Functional Rescue of Trafficking-Impaired ABCB4 Mutants by Chemical Chaperones

Volume: 11, Issue: 2, Pages: e0150098 - e0150098
Published: Feb 22, 2016
Abstract
Multidrug resistance protein 3 (MDR3, ABCB4) is a hepatocellular membrane protein that mediates biliary secretion of phosphatidylcholine. Null mutations in ABCB4 gene give rise to severe early-onset cholestatic liver disease. We have previously shown that the disease-associated mutations p.G68R, p.G228R, p.D459H, and p.A934T resulted in retention of ABCB4 in the endoplasmic reticulum, thus failing to target the plasma membrane. In the present...
Paper Details
Title
Functional Rescue of Trafficking-Impaired ABCB4 Mutants by Chemical Chaperones
Published Date
Feb 22, 2016
Journal
Volume
11
Issue
2
Pages
e0150098 - e0150098
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