Inhibition of VCP preserves retinal structure and function in autosomal dominant retinal degeneration

Abstract
Due to continuously high production rates of rhodopsin (RHO) and high metabolic activity, photoreceptor neurons are especially vulnerable to defects in proteostasis. A proline to histidine substitution at position 23 (P23H) leads to production of structurally misfolded RHO, causing the most common form of autosomal dominant Retinitis Pigmentosa (adRP) in North America. The AAA-ATPase valosin-containing protein (VCP) extracts misfolded proteins...
Paper Details
Title
Inhibition of VCP preserves retinal structure and function in autosomal dominant retinal degeneration
Published Date
Nov 19, 2020
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