-
1
-
-
0030154620
-
Chemical chaperones correct the mutant phenotype of the ΔF508 cystic fibrosis transmembrane conductance regulator protein
-
BROWN, C. R., L. Q. HONG-BROWN, J. BIWERSI, A. S. VERKMAN, AND W. J. WELCH. Chemical chaperones correct the mutant phenotype of the ΔF508 cystic fibrosis transmembrane conductance regulator protein. Cell Stress Chaperones 1: 117-125, 1996.
-
(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
-
2
-
-
0025242929
-
Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
-
CHENG, S. H., R. J. GREGORY, J. MARSHALL, S. PAUL, D. W. SOUZA, G. A. WHITE, C. R. O'RIORDAN, AND A. E. SMITH. Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell 63: 827-834, 1990.
-
(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
-
3
-
-
0029835571
-
Effect of cystic fibrosis-associated mutations in the fourth intracellular loop of cystic fibrosis transmembrane conductance regulator
-
COTTEN, J. F., L. S. OSTEDGAARD, M. R. CARSON, AND M. J. WELSH. Effect of cystic fibrosis-associated mutations in the fourth intracellular loop of cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 271: 21279-21284, 1996.
-
(1996)
J. Biol. Chem.
, vol.271
, pp. 21279-21284
-
-
Cotten, J.F.1
Ostedgaard, L.S.2
Carson, M.R.3
Welsh, M.J.4
-
4
-
-
0026325533
-
Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation
-
DALEMANS, W., P. BARBRY, G. CHAMPIGNY, S. JALLAT, K. DOTT, D. DREYER, R. G. CRYSTAL, A. PAVIRANI, J. P. LECOCQ, AND M. LAZDUNSKI. Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation. Nature 354: 526-528, 1991.
-
(1991)
Nature
, vol.354
, pp. 526-528
-
-
Dalemans, W.1
Barbry, P.2
Champigny, G.3
Jallat, S.4
Dott, K.5
Dreyer, D.6
Crystal, R.G.7
Pavirani, A.8
Lecocq, J.P.9
Lazdunski, M.10
-
5
-
-
0026781952
-
Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive
-
DENNING, G. M., M. P. ANDERSON, J. F. AMARA, J. MARSHALL, A. E. SMITH, AND M. J. WELSH. Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive. Nature 358: 761-764, 1992.
-
(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
-
6
-
-
0026337305
-
Chloride conductance expressed by ΔF508 and other mutant CFTRs in Xenopus oocytes
-
DRUMM, M. L., D. J. WILKINSON, L. S. SMIT, R. T. WORRELL, T. V. STRONG, R. A. FRIZZELL, D. C. DAWSON, AND F. S. COLLINS. Chloride conductance expressed by ΔF508 and other mutant CFTRs in Xenopus oocytes. Science 254: 1797-1799, 1991.
-
(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
-
7
-
-
0029040991
-
Role of the endoplasmic reticulum chaperone calnexin in subunit folding and assembly of nicotinic: Acetylcholine receptors
-
GELMAN, M. S., W. CHANG, D. Y. THOMAS, J. J. BERGERON, AND J. M. PRIVES. Role of the endoplasmic reticulum chaperone calnexin in subunit folding and assembly of nicotinic: acetylcholine receptors. J. Biol. Chem. 270: 15085-15092, 1995.
-
(1995)
J. Biol. Chem.
, vol.270
, pp. 15085-15092
-
-
Gelman, M.S.1
Chang, W.2
Thomas, D.Y.3
Bergeron, J.J.4
Prives, J.M.5
-
8
-
-
0025912486
-
Maturation and function of cystic fibrosis transmembrane conductance regulator variants bearing mutations in putative nucleotide-binding domains 1 and 2
-
GREGORY, R. J., D. P. RICH, S. H. CHENG, D. W. SOUZA, S. PAUL, P. MANAVALAN, M. P. ANDERSON, M. J. WELSH, AND A. E. SMITH. Maturation and function of cystic fibrosis transmembrane conductance regulator variants bearing mutations in putative nucleotide-binding domains 1 and 2. Mol. Cell. Biol. 11: 3886-3893, 1991.
-
(1991)
Mol. Cell. Biol.
, vol.11
, pp. 3886-3893
-
-
Gregory, R.J.1
Rich, D.P.2
Cheng, S.H.3
Souza, D.W.4
Paul, S.5
Manavalan, P.6
Anderson, M.P.7
Welsh, M.J.8
Smith, A.E.9
-
9
-
-
0029094253
-
Quality control in the secretory pathway
-
HAMMOND, C., AND A. HELENIUS. Quality control in the secretory pathway. Curr. Opin. Cell Biol. 1: 523-529, 1995.
-
(1995)
Curr. Opin. Cell Biol.
, vol.1
, pp. 523-529
-
-
Hammond, C.1
Helenius, A.2
-
10
-
-
0029992278
-
Molecular chaperones in cellular protein folding
-
HARTL, F. U. Molecular chaperones in cellular protein folding. Nature 381: 571-579, 1996.
-
(1996)
Nature
, vol.381
, pp. 571-579
-
-
Hartl, F.U.1
-
11
-
-
0028198111
-
How N-linked oligosaccharides affect glycoprotein folding in the endoplasmic reticulum
-
HELENIUS, A. How N-linked oligosaccharides affect glycoprotein folding in the endoplasmic reticulum. Mol. Biol. Cell 5: 253-265, 1994.
-
(1994)
Mol. Biol. Cell
, vol.5
, pp. 253-265
-
-
Helenius, A.1
-
12
-
-
0024960594
-
Transport proteins. Export-import family expands
-
HIGGINS, C. Transport proteins. Export-import family expands. Nature 340: 342, 1989.
-
(1989)
Nature
, vol.340
, pp. 342
-
-
Higgins, C.1
-
13
-
-
0023991287
-
A family of closely related ATP-binding subunits from prokaryotic and eukaryotic cells
-
HIGGINS, C. F., M. P. GALLAGHER, M. L. MIMMACK, AND S. R. PEARCE. A family of closely related ATP-binding subunits from prokaryotic and eukaryotic cells. Bioessays 8: 111-116, 1988.
-
(1988)
Bioessays
, vol.8
, pp. 111-116
-
-
Higgins, C.F.1
Gallagher, M.P.2
Mimmack, M.L.3
Pearce, S.R.4
-
14
-
-
0028858161
-
Multiple proteolytic systems, including the proteasome, contribute to CFTR processing
-
JENSEN, T. J., M. A. LOO, S. PIND, D. B. WILLIAMS, A. L. GOLDBERG, AND J. R. RIORDAN. Multiple proteolytic systems, including the proteasome, contribute to CFTR processing. Cell 83: 129-135, 1995.
-
(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
-
15
-
-
0026907529
-
Mislocalization of delta F508 CFTR in cystic fibrosis sweat gland
-
KARTNER, N., O. AUGUSTINAS, T. J. JENSEN, A. L. NAISMITH, AND J. R. RIORDAN. Mislocalization of delta F508 CFTR in cystic fibrosis sweat gland. Nature Genet. 1: 321-327, 1992.
-
(1992)
Nature Genet.
, vol.1
, pp. 321-327
-
-
Kartner, N.1
Augustinas, O.2
Jensen, T.J.3
Naismith, A.L.4
Riordan, J.R.5
-
16
-
-
0024423668
-
Identification of the cystic fibrosis gene: Genetic analysis
-
KEREM, B.-S., J. M. ROMMENS, J. A. BUCHANAN, D. MARKIEWICZ, T. K. COX, A. CHAKRAVARTI, M. BUCHWALD, AND L.-C. TSUI. Identification of the cystic fibrosis gene: genetic analysis. Science 245: 1073-1080, 1989.
-
(1989)
Science
, vol.245
, pp. 1073-1080
-
-
Kerem, B.-S.1
Rommens, J.M.2
Buchanan, J.A.3
Markiewicz, D.4
Cox, T.K.5
Chakravarti, A.6
Buchwald, M.7
Tsui, L.-C.8
-
17
-
-
0030949874
-
ER quality control: The cytoplasmic connection
-
KOPITO, R. R. ER quality control: the cytoplasmic connection. Cell 88: 427-430, 1997.
-
(1997)
Cell
, vol.88
, pp. 427-430
-
-
Kopito, R.R.1
-
18
-
-
0031021804
-
Correction of defective protein kinesis of human P-glycoprotein mutants by substrates and modulators
-
LOO, T. W., AND D. M. CLARKE. Correction of defective protein kinesis of human P-glycoprotein mutants by substrates and modulators. J. Biol. Chem. 272: 709-712, 1997.
-
(1997)
J. Biol. Chem.
, vol.272
, pp. 709-712
-
-
Loo, T.W.1
Clarke, D.M.2
-
19
-
-
0028559511
-
Conformational maturation of CFTR but not its mutant counterpart (ΔF508) occurs in the endoplasmic reticulum and requires ATP
-
LUKACS, G. L., A. MOHAMED, N. KARTNER, X.-B. CHANG, J. R. RIORDAN, AND S. GRINSTEIN. Conformational maturation of CFTR but not its mutant counterpart (ΔF508) occurs in the endoplasmic reticulum and requires ATP. EMBOJ. 13: 6076-6086, 1994.
-
(1994)
EMBOJ.
, 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
-
20
-
-
0028129406
-
Stoichiometry of recombinant cystic fibrosis transmembrane conductance regulator in epithelial cells and its functional reconstitution into cells in vitro
-
MARSHALL, J., S. FANG, L. S. OSTEDGAARD, C. R. O'RIORDAN, D. FERRARA, J. F. AMARA, H. HOPPE, R. K. SCHEULE, M. J. WELSH, A. E. SMITH, AND S. H. CHENG. Stoichiometry of recombinant cystic fibrosis transmembrane conductance regulator in epithelial cells and its functional reconstitution into cells in vitro. J. Biol. Chem. 269: 2987-2995, 1994.
-
(1994)
J. Biol. Chem.
, vol.269
, pp. 2987-2995
-
-
Marshall, J.1
Fang, S.2
Ostedgaard, L.S.3
O'Riordan, C.R.4
Ferrara, D.5
Amara, J.F.6
Hoppe, H.7
Scheule, R.K.8
Welsh, M.J.9
Smith, A.E.10
Cheng, S.H.11
-
22
-
-
0020616951
-
Assembly in vivo of mouse muscle acetylcholine receptor: Identification of an alpha subunit species that may be an assembly intermediate
-
MERLIE, J. P., AND J. LINDSTROM. Assembly in vivo of mouse muscle acetylcholine receptor: identification of an alpha subunit species that may be an assembly intermediate. Cell 34: 747-757, 1983.
-
(1983)
Cell
, vol.34
, pp. 747-757
-
-
Merlie, J.P.1
Lindstrom, J.2
-
23
-
-
0030010769
-
Subpopulations of proteasomes in rat liver nuclei, microsomes and cytosol
-
PALMER A., A. J. RIVETT, S. THOMSON, K. B. HENDIL, G. W. BUTCHER, G. FUERTES, AND E. KNECHT. Subpopulations of proteasomes in rat liver nuclei, microsomes and cytosol. Biochem. J. 316: 401-407, 1996.
-
(1996)
Biochem. J.
, vol.316
, pp. 401-407
-
-
Palmer, A.1
Rivett, A.J.2
Thomson, S.3
Hendil, K.B.4
Butcher, G.W.5
Fuertes, G.6
Knecht, E.7
-
24
-
-
0028232167
-
Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator
-
PIND, S., J. R. RIORDAN, AND D. B. WILLIAMS. Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 269: 12784-12788, 1994.
-
(1994)
J. Biol. Chem.
, vol.269
, pp. 12784-12788
-
-
Pind, S.1
Riordan, J.R.2
Williams, D.B.3
-
25
-
-
0031006695
-
Localization and suppression of a kinetic defect in cystic fibrosis transmembrane conductance regulator folding
-
QU, B.-R, E. H. STRICKLAND, AND P. J. THOMAS. Localization and suppression of a kinetic defect in cystic fibrosis transmembrane conductance regulator folding. J. Biol. Chem. 272: 15739-15744, 1997.
-
(1997)
J. Biol. Chem.
, vol.272
, pp. 15739-15744
-
-
Qu, B.-R.1
Strickland, E.H.2
Thomas, P.J.3
-
26
-
-
0029997424
-
Alteration of the cystic fibrosis transmembrane conductance regulator folding pathway
-
QU, B. H., AND P. J. THOMAS. Alteration of the cystic fibrosis transmembrane conductance regulator folding pathway. J. Biol. Chem. 271: 7261-7264, 1996.
-
(1996)
J. Biol. Chem.
, vol.271
, pp. 7261-7264
-
-
Qu, B.H.1
Thomas, P.J.2
-
27
-
-
0024424270
-
Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
-
RIORDAN, J. R., J. M. ROMMENS, B.-S. KEREM, N. ALON, R. ROZMAHEL, Z. GRZELCZAK, J. ZIELENSKI, S. LOK, N. PLAVSIC, J.-L. CHOU, M. L. DRUMM, M. C. IANNUZZI, F. S. COLLINS, AND L-C. TSUI. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA Science 245: 1066-1073, 1989.
-
(1989)
Science
, vol.245
, pp. 1066-1073
-
-
Riordan, J.R.1
Rommens, J.M.2
Kerem, B.-S.3
Alon, N.4
Rozmahel, R.5
Grzelczak, Z.6
Zielenski, J.7
Lok, S.8
Plavsic, N.9
Chou, J.-L.10
Drumm, M.L.11
Iannuzzi, M.C.12
Collins, F.S.13
Tsui, L.-C.14
-
28
-
-
0026651895
-
Electron microscopic localization of the multicatalytic proteinase complex in rat liver and in cultured cells
-
RIVETT, A. J., A. PALMER, AND E. KNECHT. Electron microscopic localization of the multicatalytic proteinase complex in rat liver and in cultured cells. J. Histochem. Cytochem. 40: 1165-1172, 1992.
-
(1992)
J. Histochem. Cytochem.
, vol.40
, pp. 1165-1172
-
-
Rivett, A.J.1
Palmer, A.2
Knecht, E.3
-
29
-
-
0024453308
-
Identification of the cystic fibrosis gene: Chromosome walking and jumping
-
ROMMENS, J. M., M. C. IANNUZZI, B.-S. KEREM, M. L. DRUMM, G. MELMER, M. DEAN, R. ROZMAHEL, J. L. COLE, D. KENNEDY, N. HIDAKA, M. ZSIGA, M. BUCHWALD, J. R. RIORDAN, L.-C. TSUI, AND F. S. COLLINS. Identification of the cystic fibrosis gene: chromosome walking and jumping. Science 245: 1059-1065, 1989.
-
(1989)
Science
, vol.245
, pp. 1059-1065
-
-
Rommens, J.M.1
Iannuzzi, M.C.2
Kerem, B.-S.3
Drumm, M.L.4
Melmer, G.5
Dean, M.6
Rozmahel, R.7
Cole, J.L.8
Kennedy, D.9
Hidaka, N.10
Zsiga, M.11
Buchwald, M.12
Riordan, J.R.13
Tsui, L.-C.14
Collins, F.S.15
-
30
-
-
0023665223
-
Phosphorylation and assembly of nicotinic acetylcholine receptor subunits in cultured chick muscle cells
-
ROSS, A. F., M. RAPUANO, J. H. SCHMIDT, AND J. M. PRIVES. Phosphorylation and assembly of nicotinic acetylcholine receptor subunits in cultured chick muscle cells. J. Biol. Chem. 262: 14640-14647, 1987.
-
(1987)
J. Biol. Chem.
, vol.262
, pp. 14640-14647
-
-
Ross, A.F.1
Rapuano, M.2
Schmidt, J.H.3
Prives, J.M.4
-
31
-
-
0032571366
-
Cotranslational ubiquitination of cystic fibrosis transmembrane conductance regulator in vitro
-
SATO, S., C. L. WARD, AND R. R. KOPITO. Cotranslational ubiquitination of cystic fibrosis transmembrane conductance regulator in vitro. J. Biol. Chem. 273: 7189-7192, 1998.
-
(1998)
J. Biol. Chem.
, vol.273
, pp. 7189-7192
-
-
Sato, S.1
Ward, C.L.2
Kopito, R.R.3
-
32
-
-
0030042386
-
Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation
-
SATO, S., C. L. WARD, M. E. KROUSE, J. J. WINE, AND R. R. KOPITO. Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation. J. Biol. Chem. 271: 635-638, 1996.
-
(1996)
J. Biol. Chem.
, vol.271
, pp. 635-638
-
-
Sato, S.1
Ward, C.L.2
Krouse, M.E.3
Wine, J.J.4
Kopito, R.R.5
-
33
-
-
17544374096
-
Disease-associated mutations in the fourth cytoplasmic loop of cystic fibrosis transmembrane conductance regulator compromise biosynthetic processing and chloride channel activity
-
SEIBERT, F. S., P. LINSDELL, T. W. LOO, J. W. HANRAHAN, D. M. CLARKE, AND J. R. RIORDAN. Disease-associated mutations in the fourth cytoplasmic loop of cystic fibrosis transmembrane conductance regulator compromise biosynthetic processing and chloride channel activity. J. Biol. Chem. 271: 15139-15145, 1996.
-
(1996)
J. Biol. Chem.
, vol.271
, pp. 15139-15145
-
-
Seibert, F.S.1
Linsdell, P.2
Loo, T.W.3
Hanrahan, J.W.4
Clarke, D.M.5
Riordan, J.R.6
-
34
-
-
0029910820
-
Cytoplasmic loop three of cystic fibrosis transmembrane conductance regulator contributes to regulation of chloride channel activity
-
SEIBERT, F. S., P. LINSDELL, T. W. LOO, J. W. HANRAHAN, J. R. RIORDAN, AND D. M. CLARKE. Cytoplasmic loop three of cystic fibrosis transmembrane conductance regulator contributes to regulation of chloride channel activity. J. Biol Chem. 271: 27493-27499, 1996.
-
(1996)
J. Biol Chem.
, vol.271
, 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
-
35
-
-
15844397666
-
Contribution of proline residues in the membrane-spanning domains of cystic fibrosis transmembrane conductance regulator to chloride channel function
-
SHEPPARD, D. N., S. M. TRAVIS, H. ISHIHARA, AND M. J. WELSH. Contribution of proline residues in the membrane-spanning domains of cystic fibrosis transmembrane conductance regulator to chloride channel function. J. Biol. Chem. 271: 14995-15001, 1996.
-
(1996)
J. Biol. Chem.
, vol.271
, pp. 14995-15001
-
-
Sheppard, D.N.1
Travis, S.M.2
Ishihara, H.3
Welsh, M.J.4
-
36
-
-
0028006681
-
Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins
-
WARD, C. L., AND R. R. KOPITO. Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins. J. Biol. Chem. 269: 25710-25718, 1994.
-
(1994)
J. Biol. Chem.
, vol.269
, pp. 25710-25718
-
-
Ward, C.L.1
Kopito, R.R.2
-
37
-
-
0028840915
-
Degradation of CFTR by the ubiquitin proteasome pathway
-
WARD, C. L., S. OMURA, AND R. R. KOPITO. Degradation of CFTR by the ubiquitin proteasome pathway. Cell 83: 121-127, 1995.
-
(1995)
Cell
, vol.83
, pp. 121-127
-
-
Ward, C.L.1
Omura, S.2
Kopito, R.R.3
-
38
-
-
0029915568
-
The human cytomegalovirus US11 gene product dislocates MHC class I heavy chains from the endoplasmic reticulum to the cytosol
-
WIERTZ, E. J., T. R. JONES, L. SUN, M. BOGYO, H. J. GEUZE, AND H. L. PLOEGH. The human cytomegalovirus US11 gene product dislocates MHC class I heavy chains from the endoplasmic reticulum to the cytosol. Cell 84: 769-779, 1996.
-
(1996)
Cell
, vol.84
, pp. 769-779
-
-
Wiertz, E.J.1
Jones, T.R.2
Sun, L.3
Bogyo, M.4
Geuze, H.J.5
Ploegh, H.L.6
-
39
-
-
0029828991
-
Sec61-mediated transfer of a membrane protein from the endoplasmic reticulum to the proteasome for destruction
-
WIERTZ, E. J., D. TORTORELLA, M. BOGYO, J. YU, W. MOTHES, T. R. JONES, T. A. RAPOPORT, AND H. L. PLOEGH. Sec61-mediated transfer of a membrane protein from the endoplasmic reticulum to the proteasome for destruction. Nature 384: 432-438, 1996.
-
(1996)
Nature
, vol.384
, pp. 432-438
-
-
Wiertz, E.J.1
Tortorella, D.2
Bogyo, M.3
Yu, J.4
Mothes, W.5
Jones, T.R.6
Rapoport, T.A.7
Ploegh, H.L.8
-
40
-
-
0027488993
-
The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment
-
YANG, Y., S. JANICH, J. A. COHN, AMD J. M. WILSON. 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.
-
(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
-
41
-
-
0030817978
-
Cytosolic degradation of T-cell receptor alpha chains by the proteasome
-
YU, H., G. KAUNG, S. KOBAYASHI, AND R. R. KOPITO. Cytosolic degradation of T-cell receptor alpha chains by the proteasome. J. Biol. Chem. 272: 20800-20804, 1997.
-
(1997)
J. Biol. Chem.
, vol.272
, pp. 20800-20804
-
-
Yu, H.1
Kaung, G.2
Kobayashi, S.3
Kopito, R.R.4
|