Myelination is delayed during postnatal brain development in the mdx mouse model of Duchenne muscular dystrophy.

ABSTRACT:

Background

In Duchenne muscular dystrophy (DMD), the loss of the dystrophin component of the dystrophin-glycoprotein complex (DGC) compromises plasma membrane integrity in skeletal muscle, resulting in extensive muscle degeneration. In addition, many DMD patients exhibit brain deficits in which the cellular etiology remains poorly understood. We recently found that dystroglycan, a receptor component of the DGC that binds intracellularly to dystrophin, regulates the development of oligodendrocytes, the myelinating glial cells of the brain.

Results

We investigated whether dystrophin contributes to oligodendroglial function and brain myelination. We found that oligodendrocytes express up to three dystrophin isoforms, in conjunction with classic DGC components, which are develop

SUBMITTER: Aranmolate A 

PROVIDER: S-EPMC5556620 | biostudies-literature | 2017 Aug

REPOSITORIES: biostudies-literature