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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 Ca(2+)-gated Cl(−) channel in the basolateral surface of the retinal pigment epithelium (RPE). Using a stably transfected polarised epithelial cell model, we...

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Detalles Bibliográficos
Autores principales: Uggenti, Carolina, Briant, Kit, Streit, Anne-Kathrin, Thomson, Steven, Koay, Yee Hui, Baines, Richard A., Swanton, Eileithyia, Manson, Forbes D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Company of Biologists Ltd 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5117222/
https://www.ncbi.nlm.nih.gov/pubmed/27519691
http://dx.doi.org/10.1242/dmm.024216