【摘 要】
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Mutations that cause protein misfolding are implicated in conditions such as retinitis pigmentosa (RP), Usher Syndrome, and myocilin associated primary open angle glaucoma. The aggregation and continuous degradation of a highly abundant misfolded protein
【机 构】
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Department of Ophthalmology,University of Pittsburgh,Pittsburgh,PA,USA;Department of Pharmacology an
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Mutations that cause protein misfolding are implicated in conditions such as retinitis pigmentosa (RP), Usher Syndrome, and myocilin associated primary open angle glaucoma. The aggregation and continuous degradation of a highly abundant misfolded protein add proteolytic load of the affected cells. The subtle balance of cellular homeostasis, once disrupted by an overwhelmed proteolytic system, will lead to cell death and tissue degeneration. This perspective uses RHODOPSIN (RHO)-associated RP to review pharmacologic strategies for modifying protein misfolding-associated abnormalities with the goal of bringing insights to the treatment of other proteinopathies.
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