메뉴 건너뛰기




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

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

Author keywords

[No Author keywords available]

Indexed keywords

CHAPERONE; CHLORIDE; GLYCEROL; TRANSMEMBRANE CONDUCTANCE REGULATOR;

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
Times cited : (375)

References (30)
  • 1
    • 0026561138 scopus 로고
    • Intracellular protein trafficking defects in human disease
    • Amara JF, Cheng SH and Smith AE (1992) Intracellular protein trafficking defects in human disease. Trends Cell Biol. 2, 145-149.
    • (1992) Trends Cell Biol. , vol.2 , pp. 145-149
    • Amara, J.F.1    Cheng, S.H.2    Smith, A.E.3
  • 2
    • 0025868103 scopus 로고
    • Demonstration that CFTR is a chloride channel by alteration of its anion selectivity
    • 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.
    • (1991) Science , vol.253 , pp. 202-205
    • Anderson, M.P.1    Gregory, R.J.2    Thompson, S.3
  • 3
    • 0018789680 scopus 로고
    • Increased thermal stability of proteins in the presence of sugars and polyols
    • Back JF, Oakenfull D and Smith MB (1979) Increased thermal stability of proteins in the presence of sugars and polyols. Biochem. 18, 5191-5199.
    • (1979) Biochem. , vol.18 , pp. 5191-5199
    • Back, J.F.1    Oakenfull, D.2    Smith, M.B.3
  • 4
    • 0028859841 scopus 로고
    • Folding intermediates are involved in genetic diseases?
    • Bychkova VE and Ptitsyn OB (1995) Folding intermediates are involved in genetic diseases? FEBS Lett. 359, 6-8.
    • (1995) FEBS Lett. , vol.359 , pp. 6-8
    • Bychkova, V.E.1    Ptitsyn, O.B.2
  • 5
    • 0025242929 scopus 로고
    • Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
    • 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.
    • (1990) Cell , vol.63 , pp. 827-834
    • Cheng, S.H.1    Gregory, R.J.2    Marshall, J.3
  • 6
    • 0026781952 scopus 로고
    • Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature sensitive
    • 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.
    • (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
  • 7
    • 0024596704 scopus 로고
    • Inhibition of heat shock (stress) protein induction by deuterium oxide and glycerol: Additional support for the abnormal protein hypothesis of induction
    • 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.
    • (1989) J. Cell. Physiol. , vol.139 , pp. 219-228
    • Edington, B.V.1    Whelan, S.A.2    Hightower, L.E.3
  • 8
    • 0020546424 scopus 로고
    • Increased thermal stability of collagen in the presence of sugars and polyols
    • Gekko K and Koga S (1983) Increased thermal stability of collagen in the presence of sugars and polyols. J. Biochem. 94, 199-208.
    • (1983) J. Biochem. , vol.94 , pp. 199-208
    • Gekko, K.1    Koga, S.2
  • 9
    • 0019872622 scopus 로고
    • Mechanisms of protein stabilization by glycerol: Preferential hydration in glycerol-water mixtures
    • Gekko K and Timasheff SN (1981a) Mechanisms of protein stabilization by glycerol: Preferential hydration in glycerol-water mixtures. Biochem. 20, 4667-4676.
    • (1981) Biochem. , vol.20 , pp. 4667-4676
    • Gekko, K.1    Timasheff, S.N.2
  • 10
    • 0019872612 scopus 로고
    • Thermodynamic and kinetic examination of protein stabilization by glycerol
    • Gekko K and Timasheff SN (1981b) Thermodynamic and kinetic examination of protein stabilization by glycerol. Biochem. 20, 4677-4686.
    • (1981) Biochem. , vol.20 , pp. 4677-4686
    • Gekko, K.1    Timasheff, S.N.2
  • 11
    • 0000283208 scopus 로고
    • The effects of combining urea and an alcohol on the heat-induced reversible denaturation of ribonuclease
    • 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.
    • (1972) Int. J. Pept. Proteins Res. , vol.4 , pp. 372-378
    • Gerlsma, S.Y.1    Stuur, E.R.2
  • 12
    • 0025912486 scopus 로고
    • Maturation and function of cystic fibrosis transmembrane conductance regulator variants bearing mutations in putative nucleotide-binding domains 1 and 2
    • 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.
    • (1991) Mol. Cell. Biol. , vol.11 , pp. 3886-3893
    • Gregory, R.J.1    Rich, D.P.2    Cheng, S.H.3
  • 13
    • 0020528352 scopus 로고
    • Protection against heat-induced cell killing by polyols in vitro
    • Henle KJ, Peck JW and Higashikubo R (1983) Protection against heat-induced cell killing by polyols in vitro. Cancer Res. 43, 1624-1633.
    • (1983) Cancer Res. , vol.43 , pp. 1624-1633
    • Henle, K.J.1    Peck, J.W.2    Higashikubo, R.3
  • 14
    • 0028858161 scopus 로고
    • Multiple proteolytic systems, including the proteosome, contribute to CFTR processing
    • 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.
    • (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
  • 16
    • 0024423668 scopus 로고
    • Identification of the cystic fibrosis gene: Genetic analysis
    • Kerem BS, Rommens JM, Buchanan JA, et al (1989) Identification of the cystic fibrosis gene: genetic analysis. Science 245, 1073-1080.
    • (1989) Science , vol.245 , pp. 1073-1080
    • Kerem, B.S.1    Rommens, J.M.2    Buchanan, J.A.3
  • 17
    • 0019496278 scopus 로고
    • Protection of heat induced cytotoxicity by glycerol
    • Lin PS, Kwock L and Hefter K (1981) Protection of heat induced cytotoxicity by glycerol. J. Cell. Physiol. 108, 439-448.
    • (1981) J. Cell. Physiol. , vol.108 , pp. 439-448
    • Lin, P.S.1    Kwock, L.2    Hefter, K.3
  • 18
    • 0019248704 scopus 로고
    • Deuterium oxide protects against hyperthermic cell death. Evidence for role of interfacial water
    • 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.
    • (1980) Physiol. Chem. Phys. , vol.12 , pp. 369-376
    • McIver, D.J.L.1    Schurch, S.2    Sridhar, R.3
  • 19
    • 0028232167 scopus 로고
    • Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator
    • 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.
    • (1994) J. Biol. Chem. , vol.269 , pp. 12784-12788
    • Pind, S.1    Riordan, J.R.2    Williams, D.B.3
  • 20
    • 0024424270 scopus 로고
    • Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
    • 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.
    • (1989) Science , vol.245 , pp. 1066-1073
    • Riordan, J.R.1    Rommens, J.M.2    Keren, B.S.3
  • 21
    • 0024453308 scopus 로고
    • Identification of the cystic fibrosis gene: Chromosome walking and jumping
    • Rommens JM, Iannuzzi MC, Kerem BS, et al (1989) Identification of the cystic fibrosis gene: chromosome walking and jumping. Science 245, 1059-1065.
    • (1989) Science , vol.245 , pp. 1059-1065
    • Rommens, J.M.1    Iannuzzi, M.C.2    Kerem, B.S.3
  • 22
    • 0030042386 scopus 로고    scopus 로고
    • Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation
    • 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.
    • (1996) J Biol. Chem. , vol.271 , pp. 635-638
    • Sato, S.1    Ward, C.L.2    Krouse, M.E.3    Wine, J.L.4    Kopito, R.R.5
  • 23
    • 0022760562 scopus 로고
    • Protons, osmolytes and fitness of internal milieu for protein function
    • Somero GN (1986) Protons, osmolytes and fitness of internal milieu for protein function. Am J. Physiol. 251, R197-R213.
    • (1986) Am J. Physiol. , vol.251
    • Somero, G.N.1
  • 24
    • 0028856292 scopus 로고
    • Defective protein folding as a basis of human disease
    • Thomas PJ, Qu BH and Pedersen PL (1995) Defective protein folding as a basis of human disease. TIBS 20, 456-459.
    • (1995) TIBS , vol.20 , pp. 456-459
    • Thomas, P.J.1    Qu, B.H.2    Pedersen, P.L.3
  • 25
    • 0025032271 scopus 로고
    • Development and biological applications of chloride-sensitive fluorescent indicators
    • Verkman AS (1990) Development and biological applications of chloride-sensitive fluorescent indicators. Am. J. Physiol. 259, C375-C388.
    • (1990) Am. J. Physiol. , vol.259
    • Verkman, A.S.1
  • 26
    • 0012843026 scopus 로고
    • Chloride sensitive fluorescent indicators
    • Eds, J Kraicer and SJ Dixon, London, Academic Press
    • 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.
    • (1995) Methods in Neurosciences , vol.27 , pp. 328
    • Verkman, A.S.1    Biwersi, J.2
  • 27
    • 0028840915 scopus 로고
    • Degradation of CFTR by the ubiquitin-proteasome pathway
    • Ward CL and Kopito RR (1995) Degradation of CFTR by the ubiquitin-proteasome pathway. Cell 83, 121-127.
    • (1995) Cell , vol.83 , pp. 121-127
    • Ward, C.L.1    Kopito, R.R.2
  • 28
    • 0030154323 scopus 로고    scopus 로고
    • Influence of molecular and chemical chaperones on protein folding
    • Welch WJ and Brown CR (1996) Influence of molecular and chemical chaperones on protein folding. Cell Stress Chap. 1 (2), 109-115.
    • (1996) Cell Stress Chap. , vol.1 , Issue.2 , pp. 109-115
    • Welch, W.J.1    Brown, C.R.2
  • 29
    • 0025731899 scopus 로고
    • Inhibition of cell proliferation by glycerol
    • Wiebe JP and Dinsdale CJ (1991) Inhibition of cell proliferation by glycerol. Life Sci. 48, 1511-1517.
    • (1991) Life Sci. , vol.48 , pp. 1511-1517
    • Wiebe, J.P.1    Dinsdale, C.J.2
  • 30
    • 0027488993 scopus 로고
    • The common varient of cystic fibrosis transmembrane conductance regulator is recognized by Hsp 70 and degraded in a pre-golgi non-lysosomal compartment
    • 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.
    • (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


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.