Article
Suppression of wild-type rhodopsin maturation by mutants linked to autosomal dominant retinitis pigmentosa.
The Journal of biological chemistry - 14 Jan 2005
Rajan Rahul S, Kopito Ron R
Abstract excerpt
Autosomal dominant retinitis pigmentosa (ADRP) has been linked to mutations in the gene encoding rhodopsin. Most RP-linked rhodopsin mutants are unable to fold correctly in the endoplasmic reticulum, are degraded by the ubiquitin proteasome system, and are highly prone to forming detergent-insoluble high molecular weight aggregates. Here we have reported that coexpression of folding-deficient, but not...
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