Chemical Chaperone TUDCA Preserves Cone Photoreceptors in a Mouse Model of Leber Congenital Amaurosis | Zendy

Tao� Zhang | Zendy; Wolfgang Baehr | Zendy; Yingbin Fu | Zendy

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Chemical 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.

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