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

Citation
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
Citations number
64
Categorie Soggetti
Neurosciences & Behavoir
Journal title
MOLECULAR AND CELLULAR NEUROSCIENCE
ISSN journal
10447431 → ACNP
Volume
17
Issue
5
Year of publication
2001
Pages
908 - 920
Database
ISI
SICI code
1044-7431(200105)17:5<908:DDOTMO>2.0.ZU;2-0
Abstract
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.