Restoration of mutant bestrophin-1 expression, localisation and function in a polarised epithelial cell model

Restoration of mutant bestrophin-1 expression, localisation and function in a polarised epithelial cell model

Autosomal recessive bestrophinopathy (ARB) is a retinopathy caused by mutations in the bestrophin-1 protein, which is thought to function as a Ca2+-gated Cl− channel in the basolateral surface of the retinal pigment epithelium (RPE). Using a stably transfected polarised epithelial cell model, we sho...

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Bibliographic Details
Main Authors: Carolina Uggenti, Kit Briant, Anne-Kathrin Streit, Steven Thomson, Yee Hui Koay, Richard A. Baines, Eileithyia Swanton, Forbes D. Manson
Format: Article
Language:English
Published: The Company of Biologists 2016-11-01
Series:Disease Models & Mechanisms
Subjects:
Autosomal recessive bestrophinopathy
Bestrophin-1
Chemical chaperone
4-phenylbutyrate
Online Access:http://dmm.biologists.org/content/9/11/1317

Internet

http://dmm.biologists.org/content/9/11/1317

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