Open AccessChemical Chaperone TUDCA Preserves Cone Photoreceptors in a Mouse Model of Leber Congenital Amaurosis
Author(s) -
Tao Zhang,
Wolfgang Baehr,
Yingbin Fu
Publication year - 2012
Publication title -
investigative ophthalmology and visual science
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.935
H-Index - 218
eISSN - 1552-5783
pISSN - 0146-0404
DOI - 10.1167/iovs.12-9851
Subject(s) - rpe65 , tauroursodeoxycholic acid , endoplasmic reticulum , unfolded protein response , microbiology and biotechnology , retina , retinal degeneration , chemistry , biology , medicine , neuroscience , retinal pigment epithelium
Mutations in either retinoid isomerase (RPE65) or lecithin-retinol acyltransferase (LRAT) lead to Leber congenital amaurosis (LCA). By using the Lrat(-/-) mouse model, previous studies have shown that the rapid cone degeneration in LCA was caused by endoplasmic reticulum (ER) stress induced by S-opsin aggregation. The purpose of this study is to examine the efficacy of an ER chemical chaperone, tauroursodeoxycholic acid (TUDCA), in preserving cones in the Lrat(-/-) model.