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Pharmacological Modulation of the Retinal Unfolded Protein Response in Bardet-Biedl Syndrome Reduces Apoptosis and Preserves Light Detection Ability

Ciliopathies, a class of rare genetic disorders, present often with retinal degeneration caused by protein transport defects between the inner segment and the outer segment of the photoreceptors. Bardet-Biedl syndrome is one such ciliopathy, genetically heterogeneous with 17 BBS genes identified to...

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Main Authors: Mockel, Anais, Obringer, Cathy, Hakvoort, Theodorus B. M., Seeliger, Mathias, Lamers, Wouter H., Stoetzel, Corinne, Dollfus, Hélène, Marion, Vincent
格式: Artigo
語言:Inglês
出版: American Society for Biochemistry and Molecular Biology 2012
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在線閱讀:https://ncbi.nlm.nih.gov/pmc/articles/PMC3481343/
https://ncbi.nlm.nih.gov/pubmed/22869374
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M112.386821
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