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J Ophthalmic Vis Res. 2012 Jan;7(1):45-59.

Endoplasmic Reticulum Stress and Unfolded Protein Response Pathways: Potential for Treating Age-related Retinal Degeneration.

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1
SUNY Eye Institute, Departments of Neuroscience and Physiology and Ophthalmology, SUNY Upstate Medical University, Syracuse, NY, USA.

Abstract

Accumulation of misfolded proteins in the endoplasmic reticulum (ER) and their aggregation impair normal cellular function and can be toxic, leading to cell death. Prolonged expression of misfolded proteins triggers ER stress, which initiates a cascade of reactions called the unfolded protein response (UPR). Protein misfolding is the basis for a variety of disorders known as ER storage or conformational diseases. There are an increasing number of eye disorders associated with misfolded proteins and pathologic ER responses, including retinitis pigmentosa (RP). Herein we review the basic cellular and molecular biology of UPR with focus on pathways that could be potential targets for treating retinal degenerative diseases.

KEYWORDS:

Age-related Retinal Degeneration; ER Stress; Misfolded Protein; Protein Aggregation; Retinal Degenerative Diseases; Unfolded Protein Response

PMID:
22737387
PMCID:
PMC3381108
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