-
1
-
-
6844258858
-
Modeling of nucleotide binding domains of ABC transporter proteins based on a F1-ATPase/recA topology: structural model of the nucleotide binding domains of the cystic fibrosis transmembrane conductance regulator (CFTR)
-
Bianchet M.A., Ko Y.H., Amzel L.M., and Pedersen P.L. Modeling of nucleotide binding domains of ABC transporter proteins based on a F1-ATPase/recA topology: structural model of the nucleotide binding domains of the cystic fibrosis transmembrane conductance regulator (CFTR). J. Bioenerg. Biomembr. 29 (1997) 503-524
-
(1997)
J. Bioenerg. Biomembr.
, vol.29
, pp. 503-524
-
-
Bianchet, M.A.1
Ko, Y.H.2
Amzel, L.M.3
Pedersen, P.L.4
-
2
-
-
0030154620
-
Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein
-
Brown C.R., Hong-Brown L.Q., Biwersi J., Verkman A.S., and Welch W.J. Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein. Cell Stress Chaperones 1 (1996) 117-125
-
(1996)
Cell Stress Chaperones
, vol.1
, pp. 117-125
-
-
Brown, C.R.1
Hong-Brown, L.Q.2
Biwersi, J.3
Verkman, A.S.4
Welch, W.J.5
-
6
-
-
0028241858
-
Mapping of cystic fibrosis transmembrane conductance regulator membrane topology by glycosylation site insertion
-
Chang X.B., Hou Y.X., Jensen T.J., and Riordan J.R. Mapping of cystic fibrosis transmembrane conductance regulator membrane topology by glycosylation site insertion. J. Biol. Chem. 269 (1994) 18572-18575
-
(1994)
J. Biol. Chem.
, vol.269
, pp. 18572-18575
-
-
Chang, X.B.1
Hou, Y.X.2
Jensen, T.J.3
Riordan, J.R.4
-
7
-
-
0033166350
-
Removal of multiple arginine-framed trafficking signals overcomes misprocessing of delta F508 CFTR present in most patients with cystic fibrosis
-
Chang X.B., Cui L., Hou Y.X., Jensen T.J., Aleksandrov A.A., Mengos A., and Riordan J.R. Removal of multiple arginine-framed trafficking signals overcomes misprocessing of delta F508 CFTR present in most patients with cystic fibrosis. Mol. Cell 4 (1999) 137-142
-
(1999)
Mol. Cell
, vol.4
, pp. 137-142
-
-
Chang, X.B.1
Cui, L.2
Hou, Y.X.3
Jensen, T.J.4
Aleksandrov, A.A.5
Mengos, A.6
Riordan, J.R.7
-
8
-
-
4544232744
-
The deltaF508 mutation disrupts packing of the transmembrane segments of the cystic fibrosis transmembrane conductance regulator
-
Chen E.Y., Bartlett M.C., Loo T.W., and Clarke D.M. The deltaF508 mutation disrupts packing of the transmembrane segments of the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 279 (2004) 39620-39627
-
(2004)
J. Biol. Chem.
, vol.279
, pp. 39620-39627
-
-
Chen, E.Y.1
Bartlett, M.C.2
Loo, T.W.3
Clarke, D.M.4
-
9
-
-
0025242929
-
Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
-
Cheng S.H., Gregory R.J., Marshall J., Paul S., Souza D.W., White G.A., O'Riordan C.R., and Smith A.E. Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell 63 (1990) 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
-
10
-
-
0028944377
-
Functional activation of the cystic fibrosis trafficking mutant delta F508-CFTR by overexpression
-
Cheng S.H., Fang S.L., Zabner J., Marshall J., Piraino S., Schiavi S.C., Jefferson D.M., Welsh M.J., and Smith A.E. Functional activation of the cystic fibrosis trafficking mutant delta F508-CFTR by overexpression. Am. J. Physiol. 268 (1995) L615-L624
-
(1995)
Am. J. Physiol.
, vol.268
-
-
Cheng, S.H.1
Fang, S.L.2
Zabner, J.3
Marshall, J.4
Piraino, S.5
Schiavi, S.C.6
Jefferson, D.M.7
Welsh, M.J.8
Smith, A.E.9
-
11
-
-
33645533055
-
The role of cystic fibrosis transmembrane conductance regulator phenylalanine 508 side chain in ion channel gating
-
Cui L., Aleksandrov L., Hou Y.X., Gentzsch M., Chen J.H., Riordan J.R., and Aleksandrov A.A. The role of cystic fibrosis transmembrane conductance regulator phenylalanine 508 side chain in ion channel gating. J. Physiol. 572 (2006) 347-358
-
(2006)
J. Physiol.
, vol.572
, pp. 347-358
-
-
Cui, L.1
Aleksandrov, L.2
Hou, Y.X.3
Gentzsch, M.4
Chen, J.H.5
Riordan, J.R.6
Aleksandrov, A.A.7
-
12
-
-
0037184104
-
Mutations in the nucleotide binding domain 1 signature motif region rescue processing and functional defects of cystic fibrosis transmembrane conductance regulator delta F508
-
deCarvalho A.C.V., Gansheroff L.J., and Teem J.L. Mutations in the nucleotide binding domain 1 signature motif region rescue processing and functional defects of cystic fibrosis transmembrane conductance regulator delta F508. J. Biol. Chem. 277 (2002) 35896-35905
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 35896-35905
-
-
deCarvalho, A.C.V.1
Gansheroff, L.J.2
Teem, J.L.3
-
13
-
-
0026781952
-
Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive
-
Denning G.M., Anderson M.P., Amara J.F., Marshall J., Smith A.E., and Welsh M.J. Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive. Nature 358 (1992) 761-764
-
(1992)
Nature
, vol.358
, 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
-
14
-
-
0025820979
-
Cystic fibrosis with three mutations in the cystic fibrosis transmembrane conductance regulator gene
-
Dörk T., Wulbrand U., Richter T., Neumann T., Wolfes H., Wulf B., Maass G., and Tümmler B. Cystic fibrosis with three mutations in the cystic fibrosis transmembrane conductance regulator gene. Hum. Genet. 87 (1991) 441-446
-
(1991)
Hum. Genet.
, vol.87
, pp. 441-446
-
-
Dörk, T.1
Wulbrand, U.2
Richter, T.3
Neumann, T.4
Wolfes, H.5
Wulf, B.6
Maass, G.7
Tümmler, B.8
-
15
-
-
11444266284
-
The deltaF508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR
-
Du K., Sharma M., and Lukacs G.L. The deltaF508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR. Nat. Struct. Mol. Biol. 12 (2005) 17-25
-
(2005)
Nat. Struct. Mol. Biol.
, vol.12
, pp. 17-25
-
-
Du, K.1
Sharma, M.2
Lukacs, G.L.3
-
16
-
-
0028328921
-
A two-domain model for the R domain of the cystic fibrosis transmembrane conductance regulator based on sequence similarities
-
Dulhanty A.M., and Riordan J.R. A two-domain model for the R domain of the cystic fibrosis transmembrane conductance regulator based on sequence similarities. FEBS Lett. 343 (1994) 109-114
-
(1994)
FEBS Lett.
, vol.343
, pp. 109-114
-
-
Dulhanty, A.M.1
Riordan, J.R.2
-
17
-
-
20344378216
-
Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin
-
Farinha C.M., and Amaral M.D. Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin. Mol. Cell. Biol. 25 (2005) 5242-5252
-
(2005)
Mol. Cell. Biol.
, vol.25
, pp. 5242-5252
-
-
Farinha, C.M.1
Amaral, M.D.2
-
18
-
-
3242674336
-
A comparison of 14 antibodies for the biochemical detection of the cystic fibrosis transmembrane conductance regulator protein
-
Farinha C.M., Mendes F., Roxo-Rosa M., Penque D., and Amaral M.D. A comparison of 14 antibodies for the biochemical detection of the cystic fibrosis transmembrane conductance regulator protein. Mol. Cell. Probes 18 (2004) 235-242
-
(2004)
Mol. Cell. Probes
, vol.18
, pp. 235-242
-
-
Farinha, C.M.1
Mendes, F.2
Roxo-Rosa, M.3
Penque, D.4
Amaral, M.D.5
-
19
-
-
19944402159
-
Biochemical methods to assess CFTR expression and membrane localization
-
Farinha C.M., Penque D., Roxo-Rosa M., Lukacs G., Dormer R., McPherson M., Pereira M., Bot A.G., Jorna H., Willemsen R., et al. Biochemical methods to assess CFTR expression and membrane localization. J. Cyst. Fibros. 3 Suppl 2 (2004) 73-77
-
(2004)
J. Cyst. Fibros.
, vol.3
, Issue.SUPPL. 2
, pp. 73-77
-
-
Farinha, C.M.1
Penque, D.2
Roxo-Rosa, M.3
Lukacs, G.4
Dormer, R.5
McPherson, M.6
Pereira, M.7
Bot, A.G.8
Jorna, H.9
Willemsen, R.10
-
20
-
-
0034435098
-
A novel CFTR disease-associated mutation causes addition of an extra N-linked oligosaccharide
-
Hammerle M.M., Aleksandrov A.A., Chang X.B., and Riordan J.R. A novel CFTR disease-associated mutation causes addition of an extra N-linked oligosaccharide. Glycoconj. J. 17 (2000) 807-813
-
(2000)
Glycoconj. J.
, vol.17
, pp. 807-813
-
-
Hammerle, M.M.1
Aleksandrov, A.A.2
Chang, X.B.3
Riordan, J.R.4
-
22
-
-
0028858161
-
Multiple proteolytic systems, including the proteasome, contribute to CFTR processing
-
Jensen T.J., Loo M.A., Pind S., Williams D.B., Goldberg A.L., and Riordan J.R. Multiple proteolytic systems, including the proteasome, contribute to CFTR processing. Cell 83 (1995) 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
-
24
-
-
38349072478
-
Structures of human CFTR NBD1 confirm conservation of the domain structure despite the delF508 mutation
-
Lewis H. Structures of human CFTR NBD1 confirm conservation of the domain structure despite the delF508 mutation. Pediatr. Pulmonol. 40 (2005) 190-191
-
(2005)
Pediatr. Pulmonol.
, vol.40
, pp. 190-191
-
-
Lewis, H.1
-
25
-
-
10744230777
-
Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator
-
Lewis H.A., Buchanan S.G., Burley S.K., Conners K., Dickey M., Dorwart M., Fowler R., Gao X., Guggino W.B., Hendrickson W.A., et al. Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator. EMBO J. 23 (2004) 282-293
-
(2004)
EMBO J.
, vol.23
, pp. 282-293
-
-
Lewis, H.A.1
Buchanan, S.G.2
Burley, S.K.3
Conners, K.4
Dickey, M.5
Dorwart, M.6
Fowler, R.7
Gao, X.8
Guggino, W.B.9
Hendrickson, W.A.10
-
26
-
-
19944432524
-
Impact of the deltaF508 mutation in first nucleotide-binding domain of human cystic fibrosis transmembrane conductance regulator on domain folding and structure
-
Lewis H.A., Zhao X., Wang C., Sauder J.M., Rooney I., Noland B.W., Lorimer D., Kearins M.C., Conners K., Condon B., et al. Impact of the deltaF508 mutation in first nucleotide-binding domain of human cystic fibrosis transmembrane conductance regulator on domain folding and structure. J. Biol. Chem. 280 (2005) 1346-1353
-
(2005)
J. Biol. Chem.
, vol.280
, pp. 1346-1353
-
-
Lewis, H.A.1
Zhao, X.2
Wang, C.3
Sauder, J.M.4
Rooney, I.5
Noland, B.W.6
Lorimer, D.7
Kearins, M.C.8
Conners, K.9
Condon, B.10
-
27
-
-
0033536032
-
Cystic fibrosis transmembrane conductance regulator: solution structures of peptides based on the Phe508 region, the most common site of disease-causing deltaF508 mutation
-
Massiah M.A., Ko Y.H., Pedersen P.L., and Mildvan A.S. Cystic fibrosis transmembrane conductance regulator: solution structures of peptides based on the Phe508 region, the most common site of disease-causing deltaF508 mutation. Biochemistry 38 (1999) 7453-7461
-
(1999)
Biochemistry
, vol.38
, pp. 7453-7461
-
-
Massiah, M.A.1
Ko, Y.H.2
Pedersen, P.L.3
Mildvan, A.S.4
-
28
-
-
21844448588
-
A quantitative description of the activation and inhibition of CFTR by potentiators: genistein
-
Moran O., and Zegarra-Moran O. A quantitative description of the activation and inhibition of CFTR by potentiators: genistein. FEBS Lett. 579 (2005) 3979-3983
-
(2005)
FEBS Lett.
, vol.579
, pp. 3979-3983
-
-
Moran, O.1
Zegarra-Moran, O.2
-
29
-
-
0034625153
-
A functional R domain from cystic fibrosis transmembrane conductance regulator is predominantly unstructured in solution
-
Ostedgaard L.S., Baldursson O., Vermeer D.W., Welsh M.J., and Robertson A.D. A functional R domain from cystic fibrosis transmembrane conductance regulator is predominantly unstructured in solution. Proc. Natl. Acad. Sci. USA 97 (2000) 5657-5662
-
(2000)
Proc. Natl. Acad. Sci. USA
, vol.97
, pp. 5657-5662
-
-
Ostedgaard, L.S.1
Baldursson, O.2
Vermeer, D.W.3
Welsh, M.J.4
Robertson, A.D.5
-
30
-
-
0029997424
-
Alteration of the cystic fibrosis transmembrane conductance regulator folding pathway
-
Qu B.H., and Thomas P.J. Alteration of the cystic fibrosis transmembrane conductance regulator folding pathway. J. Biol. Chem. 271 (1996) 7261-7264
-
(1996)
J. Biol. Chem.
, vol.271
, pp. 7261-7264
-
-
Qu, B.H.1
Thomas, P.J.2
-
31
-
-
15544371839
-
Assembly of functional CFTR chloride channels
-
Riordan J.R. Assembly of functional CFTR chloride channels. Annu. Rev. Physiol. 67 (2005) 701-718
-
(2005)
Annu. Rev. Physiol.
, vol.67
, pp. 701-718
-
-
Riordan, J.R.1
-
32
-
-
0024424270
-
Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA
-
Riordan J.R., Rommens J.M., Kerem B., Alon N., Rozmahel R., Grzelczak Z., Zielenski J., Lok S., Plavsic N., and Chou J.L. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 245 (1989) 1066-1073
-
(1989)
Science
, vol.245
, pp. 1066-1073
-
-
Riordan, J.R.1
Rommens, J.M.2
Kerem, B.3
Alon, N.4
Rozmahel, R.5
Grzelczak, Z.6
Zielenski, J.7
Lok, S.8
Plavsic, N.9
Chou, J.L.10
-
34
-
-
33845197320
-
Revertant mutants G550E and 4RK rescue cystic fibrosis mutants in the first nucleotide-binding domain of CFTR by different mechanisms
-
Roxo-Rosa M., Xu Z., Schmidt A., Neto M., Cai Z., Soares C.M., Sheppard D.N., and Amaral M.D. Revertant mutants G550E and 4RK rescue cystic fibrosis mutants in the first nucleotide-binding domain of CFTR by different mechanisms. Proc. Natl. Acad. Sci. USA 103 (2006) 17891-17896
-
(2006)
Proc. Natl. Acad. Sci. USA
, vol.103
, pp. 17891-17896
-
-
Roxo-Rosa, M.1
Xu, Z.2
Schmidt, A.3
Neto, M.4
Cai, Z.5
Soares, C.M.6
Sheppard, D.N.7
Amaral, M.D.8
-
35
-
-
38349033879
-
Prolonged treatment of cells with genistein modulates the expression and function of the cystic fibrosis transmembrane conductance regulator
-
in press
-
Schmidt A., Hughes L.K., Cai Z., Mendes F., Li H., Sheppard D.N., and Amaral M.D. Prolonged treatment of cells with genistein modulates the expression and function of the cystic fibrosis transmembrane conductance regulator. Br. J. Pharmacol. (2008) in press
-
(2008)
Br. J. Pharmacol.
-
-
Schmidt, A.1
Hughes, L.K.2
Cai, Z.3
Mendes, F.4
Li, H.5
Sheppard, D.N.6
Amaral, M.D.7
-
36
-
-
0027153083
-
Identification of revertants for the cystic fibrosis delta F508 mutation using STE6-CFTR chimeras in yeast
-
Teem J.L., Berger H.A., Ostedgaard L.S., Rich D.P., Tsui L.C., and Welsh M.J. Identification of revertants for the cystic fibrosis delta F508 mutation using STE6-CFTR chimeras in yeast. Cell 73 (1993) 335-346
-
(1993)
Cell
, vol.73
, pp. 335-346
-
-
Teem, J.L.1
Berger, H.A.2
Ostedgaard, L.S.3
Rich, D.P.4
Tsui, L.C.5
Welsh, M.J.6
-
37
-
-
0029864612
-
Mutation of R555 in CFTR-delta F508 enhances function and partially corrects defective processing
-
Teem J.L., Carson M.R., and Welsh M.J. Mutation of R555 in CFTR-delta F508 enhances function and partially corrects defective processing. Receptors Channels 4 (1996) 63-72
-
(1996)
Receptors Channels
, vol.4
, pp. 63-72
-
-
Teem, J.L.1
Carson, M.R.2
Welsh, M.J.3
-
39
-
-
2442451126
-
Misfolded proteins are sorted by a sequential checkpoint mechanism of ER quality control
-
Vashist S., and Ng D.T. Misfolded proteins are sorted by a sequential checkpoint mechanism of ER quality control. J. Cell Biol. 165 (2004) 41-52
-
(2004)
J. Cell Biol.
, vol.165
, pp. 41-52
-
-
Vashist, S.1
Ng, D.T.2
-
40
-
-
14544300522
-
CFTR channel opening by ATP-driven tight dimerization of its nucleotide-binding domains
-
Vergani P., Lockless S.W., Nairn A.C., and Gadsby D.C. CFTR channel opening by ATP-driven tight dimerization of its nucleotide-binding domains. Nature 433 (2005) 876-880
-
(2005)
Nature
, vol.433
, pp. 876-880
-
-
Vergani, P.1
Lockless, S.W.2
Nairn, A.C.3
Gadsby, D.C.4
-
41
-
-
0028840915
-
Degradation of CFTR by the ubiquitin-proteasome pathway
-
Ward C.L., Omura S., and Kopito R.R. Degradation of CFTR by the ubiquitin-proteasome pathway. Cell 83 (1995) 121-127
-
(1995)
Cell
, vol.83
, pp. 121-127
-
-
Ward, C.L.1
Omura, S.2
Kopito, R.R.3
|