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

• Main Authors: Carolina Uggenti (Author), Kit Briant (Author), Anne-Kathrin Streit (Author), Steven Thomson (Author), Yee Hui Koay (Author), Richard A. Baines (Author), Eileithyia Swanton (Author), Forbes D. Manson (Author)
• Format: Book
• Published: The Company of Biologists, 2016-11-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

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...