
0013690743
-
Patch clamp studies of apical membrane chloride channels
-
J.A. Dodge, D.J.H. Brock, J.H. Widdicombe (Eds), London: John Wiley and Sons
-
J.W. Hanrahan, J.A. Tabcharani and R. Grygorczyk (1993) Patch clamp studies of apical membrane chloride channels. J.A. Dodge, D.J.H. Brock, J.H. Widdicombe (Eds) Cystic Fibrosis-Current Topics London: John Wiley and Sons 93-137.
-
(1993)
Cystic Fibrosis-Current Topics
, pp. 93-137
-
-
Hanrahan, J.W.1
Tabcharani, J.A.2
Grygorczyk, R.3
-
83
-
-
0026702994
-
Recombinant synthesis purification and nucleotide binding characteristics of the first nucleotide binding domains of the cystic fibrosis gene product
-
J. Hartman, Z. Huong, T.A. Rado, S. Peng, T. Jilling, D.D. Muccio and E.J. Sorscher (1992) Recombinant synthesis purification and nucleotide binding characteristics of the first nucleotide binding domains of the cystic fibrosis gene product. J. Biol. Chem. 267 6455-6458.
-
(1992)
J. Biol. Chem.
, vol.267
, pp. 6455-6458
-
-
Hartman, J.1
Huong, Z.2
Rado, T.A.3
Peng, S.4
Jilling, T.5
Muccio, D.D.6
Sorscher, E.J.7
-
84
-
-
0026621245
-
ABC transporters: from microorganisms to man
-
C.F. Higgins (1992) ABC transporters: from microorganisms to man. Annu. Rev. Cell Biol. 8 67-113.
-
(1992)
Annu. Rev. Cell Biol.
, vol.8
, pp. 67-113
-
-
Higgins, C.F.1
-
86
-
-
0029860763
-
On the use of thiol-modifying agents to determine channel topology
-
M. Holmgren, Y. Liu, Y. Xu and G. Yellen (1996) On the use of thiol-modifying agents to determine channel topology. Neuropharmacology 35(7), 797-804.
-
(1996)
Neuropharmacology
, vol.35
, Issue.7
, pp. 797-804
-
-
Holmgren, M.1
Liu, Y.2
Xu, Y.3
Yellen, G.4
-
87
-
-
0034705293
-
Structural biology of Rad50 ATPase: ATP-driven conformation control in DNA double-strand break repair and the ABC-ATPase superfamily
-
K.P. Hopfner, A. Karcher, D.S. Shin, L. Craig, L.M. Arthur, J.P. Carney and J.A. Tainer (2000) Structural biology of Rad50 ATPase: ATP-driven conformation control in DNA double-strand break repair and the ABC-ATPase superfamily. Cell 101(7), 789-800.
-
(2000)
Cell
, vol.101
, Issue.7
, pp. 789-800
-
-
Hopfner, K.P.1
Karcher, A.2
Shin, D.S.3
Craig, L.4
Arthur, L.M.5
Carney, J.P.6
Tainer, J.A.7
-
88
-
-
0034617097
-
Allosteric interactions between the two non-equivalent nucleotide binding domains of multidrug resistance protein MRP1
-
Y.-X. Hou, L. Cui, J.R. Riordan and X.-B. Chang (2000) Allosteric interactions between the two non-equivalent nucleotide binding domains of multidrug resistance protein MRP1. J. Biol. Chem. 275 20280-20287.
-
(2000)
J. Biol. Chem.
, vol.275
, pp. 20280-20287
-
-
Hou, Y.-X.1
Cui, L.2
Riordan, J.R.3
Chang, X.-B.4
-
89
-
-
0034630742
-
ATP hydrolysis by a CFTR domain: pharmacology and effects of G551D mutation
-
L.D. Howell, R. Borchardt and J.A. Cohn (2000) ATP hydrolysis by a CFTR domain: pharmacology and effects of G551D mutation. Biochem. Biophys. Res. Commun. 271(2), 518-525.
-
(2000)
Biochem. Biophys. Res. Commun.
, vol.271
, Issue.2
, pp. 518-525
-
-
Howell, L.D.1
Borchardt, R.2
Cohn, J.A.3
-
90
-
-
0035923676
-
Compartmentalized autocrine signaling to cystic fibrosis transmembrane conductance regulator at the apical membrane of airway epithelial cells
-
P. Huang, E.R. Lazarowski, R. Tarran, S.L. Milgram, R.C. Boucher and M.J. Stutts (2001) Compartmentalized autocrine signaling to cystic fibrosis transmembrane conductance regulator at the apical membrane of airway epithelial cells. Proc. Natl Acad. Sci. USA 98(24), 14120-14125.
-
(2001)
Proc. Natl Acad. Sci. USA
, vol.98
, Issue.24
, pp. 14120-14125
-
-
Huang, P.1
Lazarowski, E.R.2
Tarran, R.3
Milgram, S.L.4
Boucher, R.C.5
Stutts, M.J.6
-
91
-
-
0024353683
-
− channels in CF: Lack of activation by protein kinase C and cAMP-dependent protein kinase
-
T. Hwang, L. Lu, P.L. Zeitlin, D.C. Gruenert, R. Huganir and W.B. Guggino (1989) Cl− channels in CF: Lack of activation by protein kinase C and cAMP-dependent protein kinase. Science 244 1351-1353.
-
(1989)
Science
, vol.244
, pp. 1351-1353
-
-
Hwang, T.1
Lu, L.2
Zeitlin, P.L.3
Gruenert, D.C.4
Huganir, R.5
Guggino, W.B.6
-
92
-
-
0028340159
-
Regulation of gating of cystic fibrosis transmembrane conductance regulator C1 channels by phosphorylation and ATP hydrolysis
-
T.-C. Hwang, G. Baukrowtiz, G. Nagel, A.C. Horie and D.C. Gadsby (1994) Regulation of gating of cystic fibrosis transmembrane conductance regulator C1 channels by phosphorylation and ATP hydrolysis. Proc. Natl Acad. Sci. USA 91 4698-4702.
-
(1994)
Proc. Natl Acad. Sci. USA
, vol.91
, pp. 4698-4702
-
-
Hwang, T.-C.1
Baukrowtiz, G.2
Nagel, G.3
Horie, A.C.4
Gadsby, D.C.5
-
93
-
-
0034682521
-
− channel gating by ATP binding and hydrolysis
-
M. Ikuma and M.J. Welsh (2000) Regulation of CFTR Cl− channel gating by ATP binding and hydrolysis. Proc. Natl Acad. Sci. USA 97(15), 8675-8680.
-
(2000)
Proc. Natl Acad. Sci. USA
, vol.97
, Issue.15
, pp. 8675-8680
-
-
Ikuma, M.1
Welsh, M.J.2
-
94
-
-
0029898253
-
+ channels
-
N. Inagaki, T. Gonoi, J.P. Clement, C.Z. Wang, L. Aguilar-Bryan, J. Bryan and S. Seino (1996) A family of sulfonylurea receptors determines the pharmacological properties of ATP-sensitive K+ channels. Neuron 16(5), 1011-1017.
-
(1996)
Neuron
, vol.16
, Issue.5
, pp. 1011-1017
-
-
Inagaki, N.1
Gonoi, T.2
Clement, J.P.3
Wang, C.Z.4
Aguilar-Bryan, L.5
Bryan, J.6
Seino, S.7
-
95
-
-
0030668944
-
Block by MOPS reveals a conformation change in the CFTR pore produced by ATP hydrolysis
-
Pt 1
-
H. Ishihara and M.J. Welsh (1997) Block by MOPS reveals a conformation change in the CFTR pore produced by ATP hydrolysis. Am. J. Physiol. 273(4), C1278-C1289. Pt 1
-
(1997)
Am. J. Physiol.
, vol.273
, Issue.4
, pp. C1278-C1289
-
-
Ishihara, H.1
Welsh, M.J.2
-
96
-
-
0028858161
-
Multiple proteolytic systems, including the proteasome, contribute to CFTR processing
-
T.J. Jensen, M.A. Loo, S. Pind, D.B. Williams, A.L. Goldberg and J.R. Riordan (1995) Multiple proteolytic systems, including the proteasome, contribute to CFTR processing. Cell 83 129-135.
-
(1995)
Cell
, vol.83
, pp. 129-135
-
-
Jensen, T.J.1
Loo, M.A.2
Pind, S.3
Williams, D.B.4
Goldberg, A.L.5
Riordan, J.R.6
-
97
-
-
0031046796
-
Phosphorylation by protein kinase C is required for acute activation of cystic fibrosis transmembrane conductance regulator by protein kinase A
-
Y. Jia, C.J. Mathews and J.W. Hanrahan (1997) Phosphorylation by protein kinase C is required for acute activation of cystic fibrosis transmembrane conductance regulator by protein kinase A. J. Biol. Chem. 272(8), 4978-4984.
-
(1997)
J. Biol. Chem.
, vol.272
, Issue.8
, pp. 4978-4984
-
-
Jia, Y.1
Mathews, C.J.2
Hanrahan, J.W.3
-
98
-
-
0032588980
-
DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis [see comments]
-
N. Kalin, A. Claass, M. Sommer, E. Puchelle and B. Tummler (1999) DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis [see comments]. J. Clin. Invest. 103(10), 1379-1389.
-
(1999)
J. Clin. Invest.
, vol.103
, Issue.10
, pp. 1379-1389
-
-
Kalin, N.1
Claass, A.2
Sommer, M.3
Puchelle, E.4
Tummler, B.5
-
99
-
-
0034941969
-
Crystal structures of the MJ1267 ATP binding cassette reveal an induced-fit effect at the ATPase active site of an ABC transporter
-
N. Karpowich, O. Martsinkevich, L. Millen, Y.R. Yuan, P.L. Dai, K. MacVey, P.J. Thomas and J.F. Hunt (2001) Crystal structures of the MJ1267 ATP binding cassette reveal an induced-fit effect at the ATPase active site of an ABC transporter. Structure (Camb) 9(7), 571-586.
-
(2001)
Structure (Camb)
, vol.9
, Issue.7
, pp. 571-586
-
-
Karpowich, N.1
Martsinkevich, O.2
Millen, L.3
Yuan, Y.R.4
Dai, P.L.5
MacVey, K.6
Thomas, P.J.7
Hunt, J.F.8
-
100
-
-
0026073070
-
Expression of the cystic fibrosis gene in non-epithelial invertebrate cells produces a regulated anion conductance
-
N. Kartner, J.W. Hanrahan, T.J. Jensen, A.L. Naismith, S. Sun, C.A. Ackerley, et al. (1991) Expression of the cystic fibrosis gene in non-epithelial invertebrate cells produces a regulated anion conductance. Cell 64(4), 681-691.
-
(1991)
Cell
, vol.64
, Issue.4
, pp. 681-691
-
-
Kartner, N.1
Hanrahan, J.W.2
Jensen, T.J.3
Naismith, A.L.4
Sun, S.5
Ackerley, C.A.6
-
101
-
-
0026907529
-
Mislocalization of delta F508 CFTR in cystic fibrosis sweat gland
-
N. Kartner, O. Augustinas, T.J. Jensen, A.L. Naismith and J.R. Riordan (1992) Mislocalization of delta F508 CFTR in cystic fibrosis sweat gland. Nat. Genet. 1(5), 321-327.
-
(1992)
Nat. Genet.
, vol.1
, Issue.5
, pp. 321-327
-
-
Kartner, N.1
Augustinas, O.2
Jensen, T.J.3
Naismith, A.L.4
Riordan, J.R.5
-
102
-
-
0033652766
-
Impaired protein maturation of the conjugate export pump multidrug resistance protein 2 as a consequence of a deletion mutation in Dubin-Johnson syndrome
-
V. Keitel, J. Kartenbeck, A.T. Nies, H. Spring, M. Brom and D. Keppler (2000) Impaired protein maturation of the conjugate export pump multidrug resistance protein 2 as a consequence of a deletion mutation in Dubin-Johnson syndrome. Hepatology 32(6), 1317-1328.
-
(2000)
Hepatology
, vol.32
, Issue.6
, pp. 1317-1328
-
-
Keitel, V.1
Kartenbeck, J.2
Nies, A.T.3
Spring, H.4
Brom, M.5
Keppler, D.6
-
103
-
-
0034127176
-
Rational design of potent human transthyretin amyloid disease inhibitors
-
T. Klabunde, H.M. Petrassi, V.B. Oza, P. Raman, J.W. Kelly and J.C. Sacchettini (2000) Rational design of potent human transthyretin amyloid disease inhibitors. Nat. Struct. Biol. 7(4), 312-321.
-
(2000)
Nat. Struct. Biol.
, vol.7
, Issue.4
, pp. 312-321
-
-
Klabunde, T.1
Petrassi, H.M.2
Oza, V.B.3
Raman, P.4
Kelly, J.W.5
Sacchettini, J.C.6
-
104
-
-
0020610435
-
Abnormal ion permeation through cystic fibrosis respiratory epithelium
-
M.R. Knowles, M.J. Stutts, A. Spock, N. Fischer, J.T. Gatzy and R.C. Boucher (1983) Abnormal ion permeation through cystic fibrosis respiratory epithelium. Science 221 1067-1070.
-
(1983)
Science
, vol.221
, pp. 1067-1070
-
-
Knowles, M.R.1
Stutts, M.J.2
Spock, A.3
Fischer, N.4
Gatzy, J.T.5
Boucher, R.C.6
-
105
-
-
0029113976
-
The first nucleotide binding fold of the cystic fibrosis transmembrane conductance regulator can function as an active ATPase
-
Y.H. Ko and P.L. Pedersen (1995) The first nucleotide binding fold of the cystic fibrosis transmembrane conductance regulator can function as an active ATPase. J. Biol. Chem. 270(38), 22093-22096.
-
(1995)
J. Biol. Chem.
, vol.270
, Issue.38
, pp. 22093-22096
-
-
Ko, Y.H.1
Pedersen, P.L.2
-
106
-
-
0027524866
-
The cystic fibrosis transmembrane conductance regulator. Overexpression, purification, and characterization of wild type and delta F508 mutant forms of the first nucleotide binding fold in fusion with the maltose-binding protein
-
Y.H. Ko, P.J. Thomas, M.R. Delannoy and P.L. Pedersen (1993) The cystic fibrosis transmembrane conductance regulator. Overexpression, purification, and characterization of wild type and delta F508 mutant forms of the first nucleotide binding fold in fusion with the maltose-binding protein. J. Biol. Chem. 268(32), 24330-24338.
-
(1993)
J. Biol. Chem.
, vol.268
, Issue.32
, pp. 24330-24338
-
-
Ko, Y.H.1
Thomas, P.J.2
Delannoy, M.R.3
Pedersen, P.L.4
-
107
-
-
0035853675
-
Perturbation of the pore of the cystic fibrosis transmembrane conductance regulator (CFTR) inhibits its ATPase activity
-
I. Kogan, M. Ramjeesingh, L.J. Huan, Y. Wang and C.E. Bear (2001) Perturbation of the pore of the cystic fibrosis transmembrane conductance regulator (CFTR) inhibits its ATPase activity. J. Biol. Chem. 276(15), 11575-11581.
-
(2001)
J. Biol. Chem.
, vol.276
, Issue.15
, pp. 11575-11581
-
-
Kogan, I.1
Ramjeesingh, M.2
Huan, L.J.3
Wang, Y.4
Bear, C.E.5
-
108
-
-
0035203716
-
− concentration
-
J. Konig, R. Schreiber, T. Voelcker, M. Mall and K. Kunzelmann (2001) The cystic fibrosis transmembrane conductance regulator (CFTR) inhibits ENaC through an increase in the intracellular Cl− concentration. EMBO Rep. 2(11), 1047-1051.
-
(2001)
EMBO Rep.
, vol.2
, Issue.11
, pp. 1047-1051
-
-
Konig, J.1
Schreiber, R.2
Voelcker, T.3
Mall, M.4
Kunzelmann, K.5
-
109
-
-
0032957204
-
Biosynthesis and degradation of CFTR
-
R.R. Kopito (1999) Biosynthesis and degradation of CFTR. Physiol. Rev. 79(1 Suppl.), S167-S173.
-
(1999)
Physiol. Rev.
, vol.79
, Issue.1
, pp. S167-S173
-
-
Kopito, R.R.1
-
110
-
-
85163468112
-
CFTR expression in epithelia from cystic fibrosis patients
-
(Abstract 2137)
-
J.M. Kreda, M. Mall, H. Seydewitz, J. Kuehr, A. Mengos, T.J. Jensen, J.R. Riordan and R. Boucher (2001) CFTR expression in epithelia from cystic fibrosis patients. Mol. Biol. Cell (Suppl. 12), (Abstract 2137)
-
(2001)
Mol. Biol. Cell
-
-
Kreda, J.M.1
Mall, M.2
Seydewitz, H.3
Kuehr, J.4
Mengos, A.5
Jensen, T.J.6
Riordan, J.R.7
Boucher, R.8
-
111
-
-
0000427456
-
− exchange in mouse submandibular and pancreatic ducts
-
M.G. Lee, J.Y. Choi, X. Luo, E. Strickland, P.J. Thomas and S. Muallem (1999) Cystic fibrosis transmembrane conductance regulator regulates luminal Cl−/HCO3 − exchange in mouse submandibular and pancreatic ducts. J. Biol. Chem. 274(21), 14670-14677.
-
(1999)
J. Biol. Chem.
, vol.274
, Issue.21
, pp. 14670-14677
-
-
Lee, M.G.1
Choi, J.Y.2
Luo, X.3
Strickland, E.4
Thomas, P.J.5
Muallem, S.6
-
112
-
-
0033071210
-
Genotype/Phenotype analysis of a photoreceptor-specific ATP-binding cassette transporter gene, ABCR, in Stargardt disease
-
R.A. Lewis, N.F. Shroyer, N. Singh, R. Allikmets, A. Hutchinson, Y. Li, J.R. Lupski, M. Leppert and M. Dean (1999) Genotype/Phenotype analysis of a photoreceptor-specific ATP-binding cassette transporter gene, ABCR, in Stargardt disease. Am. J. Hum. Genet. 64(2), 422-434.
-
(1999)
Am. J. Hum. Genet.
, vol.64
, Issue.2
, pp. 422-434
-
-
Lewis, R.A.1
Shroyer, N.F.2
Singh, N.3
Allikmets, R.4
Hutchinson, A.5
Li, Y.6
Lupski, J.R.7
Leppert, M.8
Dean, M.9
-
113
-
-
0027483610
-
The cystic fibrosis mutation (delta F508) does not influence the chloride channel activity of CFTR
-
C. Li, M. Ramjeesingh, E. Reyes, T. Jensen, X. Chang, J.M. Rommens and C.E. Bear (1993) The cystic fibrosis mutation (delta F508) does not influence the chloride channel activity of CFTR. Nat. Genet. 3(4), 311-316.
-
(1993)
Nat. Genet.
, vol.3
, Issue.4
, pp. 311-316
-
-
Li, C.1
Ramjeesingh, M.2
Reyes, E.3
Jensen, T.4
Chang, X.5
Rommens, J.M.6
Bear, C.E.7
-
114
-
-
0029904733
-
ATPase activity of the cystic fibrosis transmembrane conductance regulator
-
C. Li, M. Ramjeesingh, W. Wang, E. Garami, M. Hewryk, D. Lee, J.M. Rommens, K. Galley and C.E. Bear (1996) ATPase activity of the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 271(45), 28463-28468.
-
(1996)
J. Biol. Chem.
, vol.271
, Issue.45
, pp. 28463-28468
-
-
Li, C.1
Ramjeesingh, M.2
Wang, W.3
Garami, E.4
Hewryk, M.5
Lee, D.6
Rommens, J.M.7
Galley, K.8
Bear, C.E.9
-
115
-
-
0023931773
-
Cyclic AMP-dependent protein kinase opens chloride channels in normal but not cystic fibrosis airway epithelium
-
M. Li, J.D. McCann, C.M. Liedtke, A.C. Nairn, P. Greengard and M.J. Welsh (1988) Cyclic AMP-dependent protein kinase opens chloride channels in normal but not cystic fibrosis airway epithelium. Nature 331 358-360.
-
(1988)
Nature
, vol.331
, pp. 358-360
-
-
Li, M.1
McCann, J.D.2
Liedtke, C.M.3
Nairn, A.C.4
Greengard, P.5
Welsh, M.J.6
-
116
-
-
0029861859
-
− channels expressed in a mammalian cell line and its regulation by a critical pore residue
-
P. Linsdell and J.W. Hanrahan (1996) Disulphonic stilbene block of cystic fibrosis transmembrane conductance regulator Cl− channels expressed in a mammalian cell line and its regulation by a critical pore residue. J. Physiol. 496(Pt 3), 687-693.
-
(1996)
J. Physiol.
, vol.496
, pp. 687-693
-
-
Linsdell, P.1
Hanrahan, J.W.2
-
117
-
-
0031954021
-
Adenosine triphosphate-dependent asymmetry of anion permeation in the cystic fibrosis transmembrane conductance regulator chloride channel
-
P. Linsdell and J.W. Hanrahan (1998) Adenosine triphosphate-dependent asymmetry of anion permeation in the cystic fibrosis transmembrane conductance regulator chloride channel. J. Gen. Physiol. 111(4), 601-614.
-
(1998)
J. Gen. Physiol.
, vol.111
, Issue.4
, pp. 601-614
-
-
Linsdell, P.1
Hanrahan, J.W.2
-
118
-
-
0030885564
-
Permeability of wild-type and mutant cystic fibrosis transmembrane conductance regulator chloride channels to polyatomic anions
-
P. Linsdell, J.A. Tabcharani, J.M. Rommens, Y.X. Hou, X.B. Chang, L.C. Tsui, J.R. Riordan and J.W. Hanrahan (1997) Permeability of wild-type and mutant cystic fibrosis transmembrane conductance regulator chloride channels to polyatomic anions. J. Gen. Physiol. 110(4), 355-364.
-
(1997)
J. Gen. Physiol.
, vol.110
, Issue.4
, pp. 355-364
-
-
Linsdell, P.1
Tabcharani, J.A.2
Rommens, J.M.3
Hou, Y.X.4
Chang, X.B.5
Tsui, L.C.6
Riordan, J.R.7
Hanrahan, J.W.8
-
119
-
-
0030886246
-
Multi-ion mechanism for ion permeation and block in the cystic fibrosis transmembrane conductance regulator chloride channels
-
P. Linsdell, J.A. Tabcharani and J.W. Hanrahan (1997) Multi-ion mechanism for ion permeation and block in the cystic fibrosis transmembrane conductance regulator chloride channels. J. Gen. Physiol. 110(4), 365-377.
-
(1997)
J. Gen. Physiol.
, vol.110
, Issue.4
, pp. 365-377
-
-
Linsdell, P.1
Tabcharani, J.A.2
Hanrahan, J.W.3
-
120
-
-
0034084793
-
Molecular determinants of anion selectivity in the cystic fibrosis transmembrane conductance regulator chloride channel pore
-
P. Linsdell, A. Evagelidis and J.W. Hanrahan (2000) Molecular determinants of anion selectivity in the cystic fibrosis transmembrane conductance regulator chloride channel pore. Biophys. J. 78(6), 2973-2982.
-
(2000)
Biophys. J.
, vol.78
, Issue.6
, pp. 2973-2982
-
-
Linsdell, P.1
Evagelidis, A.2
Hanrahan, J.W.3
-
121
-
-
0032401771
-
Perturbation of Hsp90 interaction with nascent CFTR prevents its maturation and accelerates its degradation by the proteasome
-
M.A. Loo, T.J. Jensen, L. Cui, Y.-X. Hou, X.-B. chang and J.R. Riordan (1998) Perturbation of Hsp90 interaction with nascent CFTR prevents its maturation and accelerates its degradation by the proteasome. EMBO J. 17 6879-6887.
-
(1998)
EMBO J.
, vol.17
, pp. 6879-6887
-
-
Loo, M.A.1
Jensen, T.J.2
Cui, L.3
Hou, Y.-X.4
chang, X.-B.5
Riordan, J.R.6
-
122
-
-
0034616637
-
Blockage of drug resistance in vitro by disulfiram, a drug used to treat alcoholism
-
T.W. Loo and D.M. Clarke (2000) Blockage of drug resistance in vitro by disulfiram, a drug used to treat alcoholism. J. Natl CancerInst. 92(11), 898-902.
-
(2000)
J. Natl CancerInst.
, vol.92
, Issue.11
, pp. 898-902
-
-
Loo, T.W.1
Clarke, D.M.2
-
123
-
-
0028559511
-
Confirmational maturation of CFTR but not its mutant counterpart (DF508) occurs in the endoplasmic reticulum and requires
-
G.L. Lukacs, A. Mohamed, N. Kartner, X.-B. Chang, J.R. Riordan and S. Grinstein (1994) Confirmational maturation of CFTR but not its mutant counterpart (DF508) occurs in the endoplasmic reticulum and requires. EMBO J. 13 6076-6086.
-
(1994)
EMBO J.
, vol.13
, pp. 6076-6086
-
-
Lukacs, G.L.1
Mohamed, A.2
Kartner, N.3
Chang, X.-B.4
Riordan, J.R.5
Grinstein, S.6
-
124
-
-
0031802827
-
Differential regulation of single CFTR channels by PP2C, PP2A, and other phosphatases
-
Pt 1
-
J. Luo, M.D. Pato, J.R. Riordan and J.W. Hanrahan (1998) Differential regulation of single CFTR channels by PP2C, PP2A, and other phosphatases. Am. J. Physiol. 274(5), C1397-C1410. Pt 1
-
(1998)
Am. J. Physiol.
, vol.274
, Issue.5
, pp. C1397-C1410
-
-
Luo, J.1
Pato, M.D.2
Riordan, J.R.3
Hanrahan, J.W.4
-
125
-
-
0031910471
-
Cystic fibrosis transmembrane conductance regulator (CFTR) anion binding as a probe of the pore
-
M.K. Mansoura, S.S. Smith, A.D. Choi, N.W. Richards, T.V. Strong, M.L. Drumm, F.S. Collins and D.C. Dawson (1998) Cystic fibrosis transmembrane conductance regulator (CFTR) anion binding as a probe of the pore. Biophys. J. 74(3), 1320-1332.
-
(1998)
Biophys. J.
, vol.74
, Issue.3
, pp. 1320-1332
-
-
Mansoura, M.K.1
Smith, S.S.2
Choi, A.D.3
Richards, N.W.4
Strong, T.V.5
Drumm, M.L.6
Collins, F.S.7
Dawson, D.C.8
-
126
-
-
0032079760
-
The CFTR chloride channel: nucleotide interactions and temperature-dependent gating
-
C.J. Mathews, J.A. Tabcharani and J.W. Hanrahan (1998) The CFTR chloride channel: nucleotide interactions and temperature-dependent gating. J. Membr. Biol. 163(1), 55-66.
-
(1998)
J. Membr. Biol.
, vol.163
, Issue.1
, pp. 55-66
-
-
Mathews, C.J.1
Tabcharani, J.A.2
Hanrahan, J.W.3
-
127
-
-
0032433707
-
Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease
-
H. Matsui, B.R. Grubb, R. Tarran, S.H. Randell, J.T. Gatzy, C.W. Davis and R.C. Boucher (1998) Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell 95(7), 1005-1015.
-
(1998)
Cell
, vol.95
, Issue.7
, pp. 1005-1015
-
-
Matsui, H.1
Grubb, B.R.2
Tarran, R.3
Randell, S.H.4
Gatzy, J.T.5
Davis, C.W.6
Boucher, R.C.7
-
128
-
-
0033898228
-
Permeation through the CFTR chloride channel
-
N.A. McCarty (2000) Permeation through the CFTR chloride channel. J. Exp. Biol. 203(Pt 13), 1947-1962.
-
(2000)
J. Exp. Biol.
, vol.203
, pp. 1947-1962
-
-
McCarty, N.A.1
-
129
-
-
0028111941
-
Novel pore-lining residues in CFTR that govern permeation and open-channel block
-
S. McDonough, N. Davidson, H.A. Lester and N.A. McCarty (1994) Novel pore-lining residues in CFTR that govern permeation and open-channel block. Neuron 13(3), 623-634.
-
(1994)
Neuron
, vol.13
, Issue.3
, pp. 623-634
-
-
McDonough, S.1
Davidson, N.2
Lester, H.A.3
McCarty, N.A.4
-
130
-
-
0033559258
-
The Hdj-2/Hsc 70 chaperone pair facilitates early steps in CFTR biogenesis
-
G.C. Meacham, Z. Lu, S. King, E. Sorscher, A. Tousson and D.M. Cyr (1999) The Hdj-2/Hsc 70 chaperone pair facilitates early steps in CFTR biogenesis. EMBO J. 18 1492-1505.
-
(1999)
EMBO J.
, vol.18
, pp. 1492-1505
-
-
Meacham, G.C.1
Lu, Z.2
King, S.3
Sorscher, E.4
Tousson, A.5
Cyr, D.M.6
-
131
-
-
0035142877
-
The Hsc70 co-chaperone CHIP targets immature CFTR for proteasonal degradation
-
G.C. Meacham, C. Patterson, W. Zhang, J.M. Younger and D.M. Cyr (2001) The Hsc70 co-chaperone CHIP targets immature CFTR for proteasonal degradation. Nat. Cell Biol. 3(1), 100-105.
-
(2001)
Nat. Cell Biol.
, vol.3
, Issue.1
, pp. 100-105
-
-
Meacham, G.C.1
Patterson, C.2
Zhang, W.3
Younger, J.M.4
Cyr, D.M.5
-
132
-
-
0031873373
-
− channel function in guinea pig ventricular myocytes
-
Pt 1
-
L.M. Middleton and R.D. Harvey (1998) PKC regulation of cardiac CFTR Cl− channel function in guinea pig ventricular myocytes. Am. J. Physiol. 275(1), C293-C302. Pt 1
-
(1998)
Am. J. Physiol.
, vol.275
, Issue.1
, pp. C293-C302
-
-
Middleton, L.M.1
Harvey, R.D.2
-
133
-
-
0035085578
-
A PDZ-binding motif is essential but not sufficient to localize the C terminus of CFTR to the apical membrane
-
M.I. Milewski, J.E. Mickle, J.K. Forrest, D.M. Stafford, B.D. Moyer, J. Cheng, W.B. Guggino, B.A. Stanton and G.R. Cutting (2001) A PDZ-binding motif is essential but not sufficient to localize the C terminus of CFTR to the apical membrane. J. Cell Sci. 114(Pt 4), 719-726.
-
(2001)
J. Cell Sci.
, vol.114
, pp. 719-726
-
-
Milewski, M.I.1
Mickle, J.E.2
Forrest, J.K.3
Stafford, D.M.4
Moyer, B.D.5
Cheng, J.6
Guggino, W.B.7
Stanton, B.A.8
Cutting, G.R.9
-
134
-
-
0029846003
-
Both the wild type and a functional isoform of CFTR are expressed in kidney
-
Pt 2
-
M.M. Morales, T.P. Carroll, T. Morita, E.M. Schwiebert, O. Devuyst, P.D. Wilson, et al. (1996) Both the wild type and a functional isoform of CFTR are expressed in kidney. Am. J. Physiol. 270(6), F1038-F1048. Pt 2
-
(1996)
Am. J. Physiol.
, vol.270
, Issue.6
, pp. F1038-F1048
-
-
Morales, M.M.1
Carroll, T.P.2
Morita, T.3
Schwiebert, E.M.4
Devuyst, O.5
Wilson, P.D.6
-
135
-
-
0034118221
-
Pharmacological chaperones rescue cell-surface expression and function of misfolded V2 vasopressin receptor mutants
-
J.P. Morello, A. Salahpour, A. Laperriere, V. Bernier, M.F. Arthus, M. Lonergan, et al. (2000) Pharmacological chaperones rescue cell-surface expression and function of misfolded V2 vasopressin receptor mutants. J. Clin. Invest. 105(7), 887-895.
-
(2000)
J. Clin. Invest.
, vol.105
, Issue.7
, pp. 887-895
-
-
Morello, J.P.1
Salahpour, A.2
Laperriere, A.3
Bernier, V.4
Arthus, M.F.5
Lonergan, M.6
-
136
-
-
0034625350
-
Nonequivalent nucleotide trapping in the two nucleotide binding folds of the human multidrug resistance protein MRP1
-
K. Nagata, M. Nishitani, M. Matsuo, N. Kioka, T. Amachi and K. Ueda (2000) Nonequivalent nucleotide trapping in the two nucleotide binding folds of the human multidrug resistance protein MRP1. J. Biol. Chem. 275(23), 17626-17630.
-
(2000)
J. Biol. Chem.
, vol.275
, Issue.23
, pp. 17626-17630
-
-
Nagata, K.1
Nishitani, M.2
Matsuo, M.3
Kioka, N.4
Amachi, T.5
Ueda, K.6
-
137
-
-
0032750221
-
Differential function of the two nucleotide binding domains on cystic fibrosis transmembrane conductance regulator
-
G. Nagel (1999) Differential function of the two nucleotide binding domains on cystic fibrosis transmembrane conductance regulator. Biochim. Biophys. Acta 1461(2), 263-274.
-
(1999)
Biochim. Biophys. Acta
, vol.1461
, Issue.2
, pp. 263-274
-
-
Nagel, G.1
-
138
-
-
0035065745
-
Non-specific activation of the epithelial sodium channel by the CFTR chloride channel
-
G. Nagel, T. Szellas, J.R. Riordan, T. Friedrich and K. Hartung (2001) Non-specific activation of the epithelial sodium channel by the CFTR chloride channel. EMBO Rep. 2(3), 249-254.
-
(2001)
EMBO Rep.
, vol.2
, Issue.3
, pp. 249-254
-
-
Nagel, G.1
Szellas, T.2
Riordan, J.R.3
Friedrich, T.4
Hartung, K.5
-
139
-
-
0344483932
-
Identification of sites in the R-domain of CFTR phosphorylated by cAMP-dependent protein kinase and dephosphorylated by protein phosphatases 2A and 2C
-
(Abstract)
-
A.C. Nairn, J. Qin, B.T. Chait and D.C. Gadsby (1996) Identification of sites in the R-domain of CFTR phosphorylated by cAMP-dependent protein kinase and dephosphorylated by protein phosphatases 2A and 2C. Pediatr. Pulm. (Suppl. 13), 211. (Abstract)
-
(1996)
Pediatr. Pulm.
, pp. 211
-
-
Nairn, A.C.1
Qin, J.2
Chait, B.T.3
Gadsby, D.C.4
-
140
-
-
0030732101
-
Regulation of CFTR chloride channels by syntaxin and Munc18 isoforms
-
A.P. Naren, D.J. Nelson, W. Xie, B. Jovov, J. Pevsner, M.K. Bennett, D.J. Benos, M.W. Quick and K.L. Kirk (1997) Regulation of CFTR chloride channels by syntaxin and Munc18 isoforms. Nature 390(6657), 302-305.
-
(1997)
Nature
, vol.390
, Issue.6657
, pp. 302-305
-
-
Naren, A.P.1
Nelson, D.J.2
Xie, W.3
Jovov, B.4
Pevsner, J.5
Bennett, M.K.6
Benos, D.J.7
Quick, M.W.8
Kirk, K.L.9
-
141
-
-
0032167764
-
Syntaxin 1A inhibits CFTR chloride channels by means of domain-specific protein-protein interactions
-
A.P. Naren, M.W. Quick, J.F. Collawn, D.J. Nelson and K.L. Kirk (1998) Syntaxin 1A inhibits CFTR chloride channels by means of domain-specific protein-protein interactions. Proc. Natl Acad. Sci. USA 95(18), 10972-10977.
-
(1998)
Proc. Natl Acad. Sci. USA
, vol.95
, Issue.18
, pp. 10972-10977
-
-
Naren, A.P.1
Quick, M.W.2
Collawn, J.F.3
Nelson, D.J.4
Kirk, K.L.5
-
142
-
-
0033569511
-
CFTR chloride channel regulation by a interdomain interaction
-
A.P. Naren, E. Cormet-Boyaka, J. Fu, M. Villain, J.E. Blalock, M.W. Quick and K.L. Kirk (1999) CFTR chloride channel regulation by a interdomain interaction. Science 286(5439), 544-548.
-
(1999)
Science
, vol.286
, Issue.5439
, pp. 544-548
-
-
Naren, A.P.1
Cormet-Boyaka, E.2
Fu, J.3
Villain, M.4
Blalock, J.E.5
Quick, M.W.6
Kirk, K.L.7
-
143
-
-
0013299189
-
CFTR associates with the P11 subunit of the annexin II heterotetramer
-
(abstract)
-
D.J. Nelson, et al. (2001) CFTR associates with the P11 subunit of the annexin II heterotetramer. Pediatr. Pulm. (Suppl. 18), 178. (abstract)
-
(2001)
Pediatr. Pulm.
, pp. 178
-
-
Nelson, D.J.1
-
144
-
-
0030667705
-
Evidence for phosphorylation of serine 753 in CFTR using a novel metal-ion affinity resin and matrix-assisted laser desorption mass spectrometry
-
D.C. Neville, C.R. Rozanas, E.M. Price, D.B. Gruis, A.S. Verkman and R.R. Townsend (1997) Evidence for phosphorylation of serine 753 in CFTR using a novel metal-ion affinity resin and matrix-assisted laser desorption mass spectrometry. Protein Sci. 6(11), 2436-2445.
-
(1997)
Protein Sci.
, vol.6
, Issue.11
, pp. 2436-2445
-
-
Neville, D.C.1
Rozanas, C.R.2
Price, E.M.3
Gruis, D.B.4
Verkman, A.S.5
Townsend, R.R.6
-
145
-
-
0032562143
-
Expression and characterization of the NBD1-R domain region of CFTR: evidence for subunit-subunit interactions
-
D.C. Neville, C.R. Rozanas, B.M. Tulk, R.R. Townsend and A.S. Verkman (1998) Expression and characterization of the NBD1-R domain region of CFTR: evidence for subunit-subunit interactions. Biochemistry 37(8), 2401-2409.
-
(1998)
Biochemistry
, vol.37
, Issue.8
, pp. 2401-2409
-
-
Neville, D.C.1
Rozanas, C.R.2
Tulk, B.M.3
Townsend, R.R.4
Verkman, A.S.5
-
146
-
-
0033018150
-
A di-acidic (DXE) code directs concentration of cargo during export from the endoplasmic reticulum
-
N. Nishimura, S. Bannykh, S. Slabough, J. Matteson, Y. Altschuler, K. Hahn and W.E. Balch (1999) A di-acidic (DXE) code directs concentration of cargo during export from the endoplasmic reticulum. J. Biol. Chem. 274(22), 15937-15946.
-
(1999)
J. Biol. Chem.
, vol.274
, Issue.22
, pp. 15937-15946
-
-
Nishimura, N.1
Bannykh, S.2
Slabough, S.3
Matteson, J.4
Altschuler, Y.5
Hahn, K.6
Balch, W.E.7
-
147
-
-
0035722764
-
CFTR-opathies': disease phenotypes associated with cystic fibrosis transmembrane regulator gene mutations
-
P.G. Noone and M.R. Knowles (2001) CFTR-opathies': disease phenotypes associated with cystic fibrosis transmembrane regulator gene mutations. Respir. Res. 2(6), 328-332.
-
(2001)
Respir. Res.
, vol.2
, Issue.6
, pp. 328-332
-
-
Noone, P.G.1
Knowles, M.R.2
-
148
-
-
0034036091
-
Cystic fibrosis transmembrane conductance regulator currents in guinea pig pancreatic duct cells: inhibition by bicarbonate ions
-
C.M. O'Reilly, J.P. Winpenny, B.E. Argent and M.A. Gray (2000) Cystic fibrosis transmembrane conductance regulator currents in guinea pig pancreatic duct cells: inhibition by bicarbonate ions. Gastroenterology 118(6), 1187-1196.
-
(2000)
Gastroenterology
, vol.118
, Issue.6
, pp. 1187-1196
-
-
O'Reilly, C.M.1
Winpenny, J.P.2
Argent, B.E.3
Gray, M.A.4
-
149
-
-
0027438374
-
Nasal epithelial ion transport and genetic analysis of infertile men with congenital bilateral absence of the vas deferens
-
L.R. Osborne, M. Lynch, P.G. Middleton, E.W. Alton, D.M. Geddes, J.P. Pryor, M.E. Hodson and G.K. Santis (1993) Nasal epithelial ion transport and genetic analysis of infertile men with congenital bilateral absence of the vas deferens. Hum. Mol. Genet. 2(10), 1605-1609.
-
(1993)
Hum. Mol. Genet.
, vol.2
, Issue.10
, pp. 1605-1609
-
-
Osborne, L.R.1
Lynch, M.2
Middleton, P.G.3
Alton, E.W.4
Geddes, D.M.5
Pryor, J.P.6
Hodson, M.E.7
Santis, G.K.8
-
150
-
-
0035896542
-
− channel by its R domain
-
L.S. Ostedgaard, O. Baldursson and M.J. Welsh (2001) Regulation of the cystic fibrosis transmembrane conductance regulator Cl− channel by its R domain. J. Biol. Chem. 276(11), 7689-7692.
-
(2001)
J. Biol. Chem.
, vol.276
, Issue.11
, pp. 7689-7692
-
-
Ostedgaard, L.S.1
Baldursson, O.2
Welsh, M.J.3
-
151
-
-
0026681083
-
Phosphorylation of the cystic fibrosis transmembrane conductance regulator
-
M.R. Picciotto, J.A. Cohn, G. Bertuzzi, P. Greengard and A.C. Nairn (1992) Phosphorylation of the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 267 12742-12752.
-
(1992)
J. Biol. Chem.
, vol.267
, pp. 12742-12752
-
-
Picciotto, M.R.1
Cohn, J.A.2
Bertuzzi, G.3
Greengard, P.4
Nairn, A.C.5
-
152
-
-
0029960243
-
CFTR gene variant IVS8-5T in disseminated bronchiectasis
-
P.F. Pignatti, C. Bombieri, M. Benetazzo, A. Casartelli, E. Trabetti, L.S. Gile, L.C. Martinati, A.L. Boner and M. Luisetti (1996) CFTR gene variant IVS8-5T in disseminated bronchiectasis. Am. J. Hum. Genet. 58(4), 889-892.
-
(1996)
Am. J. Hum. Genet.
, vol.58
, Issue.4
, pp. 889-892
-
-
Pignatti, P.F.1
Bombieri, C.2
Benetazzo, M.3
Casartelli, A.4
Trabetti, E.5
Gile, L.S.6
Martinati, L.C.7
Boner, A.L.8
Luisetti, M.9
-
153
-
-
0028232167
-
Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator
-
S. Pind, J.R. Riordan and D.B. Williams (1994) Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 269(12), 12784-12788.
-
(1994)
J. Biol. Chem.
, vol.269
, Issue.12
, pp. 12784-12788
-
-
Pind, S.1
Riordan, J.R.2
Williams, D.B.3
-
154
-
-
0028232830
-
Bicarbonate conductance and pH regulatory capability of cystic fibrosis transmembrane conductance regulator
-
J.H. Poulsen, H. Fischer, B. Illek and T.E. Machen (1994) Bicarbonate conductance and pH regulatory capability of cystic fibrosis transmembrane conductance regulator. Proc. Natl Acad. Sci. USA 91(12), 5340-5344.
-
(1994)
Proc. Natl Acad. Sci. USA
, vol.91
, Issue.12
, pp. 5340-5344
-
-
Poulsen, J.H.1
Fischer, H.2
Illek, B.3
Machen, T.E.4
-
155
-
-
0029816943
-
Function of Xenopus cystic fibrosis transmembrane conductance regulator (CFTR) CI channels and use of human-Xenopus chimeras to investigate the pore properties of CFTR
-
M.P. Price, H. Ishihara, D.N. Sheppard and M.J. Welsh (1996) Function of Xenopus cystic fibrosis transmembrane conductance regulator (CFTR) CI channels and use of human-Xenopus chimeras to investigate the pore properties of CFTR. J. Biol. Chem. 271(41), 25184-25191.
-
(1996)
J. Biol. Chem.
, vol.271
, Issue.41
, pp. 25184-25191
-
-
Price, M.P.1
Ishihara, H.2
Sheppard, D.N.3
Welsh, M.J.4
-
156
-
-
0029997424
-
Alteration of the cystic fibrosis transmembrane conductance regulator folding pathway
-
B.H. Qu and P.J. Thomas (1996) Alteration of the cystic fibrosis transmembrane conductance regulator folding pathway. J. Biol. Chem. 271(13), 7261-7264.
-
(1996)
J. Biol. Chem.
, vol.271
, Issue.13
, pp. 7261-7264
-
-
Qu, B.H.1
Thomas, P.J.2
-
157
-
-
0020700816
-
Chloride impermeability in cystic fibrosis
-
P.M. Quinton (1983) Chloride impermeability in cystic fibrosis. Nature 301 421-422.
-
(1983)
Nature
, vol.301
, pp. 421-422
-
-
Quinton, P.M.1
-
158
-
-
0034745438
-
3
-
P.M. Quinton (2001) The neglected ion: HCO3. Nat. Med. 7(3), 292-293.
-
(2001)
Nat. Med.
, vol.7
, Issue.3
, pp. 292-293
-
-
Quinton, P.M.1
-
159
-
-
0020533435
-
Higher bioelectric potentials due to decreased chloride absorption in the sweat glands of patients with cystic fibrosis
-
P.M. Quinton and J. Bijman (1983) Higher bioelectric potentials due to decreased chloride absorption in the sweat glands of patients with cystic fibrosis. N. Engl. J. Med. 308 1185-1189.
-
(1983)
N. Engl. J. Med.
, vol.308
, pp. 1185-1189
-
-
Quinton, P.M.1
Bijman, J.2
-
160
-
-
0026437322
-
Control of CFTR chloride conductance by ATP levels through non-hydrolytic binding
-
P.M. Quinton and M.M. Reddy (1992) Control of CFTR chloride conductance by ATP levels through non-hydrolytic binding. Nature 360(6399), 79-81.
-
(1992)
Nature
, vol.360
, Issue.6399
, pp. 79-81
-
-
Quinton, P.M.1
Reddy, M.M.2
-
161
-
-
0035970113
-
Regulation of cystic fibrosis transmembrane conductance regulator single-channel gating by bivalent PDZ-domain-mediated interaction
-
V. Raghuram, D.D. Mak and J.K. Foskett (2001) Regulation of cystic fibrosis transmembrane conductance regulator single-channel gating by bivalent PDZ-domain-mediated interaction. Proc. Natl Acad. Sci. USA 98(3), 1300-1305.
-
(2001)
Proc. Natl Acad. Sci. USA
, vol.98
, Issue.3
, pp. 1300-1305
-
-
Raghuram, V.1
Mak, D.D.2
Foskett, J.K.3
-
162
-
-
0033514315
-
Walker mutations reveal loose relationship between catalytic and channel-gating activities of purified CFTR (cystic fibrosis transmembrane conductance regulator)
-
M. Ramjeesingh, C. Li, E. Garami, L.J. Huan, K. Galley, Y. Wang and C.E. Bear (1999) Walker mutations reveal loose relationship between catalytic and channel-gating activities of purified CFTR (cystic fibrosis transmembrane conductance regulator). Biochemistry 38(5), 1463-1468.
-
(1999)
Biochemistry
, vol.38
, Issue.5
, pp. 1463-1468
-
-
Ramjeesingh, M.1
Li, C.2
Garami, E.3
Huan, L.J.4
Galley, K.5
Wang, Y.6
Bear, C.E.7
-
163
-
-
0035807019
-
A monomer is the minimum functional unit required for channel and ATPase activity of the cystic fibrosis transmembrane conductance regulator
-
M. Ramjeesingh, C. Li, I. Kogan, Y. Wang, L.J. Huan and C.E. Bear (2001) A monomer is the minimum functional unit required for channel and ATPase activity of the cystic fibrosis transmembrane conductance regulator. Biochemistry 40(35), 10700-10706.
-
(2001)
Biochemistry
, vol.40
, Issue.35
, pp. 10700-10706
-
-
Ramjeesingh, M.1
Li, C.2
Kogan, I.3
Wang, Y.4
Huan, L.J.5
Bear, C.E.6
-
164
-
-
0028923471
-
Expression and functional properties of the second predicted nucleotide binding fold of CFTR fused to GST
-
C. Randak, A.A. Roscher, H.B. Hadorn, I. Assfalg-Machleidt, E.A. Auerswald and W. Machleidt (1995) Expression and functional properties of the second predicted nucleotide binding fold of CFTR fused to GST. FEBS Lett. 363 189-194.
-
(1995)
FEBS Lett.
, vol.363
, pp. 189-194
-
-
Randak, C.1
Roscher, A.A.2
Hadorn, H.B.3
Assfalg-Machleidt, I.4
Auerswald, E.A.5
Machleidt, W.6
-
165
-
-
0030602191
-
A recombinant polypeptide model of the second predicted nucleotide binding fold of the cystic fibrosis transmembrane conductance regulator is a GTP-binding protein
-
C. Randak, P. Neth, E.A. Auerswald, I. Assfalg-Machleidt, A.A. Roscher, H.B. Hadorn and W. Machleidt (1996) A recombinant polypeptide model of the second predicted nucleotide binding fold of the cystic fibrosis transmembrane conductance regulator is a GTP-binding protein. FEBS Lett. 398(1), 97-100.
-
(1996)
FEBS Lett.
, vol.398
, Issue.1
, pp. 97-100
-
-
Randak, C.1
Neth, P.2
Auerswald, E.A.3
Assfalg-Machleidt, I.4
Roscher, A.A.5
Hadorn, H.B.6
Machleidt, W.7
-
166
-
-
0030806301
-
A recombinant polypeptide model of the second nucleotide-binding fold of the cystic fibrosis transmembrane conductance regulator functions as an active ATPase, GTPase and adenylate kinase
-
C. Randak, P. Neth, E.A. Auerswald, C. Eckerskorn, I. Assfalg-Machleidt and W. Machleidt (1997) A recombinant polypeptide model of the second nucleotide-binding fold of the cystic fibrosis transmembrane conductance regulator functions as an active ATPase, GTPase and adenylate kinase. FEBS Lett. 410(2–3), 180-186.
-
(1997)
FEBS Lett.
, vol.410
, Issue.2-3
, pp. 180-186
-
-
Randak, C.1
Neth, P.2
Auerswald, E.A.3
Eckerskorn, C.4
Assfalg-Machleidt, I.5
Machleidt, W.6
-
167
-
-
0033581885
-
− channel function
-
M.M. Reddy, M.J. Light and P.M. Quinton (1999) Activation of the epithelial Na+ channel (ENaC) requires CFTR Cl− channel function. Nature 402(6759), 301-304.
-
(1999)
Nature
, vol.402
, Issue.6759
, pp. 301-304
-
-
Reddy, M.M.1
Light, M.J.2
Quinton, P.M.3
-
168
-
-
0033361889
-
Cystic fibrosis as a disease of misprocessing of the cystic fibrosis transmembrane conductance regulator glycoprotein
-
J.R. Riordan (1999) Cystic fibrosis as a disease of misprocessing of the cystic fibrosis transmembrane conductance regulator glycoprotein. Am. J. Hum. Genet. 64(6), 1499-1504.
-
(1999)
Am. J. Hum. Genet.
, vol.64
, Issue.6
, pp. 1499-1504
-
-
Riordan, J.R.1
-
169
-
-
0024424270
-
Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA
-
J.R. Riordan, J.M. Rommens, B. Kerem, N. Alon, R. Rozmahel, Z. Grzelczak, et al. (1989) Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 245(4922), 1066-1073.
-
(1989)
Science
, vol.245
, Issue.4922
, pp. 1066-1073
-
-
Riordan, J.R.1
Rommens, J.M.2
Kerem, B.3
Alon, N.4
Rozmahel, R.5
Grzelczak, Z.6
-
170
-
-
0034031279
-
Recognition of local glycoprotein misfolding by the ER folding sensor UDP-glucose:glycoprotein glucosyltransferase
-
C. Ritter and A. Helenius (2000) Recognition of local glycoprotein misfolding by the ER folding sensor UDP-glucose:glycoprotein glucosyltransferase. Nat. Struct. Biol. 7(4), 278-280.
-
(2000)
Nat. Struct. Biol.
, vol.7
, Issue.4
, pp. 278-280
-
-
Ritter, C.1
Helenius, A.2
-
171
-
-
0030971840
-
Structure of the multidrug resistance P-glycoprotein to 2.5 nm resolution determined by electron microscopy and image analysis
-
M.F. Rosenberg, R. Callaghan, R.C. Ford and C.F. Higgins (1997) Structure of the multidrug resistance P-glycoprotein to 2.5 nm resolution determined by electron microscopy and image analysis. J. Biol. Chem. 272(16), 10685-10694.
-
(1997)
J. Biol. Chem.
, vol.272
, Issue.16
, pp. 10685-10694
-
-
Rosenberg, M.F.1
Callaghan, R.2
Ford, R.C.3
Higgins, C.F.4
-
172
-
-
0035844237
-
The structure of the multidrug resistance protein 1 (MRP1/ABCC1) crystallization and single-particle analysis
-
M.F. Rosenberg, Q. Mao, A. Holzenburg, R.C. Ford, R.G. Deeley and S.P. Cole (2001) The structure of the multidrug resistance protein 1 (MRP1/ABCC1) crystallization and single-particle analysis. J. Biol. Chem. 276(19), 16076-16082.
-
(2001)
J. Biol. Chem.
, vol.276
, Issue.19
, pp. 16076-16082
-
-
Rosenberg, M.F.1
Mao, Q.2
Holzenburg, A.3
Ford, R.C.4
Deeley, R.G.5
Cole, S.P.6
-
173
-
-
0034099743
-
Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of Delta F508-CFTR
-
R.C. Rubenstein and P.L. Zeitlin (2000) Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of Delta F508-CFTR. Am. J. Physiol. Cell Physiol. 278(2), C259-C267.
-
(2000)
Am. J. Physiol. Cell Physiol.
, vol.278
, Issue.2
, pp. C259-C267
-
-
Rubenstein, R.C.1
Zeitlin, P.L.2
-
174
-
-
0032571366
-
Cotranslational ubiquitination of cystic fibrosis transmembrane conductance regulator in vitro
-
S. Sato, C.L. Ward and R.R. Kopito (1998) Cotranslational ubiquitination of cystic fibrosis transmembrane conductance regulator in vitro. J. Biol. Chem. 273(13), 7189-7192.
-
(1998)
J. Biol. Chem.
, vol.273
, Issue.13
, pp. 7189-7192
-
-
Sato, S.1
Ward, C.L.2
Kopito, R.R.3
-
175
-
-
0032951188
-
Getting in or out: early segregation between importers and exporters in the evolution of ATP-binding cassette (ABC) transporters
-
W. Saurin, M. Hofnung and E. Dassa (1999) Getting in or out: early segregation between importers and exporters in the evolution of ATP-binding cassette (ABC) transporters. J. Mol. Evol. 48(1), 22-41.
-
(1999)
J. Mol. Evol.
, vol.48
, Issue.1
, pp. 22-41
-
-
Saurin, W.1
Hofnung, M.2
Dassa, E.3
-
176
-
-
0023546389
-
Phosphorylation fails to activate chloride channels from cystic fibrosis airway cells
-
R.A. Schoumacher, R.L. Shoemaker, D.R. Halm, E.A. Tallant, R.W. Wallace and R.A. Frizzell (1987) Phosphorylation fails to activate chloride channels from cystic fibrosis airway cells. Nature 330 752-754.
-
(1987)
Nature
, vol.330
, pp. 752-754
-
-
Schoumacher, R.A.1
Shoemaker, R.L.2
Halm, D.R.3
Tallant, E.A.4
Wallace, R.W.5
Frizzell, R.A.6
-
177
-
-
0029883258
-
Lack of conventional ATPase properties in CFTR chloride channel gating
-
B.D. Schultz, R.J. Bridges and R.A. Frizzell (1996) Lack of conventional ATPase properties in CFTR chloride channel gating. J. Membr. Biol. 151(1), 63-75.
-
(1996)
J. Membr. Biol.
, vol.151
, Issue.1
, pp. 63-75
-
-
Schultz, B.D.1
Bridges, R.J.2
Frizzell, R.A.3
-
178
-
-
0032940125
-
Pharmacology of CFTR chloride channel activity
-
B.D. Schultz, A.K. Singh, D.C. Devor and R.J. Bridges (1999) Pharmacology of CFTR chloride channel activity. Physiol. Rev. 79(1 Suppl.), S109-S144.
-
(1999)
Physiol. Rev.
, vol.79
, Issue.1
, pp. S109-S144
-
-
Schultz, B.D.1
Singh, A.K.2
Devor, D.C.3
Bridges, R.J.4
-
179
-
-
0032956071
-
CFTR is a conductance regulator as well as a chloride channel
-
E.M. Schweibert, D.J. Benos, M.E. Egan, M.J. Stutts and W.B. Guggino (1999) CFTR is a conductance regulator as well as a chloride channel. Physiol. Rev. 79 145.
-
(1999)
Physiol. Rev.
, vol.79
, pp. 145
-
-
Schweibert, E.M.1
Benos, D.J.2
Egan, M.E.3
Stutts, M.J.4
Guggino, W.B.5
-
180
-
-
0028906638
-
cAMP-dependent protein kinase-mediated phosphorylation of cystic fibrosis transmembrane conductance regulator residue ser-753 and its role in channel activation
-
F.S. Seibert, J.A. Tabcharani, X.-B. Chang, A.M. Dulhanty, C. Mathews, J.W. Hanrahan and J.R. Riordan (1995) cAMP-dependent protein kinase-mediated phosphorylation of cystic fibrosis transmembrane conductance regulator residue ser-753 and its role in channel activation. J. Biol. Chem. 270(5), 2158-2162.
-
(1995)
J. Biol. Chem.
, vol.270
, Issue.5
, pp. 2158-2162
-
-
Seibert, F.S.1
Tabcharani, J.A.2
Chang, X.-B.3
Dulhanty, A.M.4
Mathews, C.5
Hanrahan, J.W.6
Riordan, J.R.7
-
181
-
-
17544374096
-
Disease-associated mutations in the fourth cytoplasmic loop of CFTR compromise biosynthetic processing and chloride channel activity
-
F.S. Seibert, P. Linsdell, T.W. Loo, J.W. Hanrahan, D.M. Clarke and J.R. Riordan (1996) Disease-associated mutations in the fourth cytoplasmic loop of CFTR compromise biosynthetic processing and chloride channel activity. J. Biol. Chem. 271 15139-15143.
-
(1996)
J. Biol. Chem.
, vol.271
, pp. 15139-15143
-
-
Seibert, F.S.1
Linsdell, P.2
Loo, T.W.3
Hanrahan, J.W.4
Clarke, D.M.5
Riordan, J.R.6
-
182
-
-
0029910820
-
Cytoplasmic loop three of cystic fibrosis transmembrane conductance regulator contributes to regulation of chloride channel activity
-
F.S. Seibert, P. Linsdell, T.W. Loo, J.W. Hanrahan, J.R. Riordan and D.M. Clarke (1996) Cytoplasmic loop three of cystic fibrosis transmembrane conductance regulator contributes to regulation of chloride channel activity. J. Biol. Chem. 271(44), 27493-27499.
-
(1996)
J. Biol. Chem.
, vol.271
, Issue.44
, pp. 27493-27499
-
-
Seibert, F.S.1
Linsdell, P.2
Loo, T.W.3
Hanrahan, J.W.4
Riordan, J.R.5
Clarke, D.M.6
-
183
-
-
0030775639
-
Disease-associated mutations in cytoplasmic loops 1 and 2 of cystic fibrosis transmembrane conductance regulator impede processing or opening of the channel
-
F.S. Seibert, Y. Jia, C.J. Mathews, J.W. Hanrahan, J.R. Riordan, T.W. Loo and D.M. Clarke (1997) Disease-associated mutations in cytoplasmic loops 1 and 2 of cystic fibrosis transmembrane conductance regulator impede processing or opening of the channel. Biochemistry 36(39), 11966-11974.
-
(1997)
Biochemistry
, vol.36
, Issue.39
, pp. 11966-11974
-
-
Seibert, F.S.1
Jia, Y.2
Mathews, C.J.3
Hanrahan, J.W.4
Riordan, J.R.5
Loo, T.W.6
Clarke, D.M.7
-
184
-
-
0031156388
-
ATP hydrolysis cycles and mechanism in P-glycoprotein and CFTR
-
A.E. Senior and D.C. Gadsby (1997) ATP hydrolysis cycles and mechanism in P-glycoprotein and CFTR. Semin. Cancer Biol. 8(3), 143-150.
-
(1997)
Semin. Cancer Biol.
, vol.8
, Issue.3
, pp. 143-150
-
-
Senior, A.E.1
Gadsby, D.C.2
-
185
-
-
0032480253
-
Mutations of the cystic fibrosis gene in patients with chronic pancreatitis
-
N. Sharer, M. Schwarz, G. Malone, A. Howarth, J. Painter, M. Super and J. Braganza (1998) Mutations of the cystic fibrosis gene in patients with chronic pancreatitis. N. Engl. J. Med. 339(10), 645-652.
-
(1998)
N. Engl. J. Med.
, vol.339
, Issue.10
, pp. 645-652
-
-
Sharer, N.1
Schwarz, M.2
Malone, G.3
Howarth, A.4
Painter, J.5
Super, M.6
Braganza, J.7
-
186
-
-
0032912589
-
Structure and function of the CFTR chloride channel
-
D.N. Sheppard and M.J. Welsh (1999) Structure and function of the CFTR chloride channel. Physiol. Rev. 79(1 Suppl.), S23-S45.
-
(1999)
Physiol. Rev.
, vol.79
, Issue.1
, pp. S23-S45
-
-
Sheppard, D.N.1
Welsh, M.J.2
-
187
-
-
0027408231
-
− channels with altered pore properties
-
D.N. Sheppard, D.P. Rich, L.S. Ostedgaard, R.J. Gregory, A.E. Smith and M.J. Welsh (1993) Mutations in CFTR associated with mild-disease-form Cl− channels with altered pore properties. Nature 362(6416), 160-164.
-
(1993)
Nature
, vol.362
, Issue.6416
, pp. 160-164
-
-
Sheppard, D.N.1
Rich, D.P.2
Ostedgaard, L.S.3
Gregory, R.J.4
Smith, A.E.5
Welsh, M.J.6
-
188
-
-
0028223850
-
− channel
-
D.N. Sheppard, L.S. Ostedgaard, D.P. Rich and M.J. Welsh (1994) The amino-terminal portion of CFTR forms a regulated Cl− channel. Cell 76(6), 1091-1098.
-
(1994)
Cell
, vol.76
, Issue.6
, pp. 1091-1098
-
-
Sheppard, D.N.1
Ostedgaard, L.S.2
Rich, D.P.3
Welsh, M.J.4
-
189
-
-
0032584744
-
An apical PDZ protein anchors the cystic fibrosis transmembrane conductance regulator to the cytoskeleton
-
D.B. Short, K.W. Trotter, D. Reczek, S.M. Kreda, A. Bretscher, R.C. Boucher, M.J. Stutts and S.L. Milgram (1998) An apical PDZ protein anchors the cystic fibrosis transmembrane conductance regulator to the cytoskeleton. J. Biol. Chem. 273(31), 19797-19801.
-
(1998)
J. Biol. Chem.
, vol.273
, Issue.31
, pp. 19797-19801
-
-
Short, D.B.1
Trotter, K.W.2
Reczek, D.3
Kreda, S.M.4
Bretscher, A.5
Boucher, R.C.6
Stutts, M.J.7
Milgram, S.L.8
-
190
-
-
0029870085
-
Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid
-
[published erratum appears in Cell 1996 Oct 18; 87(2), following 355]
-
J.J. Smith, S.M. Travis, E.P. Greenberg and M.J. Welsh (1996) Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid. Cell 85(2), 229-236. [published erratum appears in Cell 1996 Oct 18; 87(2), following 355]
-
(1996)
Cell
, vol.85
, Issue.2
, pp. 229-236
-
-
Smith, J.J.1
Travis, S.M.2
Greenberg, E.P.3
Welsh, M.J.4
-
191
-
-
0032893407
-
X-linked adrenoleukodystrophy: genes, mutations and phenotypes
-
K.D. Smith, S. Kemp, L.T. Braiterman, J.F. Lu, H.M. Wei, M. Geraghty, G. Stetten, J.S. Bergin, J. Pevsner and P.A. Watkins (1999) X-linked adrenoleukodystrophy: genes, mutations and phenotypes. Neurochem. Res. 24(4), 521-535.
-
(1999)
Neurochem. Res.
, vol.24
, Issue.4
, pp. 521-535
-
-
Smith, K.D.1
Kemp, S.2
Braiterman, L.T.3
Lu, J.F.4
Wei, H.M.5
Geraghty, M.6
Stetten, G.7
Bergin, J.S.8
Pevsner, J.9
Watkins, P.A.10
-
192
-
-
0034774836
-
CFTR: covalent and noncovalent modification suggests a role for fixed charges in anion conduction
-
S.S. Smith, X. Liu, Z.R. Zhang, F. Sun, T.E. Kriewall, N.A. McCarty and D.C. Dawson (2001) CFTR: covalent and noncovalent modification suggests a role for fixed charges in anion conduction. J. Gen. Physiol. 118(4), 407-431.
-
(2001)
J. Gen. Physiol.
, vol.118
, Issue.4
, pp. 407-431
-
-
Smith, S.S.1
Liu, X.2
Zhang, Z.R.3
Sun, F.4
Kriewall, T.E.5
McCarty, N.A.6
Dawson, D.C.7
-
193
-
-
0020082418
-
Quantitative evaluation of the development of tracheal submucosal glands in infants with cystic fibrosis and control infants
-
J. Sturgess and J. Imrie (1982) Quantitative evaluation of the development of tracheal submucosal glands in infants with cystic fibrosis and control infants. Am. J. Pathol. 106(3), 303-311.
-
(1982)
Am. J. Pathol.
, vol.106
, Issue.3
, pp. 303-311
-
-
Sturgess, J.1
Imrie, J.2
-
194
-
-
0028982894
-
CFTR as a cAMP-dependent regulator of sodium channels
-
M.J. Stutts, C.M. Canessa, J.C. Olsen, M. Hamrick, J.A. Cohn, B.C. Rossier and R.C. Boucher (1995) CFTR as a cAMP-dependent regulator of sodium channels. Science 269 847-850.
-
(1995)
Science
, vol.269
, pp. 847-850
-
-
Stutts, M.J.1
Canessa, C.M.2
Olsen, J.C.3
Hamrick, M.4
Cohn, J.A.5
Rossier, B.C.6
Boucher, R.C.7
-
195
-
-
0039550861
-
Nucleotide occlusion in the human cystic fibrosis transmembrane conductance regulator. Different patterns in the two nucleotide binding domains
-
K. Szabo, G. Szakacs, T. Hegeds and B. Sarkadi (1999) Nucleotide occlusion in the human cystic fibrosis transmembrane conductance regulator. Different patterns in the two nucleotide binding domains. J. Biol. Chem. 274(18), 12209-12212.
-
(1999)
J. Biol. Chem.
, vol.274
, Issue.18
, pp. 12209-12212
-
-
Szabo, K.1
Szakacs, G.2
Hegeds, T.3
Sarkadi, B.4
-
196
-
-
0025002910
-
Low conductance chloride channel activated by cAMP in the epithelial cell line T84
-
J.A. Tabcharani, D. Low, D. Elie and J.W. Hanrahan (1990) Low conductance chloride channel activated by cAMP in the epithelial cell line T84. FEBS Lett. 270 157-164.
-
(1990)
FEBS Lett.
, vol.270
, pp. 157-164
-
-
Tabcharani, J.A.1
Low, D.2
Elie, D.3
Hanrahan, J.W.4
-
197
-
-
0025885376
-
− channel in CHO cells stably expressing the cystic fibrosis gene
-
J.A. Tabcharani, X.-B. Chang, J.R. Riordan and J.W. Hanrahan (1991) Phosphorylation-regulated Cl− channel in CHO cells stably expressing the cystic fibrosis gene. Nature 352 628-631.
-
(1991)
Nature
, vol.352
, pp. 628-631
-
-
Tabcharani, J.A.1
Chang, X.-B.2
Riordan, J.R.3
Hanrahan, J.W.4
-
198
-
-
0026629383
-
The cystic fibrosis transmembrane conductance regulator chloride channel: Iodide block and permeation
-
J.A. Tabcharani, X.-B. Chang, J.R. Riordan and J.W. Hanrahan (1992) The cystic fibrosis transmembrane conductance regulator chloride channel: Iodide block and permeation. Biophys. J. 62 1-4.
-
(1992)
Biophys. J.
, vol.62
, pp. 1-4
-
-
Tabcharani, J.A.1
Chang, X.-B.2
Riordan, J.R.3
Hanrahan, J.W.4
-
199
-
-
0027423190
-
Multi-ion pore behaviour in the CFTR chloride channel
-
J.A. Tabcharani, J.M. Rommens, Y.X. Hou, X.B. Chang, L.C. Tsui, J.R. Riordan and J.W. Hanrahan (1993) Multi-ion pore behaviour in the CFTR chloride channel. Nature 366(6450), 79-82.
-
(1993)
Nature
, vol.366
, Issue.6450
, pp. 79-82
-
-
Tabcharani, J.A.1
Rommens, J.M.2
Hou, Y.X.3
Chang, X.B.4
Tsui, L.C.5
Riordan, J.R.6
Hanrahan, J.W.7
-
200
-
-
0034952420
-
Interhelical hydrogen bonds in the CFTR membrane domain
-
A.G. Therien, F.E. Grant and C.M. Deber (2001) Interhelical hydrogen bonds in the CFTR membrane domain. Nat. Struct. Biol. 8(7), 597-601.
-
(2001)
Nat. Struct. Biol.
, vol.8
, Issue.7
, pp. 597-601
-
-
Therien, A.G.1
Grant, F.E.2
Deber, C.M.3
-
201
-
-
0034765259
-
A snapshot of Nature's favorite pump
-
P.J. Thomas and J.F. Hunt (2001) A snapshot of Nature's favorite pump. Nat. Struct. Biol. 8(11), 920-923.
-
(2001)
Nat. Struct. Biol.
, vol.8
, Issue.11
, pp. 920-923
-
-
Thomas, P.J.1
Hunt, J.F.2
-
202
-
-
0027179469
-
Interaction of nucleotides with membrane-associated cystic fibrosis transmembrane conductance regulator
-
S.M. Travis, M.R. Carson, D.R. Ries and M.J. Welsh (1993) Interaction of nucleotides with membrane-associated cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 268(21), 15336-15339.
-
(1993)
J. Biol. Chem.
, vol.268
, Issue.21
, pp. 15336-15339
-
-
Travis, S.M.1
Carson, M.R.2
Ries, D.R.3
Welsh, M.J.4
-
203
-
-
0030967834
-
Protein phosphatase 2C dephosphorylates and inactivates cystic fibrosis transmembrane conductance regulator
-
S.M. Travis, H.A. Berger and M.J. Welsh (1997) Protein phosphatase 2C dephosphorylates and inactivates cystic fibrosis transmembrane conductance regulator. Proc. Natl Acad. Sci. USA 94(20), 11055-11060.
-
(1997)
Proc. Natl Acad. Sci. USA
, vol.94
, Issue.20
, pp. 11055-11060
-
-
Travis, S.M.1
Berger, H.A.2
Welsh, M.J.3
-
204
-
-
0029124166
-
P-glycoprotein is stably inhibited by vanadate-induced trapping of nucleotide at a single catalytic site
-
I.L. Urbatsch, B. Sankaran, J. Weber and A.E. Senior (1995) P-glycoprotein is stably inhibited by vanadate-induced trapping of nucleotide at a single catalytic site. J. Biol. Chem. 270(33), 19383-19390.
-
(1995)
J. Biol. Chem.
, vol.270
, Issue.33
, pp. 19383-19390
-
-
Urbatsch, I.L.1
Sankaran, B.2
Weber, J.3
Senior, A.E.4
-
205
-
-
0034730330
-
Accessory protein facilitated CFTR-CFTR interaction, a molecular mechanism to potentiate the chloride channel activity
-
S. Wang, H. Yue, R.B. Derin, W.B. Guggino and M. Li (2000) Accessory protein facilitated CFTR-CFTR interaction, a molecular mechanism to potentiate the chloride channel activity. Cell 103(1), 169-179.
-
(2000)
Cell
, vol.103
, Issue.1
, pp. 169-179
-
-
Wang, S.1
Yue, H.2
Derin, R.B.3
Guggino, W.B.4
Li, M.5
-
206
-
-
0028840915
-
Degradation of CFTR by the ubiquitin-proteasome pathway
-
C.L. Ward, S. Omura and R.R. Kopito (1995) Degradation of CFTR by the ubiquitin-proteasome pathway. Cell 83 121-127.
-
(1995)
Cell
, vol.83
, pp. 121-127
-
-
Ward, C.L.1
Omura, S.2
Kopito, R.R.3
-
207
-
-
0035824532
-
Mu 2 binding directs the cystic fibrosis transmembrane conductance regulator to the clathrin-mediated endocytic pathway
-
K.M. Weixel and N.A. Bradbury (2001) Mu 2 binding directs the cystic fibrosis transmembrane conductance regulator to the clathrin-mediated endocytic pathway. J. Biol. Chem. 276(49), 46251-46259.
-
(2001)
J. Biol. Chem.
, vol.276
, Issue.49
, pp. 46251-46259
-
-
Weixel, K.M.1
Bradbury, N.A.2
-
208
-
-
0032542093
-
Structural biology. The ABC of a versatile engine
-
M.J. Welsh, A.D. Robertson and L.S. Ostegaard (1998) Structural biology. The ABC of a versatile engine. Nature 396(6712), 623-624.
-
(1998)
Nature
, vol.396
, Issue.6712
, pp. 623-624
-
-
Welsh, M.J.1
Robertson, A.D.2
Ostegaard, L.S.3
-
209
-
-
0030816586
-
CFTR activation: additive effects of stimulatory and inhibitory phosphorylation sites in the R domain
-
D.J. Wilkinson, T.V. Strong, M.K. Mansoura, D.L. Wood, S.S. Smith, F.S. Collins and D.C. Dawson (1997) CFTR activation: additive effects of stimulatory and inhibitory phosphorylation sites in the R domain. Am. J. Physiol. 273(1 Pt 1), L127-L133.
-
(1997)
Am. J. Physiol.
, vol.273
, Issue.1
, pp. L127-L133
-
-
Wilkinson, D.J.1
Strong, T.V.2
Mansoura, M.K.3
Wood, D.L.4
Smith, S.S.5
Collins, F.S.6
Dawson, D.C.7
-
210
-
-
0024547744
-
Shunt resistance and ion permeabilities in normal and cystic fibrosis airway epithelia
-
N.J. Willumsen and R.C. Boucher (1989) Shunt resistance and ion permeabilities in normal and cystic fibrosis airway epithelia. Am. J. Physiol. 256 C1054-C1063.
-
(1989)
Am. J. Physiol.
, vol.256
, pp. C1054-C1063
-
-
Willumsen, N.J.1
Boucher, R.C.2
-
211
-
-
0032999730
-
Regulation of recombinant cardiac cystic fibrosis transmembrane conductance regulator chloride channels by protein kinase C
-
J. Yamazaki, F. Britton, M.L. Collier, B. Horowitz and J.R. Hume (1999) Regulation of recombinant cardiac cystic fibrosis transmembrane conductance regulator chloride channels by protein kinase C. Biophys. J. 76(4), 1972-1987.
-
(1999)
Biophys. J.
, vol.76
, Issue.4
, pp. 1972-1987
-
-
Yamazaki, J.1
Britton, F.2
Collier, M.L.3
Horowitz, B.4
Hume, J.R.5
-
212
-
-
0030021584
-
Molecular basis of charge movement in voltage-gated sodium channels
-
N. Yang, A.L. George Jr. and R. Horn (1996) Molecular basis of charge movement in voltage-gated sodium channels. Neuron 16(1), 113-122.
-
(1996)
Neuron
, vol.16
, Issue.1
, pp. 113-122
-
-
Yang, N.1
George, A.L.2
Horn, R.3
-
213
-
-
0027488993
-
The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment
-
Y. Yang, S. Janich, J.A. Cohn and J.M. Wilson (1993) The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment. Proc. Natl Acad. Sci. USA 90 9480-9484.
-
(1993)
Proc. Natl Acad. Sci. USA
, vol.90
, pp. 9480-9484
-
-
Yang, Y.1
Janich, S.2
Cohn, J.A.3
Wilson, J.M.4
-
214
-
-
0037192856
-
Non-conventional trafficking of the cystic fibrosis transmembrane conductance regulator (CFTR) through the early secretory pathway
-
J.-S. Yoo, B.D. Moyer, S.I. Bannykh, H.-M. Yoo, J.R. Riordan and W.E. Balch (2002) Non-conventional trafficking of the cystic fibrosis transmembrane conductance regulator (CFTR) through the early secretory pathway. J. Biol. Chem. 277 1140-1149.
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 1140-1149
-
-
Yoo, J.-S.1
Moyer, B.D.2
Bannykh, S.I.3
Yoo, H.-M.4
Riordan, J.R.5
Balch, W.E.6
-
215
-
-
0034616134
-
The two halves of CFTR form a dual-pore ion channel
-
H. Yue, S. Devidas and W.B. Guggino (2000) The two halves of CFTR form a dual-pore ion channel. J. Biol. Chem. 275(14), 10030-10034.
-
(2000)
J. Biol. Chem.
, vol.275
, Issue.14
, pp. 10030-10034
-
-
Yue, H.1
Devidas, S.2
Guggino, W.B.3
-
216
-
-
0033936843
-
Pharmacologic restoration of delta F508 CFTR-mediated chloride current
-
P.L. Zeitlin (2000) Pharmacologic restoration of delta F508 CFTR-mediated chloride current. Kidney Int. 57(3), 832-837.
-
(2000)
Kidney Int.
, vol.57
, Issue.3
, pp. 832-837
-
-
Zeitlin, P.L.1
-
217
-
-
0033103174
-
ATP channels
-
N. Zerangue, B. Schwappach, Y.N. Jan and L.Y. Jan (1999) A new ER trafficking signal regulates the subunit stoichiometry of plasma membrane KATP channels. Neuron 22 537-548.
-
(1999)
Neuron
, vol.22
, pp. 537-548
-
-
Zerangue, N.1
Schwappach, B.2
Jan, Y.N.3
Jan, L.Y.4
-
218
-
-
0033583055
-
A single conductance pore for chloride ions formed by two cystic fibrosis transmembrane conductance regulator molecules
-
B. Zerhusen, J. Xie, P.B. Davis and J. Ma (1999) A single conductance pore for chloride ions formed by two cystic fibrosis transmembrane conductance regulator molecules. J. Biol. Chem. 274(12), 7627-7630.
-
(1999)
J. Biol. Chem.
, vol.274
, Issue.12
, pp. 7627-7630
-
-
Zerhusen, B.1
Xie, J.2
Davis, P.B.3
Ma, J.4
-
219
-
-
0033931302
-
Interaction between permeation and gating in a putative pore domain mutant in the cystic fibrosis transmembrane conductance regulator
-
Z.R. Zhang, S.I. McDonough and N.A. McCarty (2000) Interaction between permeation and gating in a putative pore domain mutant in the cystic fibrosis transmembrane conductance regulator. Biophys. J. 79(1), 298-313.
-
(2000)
Biophys. J.
, vol.79
, Issue.1
, pp. 298-313
-
-
Zhang, Z.R.1
McDonough, S.I.2
McCarty, N.A.3
-
220
-
-
0032855504
-
Association of cystic fibrosis transmembrane conductance regulator and protein phosphatase 2C
-
T. Zhu, D. Dahan, A. Evagelidis, S. Zheng, J. Luo and J.W. Hanrahan (1999) Association of cystic fibrosis transmembrane conductance regulator and protein phosphatase 2C. J. Biol. Chem. 274(41), 29102-29107.
-
(1999)
J. Biol. Chem.
, vol.274
, Issue.41
, pp. 29102-29107
-
-
Zhu, T.1
Dahan, D.2
Evagelidis, A.3
Zheng, S.4
Luo, J.5
Hanrahan, J.W.6
-
221
-
-
0342468966
-
ATP hydrolysis-coupled gating of CFTR chloride channels: structure and function
-
X. Zou and T.C. Hwang (2001) ATP hydrolysis-coupled gating of CFTR chloride channels: structure and function. Biochemistry 40(19), 5579-5586.
-
(2001)
Biochemistry
, vol.40
, Issue.19
, pp. 5579-5586
-
-
Zou, X.1
Hwang, T.C.2
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