Differential distribution of the members of the dystrophin glycoprotein complex in mouse retina: Effect of the mdx(3Cv) mutation

Dystrophin glycoprotein complex (DGC) assembly and function require mediati on by dystrophin in skeletal muscle. The existence of such complexes and th e correlation with DMD phenotypes are not yet established in the central ne rvous system. Here we have studied the expression of DMD gene mRNAs and pro teins in retina from C57BV/6 and mdx(3Cv) mouse strains. Then we have compa ratively investigated the localization of dystrophin and dystrophin-associa ted proteins (DAPs) in both strains to analyze the repercussion of the mdx( 3Cv) mutation on the retinal distributions of alpha/beta -dystroglycan, alp ha1-syntrophin, alpha -dystrobrevin, and delta/gamma -sarcoglycan. Results showed that DMD gene product deficiency affects the expression of dystrogly can assembly exclusively at the outer plexiform layer without an apparent e ffect on the other DAPs. We conclude that the localization of members of th e DGC could be independent of the presence of the DMD gene products and/or utrophin.