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Volumn 22, Issue 9, 2008, Pages 3255-3263

Chaperone displacement from mutant cystic fibrosis transmembrane conductance regulator restores its function in human airway epithelia

Author keywords

Aha1; CFTR; PTD; Rescue

Indexed keywords

ANION; ANION CHANNEL; CHAPERONE; CYCLIC AMP; HEAT SHOCK PROTEIN 70; HEAT SHOCK PROTEIN 90; PHENYLALANINE; PROTEASOME; TRANSMEMBRANE CONDUCTANCE REGULATOR; UBIQUITIN;

EID: 51349113842     PISSN: 08926638     EISSN: None     Source Type: Journal    
DOI: 10.1096/fj.07-105338     Document Type: Article
Times cited : (53)

References (48)
  • 1
  • 2
    • 0346505482 scopus 로고    scopus 로고
    • New concepts of the pathogenesis of cystic fibrosis lung disease
    • Boucher, R. C. (2004) New concepts of the pathogenesis of cystic fibrosis lung disease. Eur. Respir. J. 23, 146-158
    • (2004) Eur. Respir. J , vol.23 , pp. 146-158
    • Boucher, R.C.1
  • 4
    • 0035693855 scopus 로고    scopus 로고
    • Cystic fibrosis
    • Davis, P. B. (2001) Cystic fibrosis. Pediatr. Rev. 22, 257-264
    • (2001) Pediatr. Rev , vol.22 , pp. 257-264
    • Davis, P.B.1
  • 5
    • 0025242929 scopus 로고
    • Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
    • 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 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    Paul, S.4    Souza, D.W.5    White, G.A.6    O'Riordan, C.R.7    Smith, A.E.8
  • 6
    • 0027488993 scopus 로고
    • The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment
    • 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. Natl. Acad. Sci. U. S. A. 90, 9480-9484
    • (1993) Proc. Natl. Acad. Sci. U. S. A , vol.90 , pp. 9480-9484
    • Yang, Y.1    Janich, S.2    Cohn, J.A.3    Wilson, J.M.4
  • 7
    • 0032401771 scopus 로고    scopus 로고
    • Perturbation of Hsp90 interaction with nascent CFTR prevents its maturation and accelerates its degradation by the proteasome
    • Loo, M. A., Jensen, T. J., Cui, L., Hou, Y., Chang, X. B., and Riordan, J. R. (1998) Perturbation of Hsp90 interaction with nascent CFTR prevents its maturation and accelerates its degradation by the proteasome. EMBO J. 17, 6879-6887
    • (1998) EMBO J , vol.17 , pp. 6879-6887
    • Loo, M.A.1    Jensen, T.J.2    Cui, L.3    Hou, Y.4    Chang, X.B.5    Riordan, J.R.6
  • 8
    • 0033559258 scopus 로고    scopus 로고
    • The Hdj-2/Hsc70 chaperone pair facilitates early steps in CFTR biogenesis
    • Meacham, G. C., Lu, Z., King, S., Sorscher, E., Tousson, A., and Cyr, D. M. (1999) The Hdj-2/Hsc70 chaperone pair facilitates early steps in CFTR biogenesis. EMBO J. 18, 1492-1505
    • (1999) EMBO J , vol.18 , pp. 1492-1505
    • Meacham, G.C.1    Lu, Z.2    King, S.3    Sorscher, E.4    Tousson, A.5    Cyr, D.M.6
  • 9
    • 0034813890 scopus 로고    scopus 로고
    • Chaperoning the maturation of the cystic fibrosis transmembrane conductance regulator
    • Brodsky, J. L. (2001) Chaperoning the maturation of the cystic fibrosis transmembrane conductance regulator. Am. J. Physiol. Lung Cell. Mol. Physiol. 281, L39-L42
    • (2001) Am. J. Physiol. Lung Cell. Mol. Physiol , vol.281
    • Brodsky, J.L.1
  • 10
    • 0028840915 scopus 로고
    • Degradation of CFTR by the ubiquitin-proteasome pathway
    • Ward, C. L., Omura, S., and Kopito, R. R. (1995) Degradation of CFTR by the ubiquitin-proteasome pathway. Cell 83, 121-127
    • (1995) Cell , vol.83 , pp. 121-127
    • Ward, C.L.1    Omura, S.2    Kopito, R.R.3
  • 11
    • 0028858161 scopus 로고
    • Multiple proteolytic systems, including the proteasome, contribute to CFTR processing
    • Jensen, T. J., Loo, M. A., Pind, S., Williams, D. B., Goldberg, A. L., and Riordan, J. R. (1995) Multiple proteolytic systems, including the proteasome, 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
  • 12
    • 0345465663 scopus 로고    scopus 로고
    • Integration of endoplasmic reticulum signaling in health and disease
    • Aridor, M., and Balch, W. E. (1999) Integration of endoplasmic reticulum signaling in health and disease. Nat. Med. 5, 745-751
    • (1999) Nat. Med , vol.5 , pp. 745-751
    • Aridor, M.1    Balch, W.E.2
  • 13
    • 0028006681 scopus 로고
    • Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins
    • 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
    • (1994) J. Biol. Chem , vol.269 , pp. 25710-25718
    • Ward, C.L.1    Kopito, R.R.2
  • 15
    • 0031915434 scopus 로고    scopus 로고
    • Limited proteolysis as a probe for arrested conformational maturation of delta F508 CFTR
    • Zhang, F., Kartner, N., and Lukacs, G. L. (1998) Limited proteolysis as a probe for arrested conformational maturation of delta F508 CFTR. Nat. Struct. Biol. 5, 180-183
    • (1998) Nat. Struct. Biol , vol.5 , pp. 180-183
    • Zhang, F.1    Kartner, N.2    Lukacs, G.L.3
  • 16
    • 24644464284 scopus 로고    scopus 로고
    • Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening
    • Pedemonte, N., Lukacs, G. L., Du, K., Caci, E., Zegarra-Moran, O., Galietta, L. J., and Verkman, A. S. (2005) Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening. J. Clin. Invest. 115, 2564-2571
    • (2005) J. Clin. Invest , vol.115 , pp. 2564-2571
    • Pedemonte, N.1    Lukacs, G.L.2    Du, K.3    Caci, E.4    Zegarra-Moran, O.5    Galietta, L.J.6    Verkman, A.S.7
  • 18
    • 36348989763 scopus 로고    scopus 로고
    • Correctors promote maturation of cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by binding to the protein
    • Wang, Y., Loo, T. W., Bartlett, M. C., and Clarke, D. M. (2007) Correctors promote maturation of cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by binding to the protein. J. Biol. Chem. 282, 33247-33251
    • (2007) J. Biol. Chem , vol.282 , pp. 33247-33251
    • Wang, Y.1    Loo, T.W.2    Bartlett, M.C.3    Clarke, D.M.4
  • 19
    • 0026781952 scopus 로고
    • Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive
    • 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
    • (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
  • 20
    • 0030042386 scopus 로고    scopus 로고
    • Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation
    • 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
    • (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
  • 21
    • 0142184471 scopus 로고    scopus 로고
    • Rescue of functional DeltaF508-CFTR channels by co-expression with truncated CFTR constructs in COS-1 cells
    • Owsianik, G., Cao, L., and Nilius, B. (2003) Rescue of functional DeltaF508-CFTR channels by co-expression with truncated CFTR constructs in COS-1 cells. FEBS Lett. 554, 173-178
    • (2003) FEBS Lett , vol.554 , pp. 173-178
    • Owsianik, G.1    Cao, L.2    Nilius, B.3
  • 22
    • 2942690233 scopus 로고    scopus 로고
    • A domain mimic increases DeltaF508 CFTR trafficking and restores cAMP-stimulated anion secretion in cystic fibrosis epithelia
    • Clarke, L. L., Gawenis, L. R., Hwang, T. C., Walker, N. M., Gruis, D. B., and Price, E. M. (2004) A domain mimic increases DeltaF508 CFTR trafficking and restores cAMP-stimulated anion secretion in cystic fibrosis epithelia. Am. J. Physiol. Cell. Physiol. 287, C192-199
    • (2004) Am. J. Physiol. Cell. Physiol , vol.287
    • Clarke, L.L.1    Gawenis, L.R.2    Hwang, T.C.3    Walker, N.M.4    Gruis, D.B.5    Price, E.M.6
  • 23
  • 24
    • 0034640510 scopus 로고    scopus 로고
    • Protein kinase A associates with cystic fibrosis transmembrane conductance regulator via an interaction with ezrin
    • Sun, F., Hug, M. J., Bradbury, N. A., and Frizzell, R. A. (2000) Protein kinase A associates with cystic fibrosis transmembrane conductance regulator via an interaction with ezrin. J. Biol. Chem. 275, 14360-14366
    • (2000) J. Biol. Chem , vol.275 , pp. 14360-14366
    • Sun, F.1    Hug, M.J.2    Bradbury, N.A.3    Frizzell, R.A.4
  • 25
    • 0034703069 scopus 로고    scopus 로고
    • E3KARP mediates the association of ezrin and protein kinase A with the cystic fibrosis transmembrane conductance regulator in airway cells
    • Sun, F., Hug, M. J., Lewarchik, C. M., Yun, C. H., Bradbury, N. A., and Frizzell, R. A. (2000) E3KARP mediates the association of ezrin and protein kinase A with the cystic fibrosis transmembrane conductance regulator in airway cells. J. Biol. Chem. 275, 29539-29546
    • (2000) J. Biol. Chem , vol.275 , pp. 29539-29546
    • Sun, F.1    Hug, M.J.2    Lewarchik, C.M.3    Yun, C.H.4    Bradbury, N.A.5    Frizzell, R.A.6
  • 26
    • 0029145960 scopus 로고
    • Intratracheal gene delivery with adenoviral vector induces elevated systemic IgG and mucosal IgA antibodies to adenovirus and beta-galactosidase
    • Van Ginkel, F. W., Liu, C., Simecka, J. W., Dong, J. Y., Greenway, T., Frizzell, R. A., Kiyono, H., McGhee, J. R., and Pascual, D. W. (1995) Intratracheal gene delivery with adenoviral vector induces elevated systemic IgG and mucosal IgA antibodies to adenovirus and beta-galactosidase. Hum. Gene Ther. 6, 895-903
    • (1995) Hum. Gene Ther , vol.6 , pp. 895-903
    • Van Ginkel, F.W.1    Liu, C.2    Simecka, J.W.3    Dong, J.Y.4    Greenway, T.5    Frizzell, R.A.6    Kiyono, H.7    McGhee, J.R.8    Pascual, D.W.9
  • 27
    • 0034296675 scopus 로고    scopus 로고
    • Characterization of a class of cationic peptides able to facilitate efficient protein transduction in vitro and in vivo
    • Mi, Z., Mai, J., Lu, X., and Robbins, P. D. (2000) Characterization of a class of cationic peptides able to facilitate efficient protein transduction in vitro and in vivo. Mol. Ther. 2, 339-347
    • (2000) Mol. Ther , vol.2 , pp. 339-347
    • Mi, Z.1    Mai, J.2    Lu, X.3    Robbins, P.D.4
  • 28
    • 0025801217 scopus 로고
    • Isolation and properties of nascent lipoproteins from highly purified rat hepatocytic Golgi fractions
    • Hamilton, R. L., Moorehouse, A., and Havel, R. J. (1991) Isolation and properties of nascent lipoproteins from highly purified rat hepatocytic Golgi fractions. J. Lipid Res. 32, 529-543
    • (1991) J. Lipid Res , vol.32 , pp. 529-543
    • Hamilton, R.L.1    Moorehouse, A.2    Havel, R.J.3
  • 29
    • 33846008398 scopus 로고    scopus 로고
    • Derlin-1 promotes the efficient degradation of the cystic fibrosis transmembrane conductance regulator (CFTR) and CFTR folding mutants
    • Sun, F., Zhang, R., Gong, X., Geng, X., Drain, P. F., and Frizzell, R. A. (2006) Derlin-1 promotes the efficient degradation of the cystic fibrosis transmembrane conductance regulator (CFTR) and CFTR folding mutants. J. Biol. Chem. 281, 36856-36863
    • (2006) J. Biol. Chem , vol.281 , pp. 36856-36863
    • Sun, F.1    Zhang, R.2    Gong, X.3    Geng, X.4    Drain, P.F.5    Frizzell, R.A.6
  • 30
    • 0033864526 scopus 로고    scopus 로고
    • Pharmacological modulation of ion transport across wild-type and DeltaF508 CFTR-expressing human bronchial epithelia
    • Devor, D. C., Bridges, R. J., and Pilewski, J. M. (2000) Pharmacological modulation of ion transport across wild-type and DeltaF508 CFTR-expressing human bronchial epithelia. Am. J. Physiol. Cell. Physiol. 279, C461-C479
    • (2000) Am. J. Physiol. Cell. Physiol , vol.279
    • Devor, D.C.1    Bridges, R.J.2    Pilewski, J.M.3
  • 32
    • 0033153764 scopus 로고    scopus 로고
    • Rescue of dysfunctional deltaF508-CFTR chloride channel activity by IBMX
    • Schultz, B. D., Frizzell, R. A., and Bridges, R. J. (1999) Rescue of dysfunctional deltaF508-CFTR chloride channel activity by IBMX. J. Membr. Biol. 170, 51-66
    • (1999) J. Membr. Biol , vol.170 , pp. 51-66
    • Schultz, B.D.1    Frizzell, R.A.2    Bridges, R.J.3
  • 34
    • 0029962053 scopus 로고    scopus 로고
    • In vitro assessment of variables affecting the efficiency and efficacy of adenovirus-mediated gene transfer to cystic fibrosis airway epithelia
    • Johnson, L. G., Pickles, R. J., Boyles, S. E., Morris, J. C., Ye, H., Zhou, Z., Olsen, J. C., and Boucher, R. C. (1996) In vitro assessment of variables affecting the efficiency and efficacy of adenovirus-mediated gene transfer to cystic fibrosis airway epithelia. Hum. Gene Ther. 7, 51-59
    • (1996) Hum. Gene Ther , vol.7 , pp. 51-59
    • Johnson, L.G.1    Pickles, R.J.2    Boyles, S.E.3    Morris, J.C.4    Ye, H.5    Zhou, Z.6    Olsen, J.C.7    Boucher, R.C.8
  • 36
    • 0033520487 scopus 로고    scopus 로고
    • In vivo protein transduction: Delivery of a biologically active protein into the mouse
    • Schwarze, S. R., Ho, A., Vocero-Akbani, A., and Dowdy, S. F. (1999) In vivo protein transduction: delivery of a biologically active protein into the mouse. Science 285, 1569-1572
    • (1999) Science , vol.285 , pp. 1569-1572
    • Schwarze, S.R.1    Ho, A.2    Vocero-Akbani, A.3    Dowdy, S.F.4
  • 37
    • 1942421714 scopus 로고    scopus 로고
    • Function of trigger factor and DnaK in multidomain protein folding: Increase in yield at the expense of folding speed
    • Agashe, V. R., Guha, S., Chang, H. C., Genevaux, P., Hayer-Hartl, M., Stemp, M., Georgopoulos, C., Hartl, F. U., and Barral, J. M. (2004) Function of trigger factor and DnaK in multidomain protein folding: increase in yield at the expense of folding speed. Cell 117, 199-209
    • (2004) Cell , vol.117 , pp. 199-209
    • Agashe, V.R.1    Guha, S.2    Chang, H.C.3    Genevaux, P.4    Hayer-Hartl, M.5    Stemp, M.6    Georgopoulos, C.7    Hartl, F.U.8    Barral, J.M.9
  • 38
    • 0037147191 scopus 로고    scopus 로고
    • Coordinated nonvectorial folding in a newly synthesized multidomain protein
    • Jansens, A., van Duijn, E., and Braakman, I. (2002) Coordinated nonvectorial folding in a newly synthesized multidomain protein. Science 298, 2401-2403
    • (2002) Science , vol.298 , pp. 2401-2403
    • Jansens, A.1    van Duijn, E.2    Braakman, I.3
  • 42
    • 38349125066 scopus 로고    scopus 로고
    • Building an understanding of cystic fibrosis on the foundation of ABC transporter structures
    • Mendoza, J. L., and Thomas, P. J. (2007) Building an understanding of cystic fibrosis on the foundation of ABC transporter structures. J. Bioenerg. Biomembr 39, 499-505
    • (2007) J. Bioenerg. Biomembr , vol.39 , pp. 499-505
    • Mendoza, J.L.1    Thomas, P.J.2
  • 43
    • 11444266284 scopus 로고    scopus 로고
    • The DeltaF508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR
    • Du, K., Sharma, M., and Lukacs, G. L. (2005) The DeltaF508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR. Nat. Struct. Mol. Biol. 12, 17-25
    • (2005) Nat. Struct. Mol. Biol , vol.12 , pp. 17-25
    • Du, K.1    Sharma, M.2    Lukacs, G.L.3
  • 45
    • 0036663014 scopus 로고    scopus 로고
    • In vivo delivery of a Bcl-xL fusion protein containing the TAT protein transduction domain protects against ischemic brain injury and neuronal apoptosis
    • Cao, G., Pei, W., Ge, H., Liang, Q., Luo, Y., Sharp, F. R., Lu, A., Ran, R., Graham, S. H., and Chen, J. (2002) In vivo delivery of a Bcl-xL fusion protein containing the TAT protein transduction domain protects against ischemic brain injury and neuronal apoptosis. J. Neurosci. 22, 5423-5431
    • (2002) J. Neurosci , vol.22 , pp. 5423-5431
    • Cao, G.1    Pei, W.2    Ge, H.3    Liang, Q.4    Luo, Y.5    Sharp, F.R.6    Lu, A.7    Ran, R.8    Graham, S.H.9    Chen, J.10
  • 46
    • 0033137321 scopus 로고    scopus 로고
    • Transduced p16INK4a peptides inhibit hypophosphorylation of the retinoblastoma protein and cell cycle progression prior to activation of Cdk2 complexes in late G1
    • Gius, D. R., Ezhevsky, S. A., Becker-Hapak, M., Nagahara, H., Wei, M. C., and Dowdy, S. F. (1999) Transduced p16INK4a peptides inhibit hypophosphorylation of the retinoblastoma protein and cell cycle progression prior to activation of Cdk2 complexes in late G1. Cancer Res. 59, 2577-2580
    • (1999) Cancer Res , vol.59 , pp. 2577-2580
    • Gius, D.R.1    Ezhevsky, S.A.2    Becker-Hapak, M.3    Nagahara, H.4    Wei, M.C.5    Dowdy, S.F.6
  • 47
    • 4344662352 scopus 로고    scopus 로고
    • Targeting p53 by PTD-mediated transduction
    • Wang, W., and El-Deiry, W. S. (2004) Targeting p53 by PTD-mediated transduction. Trends Biotechnol. 22, 431-434
    • (2004) Trends Biotechnol , vol.22 , pp. 431-434
    • Wang, W.1    El-Deiry, W.S.2
  • 48
    • 0242289417 scopus 로고    scopus 로고
    • Glucose-regulated stress proteins and antibacterial immunity
    • Rapp, U. K., and Kaufmann, S. H. (2003) Glucose-regulated stress proteins and antibacterial immunity. Trends Microbiol. 11, 519-526
    • (2003) Trends Microbiol , vol.11 , pp. 519-526
    • Rapp, U.K.1    Kaufmann, S.H.2


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