Processing of CFTR bearing the P574H mutation differs from wild-type and deltaF508-CFTR

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Anderson, R. G. W., Orci, L., Brown, M. S., Garcia-Segura, L. M. and Goldstein, J. L (1983). Ultrastructural analysis of crystalloid endoplasmic reticulum in UT-1 cells and its disappearance in response to cholesterol. J. Cell Sci 63, 1-20.[Abstract]

Bloom, G. S. and Brashear, T. A (1989). A novel 58-kDa protein associates with the Golgi apparatus and microtubules. J. Biol. Chem 264, 16083-16092.[Abstract/Free Full Text]

Bradbury, N. A., Jilling, T., Berta, G., Sorscher, E. J., Bridges, R. J. and Kirk, K. L (1992). Regulation of plasma membrane recycling by CFTR. Science 256, 530-532.[Abstract/Free Full Text]

Bradbury, N. A., Cohn, J. A., Venglarik, C. J. and Bridges, R. J (1994). Biochemical and biophysical identification of cystic fibrosis transmembrane conductance regulator chloride channels as components of endocytic clathrin-coated vesicles. J. Biol. Chem 269, 8296-8302.[Abstract/Free Full Text]

Brodsky, F. M (1988). Living with clathrin: its role in intracellular membrane traffic. Science 242, 1396-1402.[Abstract/Free Full Text]

Champigny, G., Imler, J.-L., Puchelle, E., Dalemans, W., Gribkoff, V., Hinnrasky, J., Dott, K., Barbry, P., Pavirani, A. and Lazdunski, M (1995). A change in gating mode leading to increased intrinsic Clchannel activity compensates for defective processing in a cystic fibrosis mutant corresponding to a mild form of the disease. EMBO J 14, 2417-2423.[Medline]

Cheng, S. H., Gregory, R. J., Marshall, J., Paul, S., Souza, D. W., White, G. A., O'Riordan, C. R. and Smith, A. E (1990). Defective intracellulartransport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell 63, 827-834.[Medline]

Chin, D. J., Luskey, K. I., Anderson, R. G., Faust, J. R., Goldstein, J. L. and Brown, M. S (1982). Appearance of crystalloid endoplasmic reticulum in compactin-resistant Chinese hamster cells with a 500-fold increase in 3-hydroxy-3-methylglutaryl-coenzyme A reductance. Proc. Nat. Acad. Sci. USA 79, 1185-1189.[Abstract/Free Full Text]

Collins, F. S (1992). Cystic fibrosis: molecular biology and therapeutic implications. Science 256, 774-779.[Abstract/Free Full Text]

Denning, G. M., Anderson, M. P., Amara, J. F., Marshall, J., Smith, A. E. and Welsh, M. J (1992). Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive. Nature 358, 761-764.[Medline]

Denning, G. M., Ostedgaard, L. S., Cheng, S. H., Smith, A. E. and Welsh, M. J (1992). Localization of cystic fibrosis transmembrane conductance regulator in chloride secretory epithelia. J. Clin. Invest 89, 339-349.

Duden, R., Griffiths, G., Frank, R., Argos, P. and Kreis, T. E (1991). Beta-COP, a 110 kd protein associated with non-clathrin-coated vesicles and the Golgi complex, shows homology to beta-adaptin. Cell 64, 649-665.[Medline]

Elroy-Stein, O., Fuerst, T. R. and Moss, B (1989). Cap-independent translation of mRNA conferred by encephalomyocarditis virus 5sequence improves the performance of the vaccinia virus/bacteriophage T7 hybrid expression system. Proc. Nat. Acad. Sci. USA 86, 6126-6130.[Abstract/Free Full Text]

Gan, K. H., Veeze, H. J., van den Ouweland, A. M., Halley, D. J., Scheffer, H., van der Hout, A., Overbeek, S. E., de Jongste, J. C., Bakker, W. and Heijerman, H. G (1995). A cystic fibrosis mutation associated with mild lung disease. New Engl. J. Med 333, 95-99.[Abstract/Free Full Text]

Gregory, R. J., Cheng, S. H., Rich, D. P., Marshall, J., Paul, S., Hehir, K., Ostedgaard, L., Klinger, K. W., Welsh, M. J. and Smith, A. E (1990). Expression and characterization of the cystic fibrosis transmembrane conductance regulator. Nature 347, 382-386.[Medline]

Hartl, F. U (1996). Molecular chaperones in cellular protein folding. Nature 381, 571-580.[Medline]

Hobman, T. C., Woodward, L. and Farquhar, M. G (1992). The rubella virus E1 glycoprotein is arrested in a novel post-ER, pre-golgi compartment. J. Cell Biol 118, 795-811.[Abstract/Free Full Text]

Ishihara, N., Yamashina, S., Sakaguchi, M., Mihara, K. and Omura, T (1995). Malfolded cytochrome P-450(M1) localized in unusual membrane structures of the endoplasmic reticulum in cultured animal cells. J. Biochem. (Tokyo) 118, 397-404.[Abstract/Free Full Text]

Kaetzel, C. S., Rao, C. K. and Lamm, M. E (1987). Protein disulphilde-isomerase from human placenta and rat liver. Biochem. J 241, 39-47.[Medline]

Kerem, B. S., Zielenski, J., Markiewicz, D., Bozon, D., Gazit, E., Yahav, J., Kennedy, D., Riordan, J. R., Collins, F. S., Rommens, J. M. et al (1990). Identification of mutations in regions corresponding to the two putative nucleotide (ATP)-binding folds of the cystic fibrosis gene. Proc. Nat. Acad. Sci. USA 87, 8447-8451.[Abstract/Free Full Text]

Kerem, E., Corey, M., Kerem, B. S., Rommens, J., Markiewicz, D., Levison, H., Tsui, L. C. and Durie, P (1990). The relation between genotype and phenotype in cystic fibrosis\320analysis of the most common mutation (delta F508). New Eng. J. Med 323, 1517-1522.[Abstract]

Kristidis, P., Bozon, D., Corey, M., Markiewicz, D., Rommens, J., Tsui, L.-C. and Durie, P (1992). Genetic determination of exocrine pancreatic function in cystic fibrosis. Am. J. Hum. Genet 50, 1178-1184.[Medline]

Kunkel, T. A (1985). Rapid and efficient site-specific mutagenesis without phenotypic selection. Proc. Nat. Acad. Sci. USA 82, 488-492.[Abstract/Free Full Text]

Lehrich, R. W., Aller, S. G., Webster, P., Marino, C. R. and Forrest, J. N. Jr (1998). Vasoactive intestinal peptide, forskolin and genistein increase apical CFTR trafficking in the rectal gland of the spiny dogfish, Squalus acanthias. J. Clin. Invest 101, 737-745.[Medline]

Lukacs, G. L., Chang, X.-B., Bear, C., Kartner, N., Mohamed, A., Riordan, J. R. and Grinstein, S (1993). TheF508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane. Determination of functional half-lives on transfected cells. J. Biol. Chem 268, 21592-21598.[Abstract/Free Full Text]

Lukacs, G. L., Mohamed, A., Kartner, N., Chang, X. B., Riordan, J. R. and Grinstein, S (1994). Conformational maturation of CFTR but not its mutant counterpart (F508) occurs in the endoplasmic reticulum and requires ATP. EMBO J 13, 6076-6086.[Medline]

Misumi, Y., Miki, K., Takatsuki, A., Tamura, G. and Ikehara, Y (1986). Novel blockade by brefeldin A of intracellular transport of secretory proteins in cultured rat hepatocytes. J. Biol. Chem 261, 11398-11403.[Abstract/Free Full Text]

Ostedgaard, L. S. and Welsh, M. J (1992). Partial purification of the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem 267, 26142-26149.[Abstract/Free Full Text]

Parrish, M. L., Sengstag, C., Rine, J. D. and Wright, R. L (1995). Identification of the sequences in HMG-CoA reductase required for karmellae assembly. Mol. Biol. Cell 6, 1535-1547.[Abstract]

Pind, S., Riordan, J. R. and Williams, D. B (1994). 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.[Abstract/Free Full Text]

Qu, B.-H. and Thomas, P. J (1996). Alteration of the cystic fibrosis transmembrane conductance regulator folding pathway. J. Biol. Chem 271, 7261-7264.[Abstract/Free Full Text]

Qu, B.-H., Strickland, E. H. and Thomas, P. J (1997). Localization and suppression of a kinetic defect in cystic fibrosis transmembrane conductance regulator folding. J. Biol. Chem 272, 15739-15744.[Abstract/Free Full Text]

Raposo, G., van Santen, H. M., Leijendekker, R., Geuze, H. J. and Ploegh, H. L (1995). Misfolded major histocompatibility complex class I molecules accumulate in an expanded ER-Golgi intermediate compartment. J. Cell Biol 131, 1403-1419.[Abstract/Free Full Text]

Rich, D. P., Anderson, M. P., Gregory, R. J., Cheng, S. H., Paul, S., Jefferson, D. M., McCann, J. D., Klinger, K. W., Smith, A. E. and Welsh, M. J (1990). Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells. Nature 347, 358-363.[Medline]

Riordan, J. R (1993). The cystic fibrosis transmembrane conductance regulator. Annu. Rev. Physiol 55, 609-630.[Medline]

Sato, S., Ward, C. L., Krouse, M. E., Wine, J. J. and Kopito, R. R (1996). Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation. J. Biol. Chem 271, 635-638.[Abstract/Free Full Text]

Sheppard, D. N., Ostedgaard, L. S., Winter, M. C. and Welsh, M. J (1995). Mechanism of dysfunction of two nucleotide binding domain mutations in cystic fibrosis transmembrane conductance regulator that are associated with pancreatic sufficiency. EMBO J 14, 876-883.[Medline]

Tsui, L. C (1995). The cystic fibrosis transmembrane conductance regulator gene. Am. J. Respir. Crit. Care Med 151, 47-.[Abstract]

van den Hoff, M. J., Moorman, A. F. and Lamers, W. H (1992). Electroporation in \324intracellular' buffer increases cell survival. Nucl. Acids Res 20, 2902-.[Free Full Text]

Veeze, H. J., Halley, D. J. J., Bijman, J., de Jongste, J. C., de Jonge, H. R. and Sinaasappel, M (1994). Determinants of mild clinical symptoms in cystic fibrosis patients. Residual chloride secretion measured in rectal biopsies in relation to the genotype. J. Clin. Invest 93, 461-466.

Ward, C. L. and Kopito, R. R (1994). 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.[Abstract/Free Full Text]

Welsh, M. J. and Ostedgaard, L. S (1998). Cystic fibrosis problem probed by proteolysis. Nature Struct. Biol 5, 167-169.[Medline]

Wright, R., Basson, M., D'Ari, L. and Rine, J (1988). Increased amounts of HMG-CoA reductase induce \324karmellae': a proliferation of stacked membrane pairs surrounding the yeast nucleus. J. Cell Biol 107, 101-114.[Abstract/Free Full Text]

Yamamoto, A., Masaki, R. and Tashiro, Y (1996). Formation of crystalloid endoplasmic reticulum in COS cells upon overexpression of microsomal aldehyde dehydrogenase by cDNA transfection. J. Cell Sci 109, 1727-1738.[Abstract]

Yang, Y., Janich, S., Cohn, J. A. and Wilson, J. M (1993). The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment. Proc. Nat. Acad. Sci. USA 90, 9480-9484.[Abstract/Free Full Text]

Zhang, F., Kartner, N. and Lukacs, G. L (1998). Limited proteolysis as a probe for arrested conformational maturation ofF508 CFTR. Nature Struct. Biol 5, 180-183.[Medline]

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Articles by Ostedgaard, L. S.

Articles by Welsh, M. J.

				S. L. Farmen, P. H. Karp, P. Ng, D. J. Palmer, D. R. Koehler, J. Hu, A. L. Beaudet, J. Zabner, and M. J. Welsh Gene transfer of CFTR to airway epithelia: low levels of expression are sufficient to correct Cl- transport and overexpression can generate basolateral CFTR Am J Physiol Lung Cell Mol Physiol, December 1, 2005; 289(6): L1123 - L1130. [Abstract] [Full Text] [PDF]

				A. L. Berger, C. O. Randak, L. S. Ostedgaard, P. H. Karp, D. W. Vermeer, and M. J. Welsh Curcumin Stimulates Cystic Fibrosis Transmembrane Conductance Regulator Cl- Channel Activity J. Biol. Chem., February 18, 2005; 280(7): 5221 - 5226. [Abstract] [Full Text] [PDF]

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