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pubmed-article:20238009pubmed:dateCreated2010-3-18lld:pubmed
pubmed-article:20238009pubmed:abstractTextMany mutations associated with retinal degeneration lead to the production of misfolded proteins by cells of the retina. Emerging evidence suggests that these abnormal proteins cause cell death by activating the Unfolded Protein Response, a set of conserved intracellular signaling pathways that detect protein misfolding within the endoplasmic reticulum and control protective and proapoptotic signal transduction pathways. Here, we review the misfolded proteins associated with select types of retinitis pigmentosa, Stargadt-like macular degeneration, and Doyne Honeycomb Retinal Dystrophy and discuss the role that endoplasmic reticulum stress and UPR signaling play in their pathogenesis. Last, we review new therapies for these diseases based on preventing protein misfolding in the retina.lld:pubmed
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pubmed-article:20238009pubmed:issn0065-2598lld:pubmed
pubmed-article:20238009pubmed:authorpubmed-author:LavailMatthew...lld:pubmed
pubmed-article:20238009pubmed:authorpubmed-author:LinJonathan...lld:pubmed
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pubmed-article:20238009pubmed:volume664lld:pubmed
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pubmed-article:20238009pubmed:pagination115-21lld:pubmed
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pubmed-article:20238009pubmed:year2010lld:pubmed
pubmed-article:20238009pubmed:articleTitleMisfolded proteins and retinal dystrophies.lld:pubmed
pubmed-article:20238009pubmed:affiliationDepartment of Pathology, University of California, San Diego, CA 92093-0612, USA.lld:pubmed
pubmed-article:20238009pubmed:publicationTypeJournal Articlelld:pubmed
pubmed-article:20238009pubmed:publicationTypeReviewlld:pubmed
pubmed-article:20238009pubmed:publicationTypeResearch Support, Non-U.S. Gov'tlld:pubmed
pubmed-article:20238009pubmed:publicationTypeResearch Support, N.I.H., Extramurallld:pubmed