CFTR function and prospects for therapy

被引：475

作者：

Riordan, John R. ^{[1
]}

机构：

[1] Univ N Carolina, Sch Med, Dept Biochem & Biophys, Cyst Fibrosis Treatment & Res Ctr, Chapel Hill, NC 27599 USA

来源：

ANNUAL REVIEW OF BIOCHEMISTRY | 2008年 / 77卷

关键词：

ABC protein; anion channel; cystic fibrosis; ER quality control; molecular therapy; protein misfolding;

D O I：

10.1146/annurev.biochem.75.103004.142532

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Mutations in the gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) epithelial anion channel cause cystic fibrosis (CF). The multidomain integral membrane glycoprotein, a member of the adenine nucleotide-bin ding cassette (ABC) transporter family, conserved in metazoan salt-transporting tissues, is required to control ion and fluid homeostasis on epithelial surfaces. This review considers different therapeutic strategies that have arisen from knowledge of CFTR structure and function as well as its biosynthetic processing, intracellular trafficking, and turnover.

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收藏

页码：701 / 726

页数：26

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