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ABSTRACT: Purpose
Misfolding mutations in rod opsin are a major cause of the inherited blindness retinitis pigmentosa. Therefore, understanding the role of molecular chaperones in facilitating rod opsin biogenesis and the response to mutant rod opsin is important for retinal disease and fundamental retinal cell biology. A recent report has shown that Drosophila rhodopsin Rh1 requires calnexin (Cnx) for its maturation and correct localization to R1-6 rhabdomeres. In this report, we investigate the role of Cnx in the processing of wild-type and mutant mammalian rod opsin.Methods
Mouse embryonic fibroblasts (MEFs) from control mice (WT) and mice that express a truncated dysfunctional version of Cnx (sCnx) were used to assess the role of Cnx in the biogenesis, maturation, degradation, an
SUBMITTER: Kosmaoglou M
PROVIDER: S-EPMC2610292 | biostudies-literature | 2008
REPOSITORIES: biostudies-literature