Inactivation of VCP/ter94 suppresses retinal pathology caused by misfolded rhodopsin in Drosophila.

The most common Rhodopsin (Rh) mutation associated with autosomal dominant retinitis pigmentosa (ADRP) in North America is the substitution of proline 23 by histidine (Rh(P23H)). Unlike the wild-type Rh, mutant Rh(P23H) exhibits folding defects and forms intracellular aggregates. The mechanisms resp...

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Bibliographic Details
Main Authors: Ana Griciuc, Liviu Aron, Michel J Roux, Rüdiger Klein, Angela Giangrande, Marius Ueffing
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2010-08-01
Series:PLoS Genetics
Online Access:http://europepmc.org/articles/PMC2928793?pdf=render