Journal
BRAIN RESEARCH
Volume 1648, Issue -, Pages 538-541Publisher
ELSEVIER SCIENCE BV
DOI: 10.1016/j.brainres.2016.04.021
Keywords
Photoreceptor; Retinitis pigmentosa; Achromatopsia; Rhodopsin; ATF6; Retina
Categories
Funding
- NEI NIH HHS [R01 EY020846, P30 EY022589] Funding Source: Medline
- NINDS NIH HHS [R01 NS088485] Funding Source: Medline
- BLRD VA [I01 BX002284] Funding Source: Medline
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Photoreceptors are specialized sensory neurons essential for light detection in the human eye. Photoreceptor cell dysfunction and death cause vision loss in many eye diseases such as retinitis pigmentosa and achromatopsia. Endoplasmic reticulum (ER) stress and Unfolded Protein Response (UPR) signaling have been implicated in the development and pathology of heritable forms of retinitis pigmentosa and achromatopsia. We review the role of ER stress and UPR in retinitis pigmentosa arising from misfolded rhodopsins (RHO) and in achromatopsia arising from genetic mutations in Activating Transcription Factor 6 (ATF6). This article is part of a Special Issue entitled SI:ER stress. Published by Elsevier B.V.
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