Chemical chaperones correct the mutant phenotype of the ΔF508 cystic fibrosis transmembrane conductance regulator protein

Cell Stress and Chaperones

Volumn 1, Issue 2, 1996, Pages 117-125

  Brown, C Randell ^a   Hong Brown, Ly Q ^a   Biwersi, Joachim ^a   Verkman, A S ^a   Welch, William J ^a  

^a UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CHAPERONE; CHLORIDE; GLYCEROL; TRANSMEMBRANE CONDUCTANCE REGULATOR;

ANIMAL; ARTICLE; CELL STRAIN 3T3; CYSTIC FIBROSIS; ELECTRIC CONDUCTIVITY; GENETICS; METABOLISM; MOUSE; MUTATION; PHENOTYPE; PROTEIN FOLDING; RABBIT;

3T3 CELLS; ANIMALS; CHLORIDES; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; ELECTRIC CONDUCTIVITY; GLYCEROL; MICE; MOLECULAR CHAPERONES; MUTATION; PHENOTYPE; PROTEIN FOLDING; RABBITS;

EID: 0030154620     PISSN: 13558145     EISSN: None     Source Type: Journal    
DOI: 10.1379/1466-1268(1996)001<0117:CCCTMP>2.3.CO;2     Document Type: Article

References (30)

1. Amara JF, Cheng SH and Smith AE (1992) Intracellular protein trafficking defects in human disease. Trends Cell Biol. 2, 145-149.
2. Anderson MP, Gregory RJ, Thompson S, et al (1991) Demonstration that CFTR is a chloride channel by alteration of its anion selectivity. Science 253, 202-205.
3. Back JF, Oakenfull D and Smith MB (1979) Increased thermal stability of proteins in the presence of sugars and polyols. Biochem. 18, 5191-5199.
4. Bychkova VE and Ptitsyn OB (1995) Folding intermediates are involved in genetic diseases? FEBS Lett. 359, 6-8.
5. Cheng SH, Gregory RJ, Marshall J, et al (1990) Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell 63, 827-834.
6. Denning GM, Anderson MP, Amara JF, Marshall J, Smith AE and Welsh MJ (1992) Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature sensitive. Nature 358, 761-764.
7. Edington BV, Whelan SA and Hightower LE (1989) Inhibition of heat shock (stress) protein induction by deuterium oxide and glycerol: Additional support for the abnormal protein hypothesis of induction. J. Cell. Physiol. 139, 219-228.
8. Gekko K and Koga S (1983) Increased thermal stability of collagen in the presence of sugars and polyols. J. Biochem. 94, 199-208.
9. Gekko K and Timasheff SN (1981a) Mechanisms of protein stabilization by glycerol: Preferential hydration in glycerol-water mixtures. Biochem. 20, 4667-4676.
10. Gekko K and Timasheff SN (1981b) Thermodynamic and kinetic examination of protein stabilization by glycerol. Biochem. 20, 4677-4686.
11. Gerlsma SY and Stuur ER (1972) The effects of combining urea and an alcohol on the heat-induced reversible denaturation of ribonuclease. Int. J. Pept. Proteins Res. 4, 372-378.
12. Gregory RJ, Rich DP, Cheng SH, et al (1991) 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.
13. Henle KJ, Peck JW and Higashikubo R (1983) Protection against heat-induced cell killing by polyols in vitro. Cancer Res. 43, 1624-1633.
14. Jensen TJ, Loo MA, Pind S, Williams DB, Goldberg AL and Riordan JR (1995) Multiple proteolytic systems, including the proteosome, contribute to CFTR processing. Cell 83, 129-135.
15. Kartner N, Augustinas O, Jensen TJ, Naismith AL and Riordan JR (1992) Mislocalization of ΔF508 CFTR in cystis fibrosis sweat gland. Nature Genetics 1, 321-326 (1992).
16. Kerem BS, Rommens JM, Buchanan JA, et al (1989) Identification of the cystic fibrosis gene: genetic analysis. Science 245, 1073-1080.
17. Lin PS, Kwock L and Hefter K (1981) Protection of heat induced cytotoxicity by glycerol. J. Cell. Physiol. 108, 439-448.
18. McIver DJL, Schurch S and Sridhar R (1980) Deuterium oxide protects against hyperthermic cell death. Evidence for role of interfacial water. Physiol. Chem. Phys. 12, 369-376.
19. Pind S, Riordan JR and Williams DB (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.
20. Riordan JR, Rommens JM, Keren BS, et al (1989) Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 245, 1066-1073.
21. Rommens JM, Iannuzzi MC, Kerem BS, et al (1989) Identification of the cystic fibrosis gene: chromosome walking and jumping. Science 245, 1059-1065.
22. Sato S, Ward CL, Krouse ME, Wine JL and Kopito RR (1996) Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation. J Biol. Chem. 271, 635-638.
23. Somero GN (1986) Protons, osmolytes and fitness of internal milieu for protein function. Am J. Physiol. 251, R197-R213.
24. Thomas PJ, Qu BH and Pedersen PL (1995) Defective protein folding as a basis of human disease. TIBS 20, 456-459.
25. Verkman AS (1990) Development and biological applications of chloride-sensitive fluorescent indicators. Am. J. Physiol. 259, C375-C388.
26. Verkman AS and Biwersi J (1995) Chloride sensitive fluorescent indicators. In: Methods in Neurosciences, vol. 27, Eds, J Kraicer and SJ Dixon, London, Academic Press, 328.
27. Ward CL and Kopito RR (1995) Degradation of CFTR by the ubiquitin-proteasome pathway. Cell 83, 121-127.
28. Welch WJ and Brown CR (1996) Influence of molecular and chemical chaperones on protein folding. Cell Stress Chap. 1 (2), 109-115.
29. Wiebe JP and Dinsdale CJ (1991) Inhibition of cell proliferation by glycerol. Life Sci. 48, 1511-1517.
30. Yang Y, Janich S, Cohn JA and Wilson JM (1993) The common varient of cystic fibrosis transmembrane conductance regulator is recognized by Hsp 70 and degraded in a pre-golgi non-lysosomal compartment. Proc. Natl. Acad. Sci. USA 90, 9480-9484.