Evidence of CFTR Function in Cystic Fibrosis after Systemic Administration of 4-Phenylbutyrate-Reference-Cited by-同舟云学术

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Evidence of CFTR Function in Cystic Fibrosis after Systemic Administration of 4-Phenylbutyrate

Published:2002-07 Issue:1 Volume:6 Page:119-126
ISSN:1525-0016
Container-title:Molecular Therapy
language:en
Short-container-title:Molecular Therapy

Author:

Zeitlin Pamela L.,Diener-West Marie,Rubenstein Ronald C.,Boyle Michael P.,Lee Carlton K.K.,Brass-Ernst Lois

Publisher

Elsevier BV

Subject

Drug Discovery,Pharmacology,Genetics,Molecular Biology,Molecular Medicine

Reference28 articles.

1. Mislocalization of ΔF508 CFTR in cystic fibrosis sweat gland;Kartner;Nat. Genet.,1992

2. Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis;Cheng;Cell,1990

3. Multiple proteolytic systems, including the proteasome, contribute to CFTR processing;Jensen;Cell,1995

4. Chemical chaperones correct the mutant phenotype of the ΔF508 cystic fibrosis transmembrane conductance regulator protein;Brown;Cell Stress Chaperones,1996

5. Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive;Denning;Nature,1992

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