Article
Suppression of retinal degeneration in Drosophila by stimulation of ER-associated degradation.
Proceedings of the National Academy of Sciences of the United States of America - 6 Oct 2009
Kang Min-Ji, Ryoo Hyung Don
Abstract excerpt
Mutations in the rhodopsin gene that disrupt the encoded protein's folding properties are a major cause of autosomal dominant retinitis pigmentosa (ADRP). This disease is faithfully modeled in Drosophila where similar mutations in the ninaE gene, encoding rhodopsin-1 (Rh-1), cause ER stress and dominantly trigger age-related retinal degeneration. In addition, mutant flies bearing certain ninaE alleles have...
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