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

PURPOSE. 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...

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Détails bibliographiques
Auteurs principaux: Zhang, Tao, Baehr, Wolfgang, Fu, Yingbin
Format: Artigo
Langue:Inglês
Publié: The Association for Research in Vision and Ophthalmology 2012
Sujets:
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC3385966/
https://ncbi.nlm.nih.gov/pubmed/22531707
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1167/iovs.12-9851
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