C. Dalloz et al., Differential distribution of the members of the dystrophin glycoprotein complex in mouse retina: Effect of the mdx(3Cv) mutation, MOL CELL NE, 17(5), 2001, pp. 908-920
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.