Strategies for Correcting the AF508 CFTR Protein-Folding Defect

[1]  W. Welch,et al.  Correcting temperature-sensitive protein folding defects. , 1997, The Journal of clinical investigation.

[2]  S. Prusiner,et al.  Chemical chaperones interfere with the formation of scrapie prion protein. , 1996, The EMBO journal.

[3]  M. Drumm,et al.  Activation of endogenous deltaF508 cystic fibrosis transmembrane conductance regulator by phosphodiesterase inhibition. , 1996, The Journal of clinical investigation.

[4]  J. Riordan,et al.  cAMP- and Ca2+-independent Activation of Cystic Fibrosis Transmembrane Conductance Regulator Channels by Phenylimidazothiazole Drugs* , 1996, The Journal of Biological Chemistry.

[5]  R. Gibson,et al.  Repeat administration of an adenovirus vector encoding cystic fibrosis transmembrane conductance regulator to the nasal epithelium of patients with cystic fibrosis. , 1996, The Journal of clinical investigation.

[6]  J. Wine,et al.  Glycerol Reverses the Misfolding Phenotype of the Most Common Cystic Fibrosis Mutation (*) , 1996, The Journal of Biological Chemistry.

[7]  P. Pedersen,et al.  Defective protein folding as a basis of human disease. , 1995, Trends in biochemical sciences.

[8]  K. Jacobson,et al.  A1 receptor antagonist 8-cyclopentyl-1,3-dipropylxanthine selectively activates chloride efflux from human epithelial and mouse fibroblast cell lines expressing the cystic fibrosis transmembrane regulator delta F508 mutation. , 1995, Biochemistry.

[9]  K. Jacobson,et al.  Stimulation by alkylxanthines of chloride efflux in CFPAC-1 cells does not involve A1 adenosine receptors. , 1995, Biochemistry.

[10]  J. Riordan,et al.  Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator. , 1994, The Journal of biological chemistry.

[11]  D. Chowdary,et al.  Accumulation of p53 in a mutant cell line defective in the ubiquitin pathway , 1994, Molecular and cellular biology.

[12]  Matthew P. Anderson,et al.  Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive , 1992, Nature.

[13]  F. Collins,et al.  Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes. , 1991, Science.

[14]  A. Hamosh,et al.  Severe deficiency of cystic fibrosis transmembrane conductance regulator messenger RNA carrying nonsense mutations R553X and W1316X in respiratory epithelial cells of patients with cystic fibrosis. , 1991, The Journal of clinical investigation.

[15]  M. Burg,et al.  Renal medullary organic osmolytes. , 1991, Physiological reviews.

[16]  J. Marshall,et al.  Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis , 1990, Cell.

[17]  L. Tsui,et al.  Erratum: Identification of the Cystic Fibrosis Gene: Genetic Analysis , 1989, Science.

[18]  L. Tsui,et al.  Erratum: Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNA , 1989, Science.

[19]  J. Riordan,et al.  Identification of the Cystic Fibrosis Gene : Chromosome Walking and Jumping Author ( s ) : , 2008 .

[20]  L. Hightower,et al.  Inhibition of heat shock (stress) protein induction by deuterium oxide and glycerol: Additional support for the abnormal protein hypothesis of induction , 1989, Journal of cellular physiology.

[21]  K. Gekko,et al.  Increased thermal stability of collagen in the presence of sugars and polyols. , 1983, Journal of biochemistry.

[22]  R. Higashikubo,et al.  Protection against heat-induced cell killing by polyols in vitro. , 1983, Cancer research.

[23]  M. E. Clark,et al.  Living with water stress: evolution of osmolyte systems. , 1982, Science.

[24]  P. Lin,et al.  Protection of heat induced cytoxicity by glycerol , 1981, Journal of cellular physiology.

[25]  K. Gekko,et al.  Thermodynamic and kinetic examination of protein stabilization by glycerol. , 1981, Biochemistry.

[26]  K. Gekko,et al.  Mechanism of protein stabilization by glycerol: preferential hydration in glycerol-water mixtures. , 1981, Biochemistry.

[27]  D Oakenfull,et al.  Increased thermal stability of proteins in the presence of sugars and polyols. , 1979, Biochemistry.

[28]  D. Hawthorne,et al.  OSMOTIC-REMEDIAL MUTANTS. A NEW CLASSIFICATION FOR NUTRITIONAL MUTANTS IN YEAST. , 1964, Genetics.