A Stabilizing Influence: CAL PDZ Inhibition Extends the Half-Life of ΔF508-CFTR

被引：60

作者：

Cushing, Patrick R. ^{[2
]}

Vouilleme, Lars ^{[1
]}

Pellegrini, Maria ^{[3
]}

Boisguerin, Prisca ^{[1
]}

Madden, Dean R. ^{[2
]}

机构：

[1] Charite, Inst Med Immunol, D-10115 Berlin, Germany

[2] Dartmouth Med Sch, Dept Biochem, Hanover, NH 03755 USA

[3] Dartmouth Coll, Dept Chem, Hanover, NH 03755 USA

来源：

ANGEWANDTE CHEMIE-INTERNATIONAL EDITION | 2010年 / 49卷 / 51期

关键词：

cystic fibrosis; epithelial cells; ion channels; PDZ domain; protein-protein interactions; TRANSMEMBRANE-CONDUCTANCE-REGULATOR; CYSTIC-FIBROSIS; CFTR; MEMBRANE; PROTEIN; TRAFFICKING; ASSOCIATION; EXPRESSION; MECHANISM;

D O I：

10.1002/anie.201005585

中图分类号：

O6 [化学];

学科分类号：

0703 ;

摘要：

It pays to recycle! The most common mutation in cystic fibrosis (CF) impedes maturation and accelerates breakdown of the ion channel CFTR. A "stabilizer" has been characterized that blocks a PDZ domain responsible for CFTR degradation. The inhibitor iCAL36 extends the chloride channel's half-life in airway epithelial cells. It also complements the activity of a corrector of the maturation defect, thus suggesting the potential for combination CF therapies. Copyright © 2010 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

引用

页码：9907 / 9911

页数：5

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