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

摘要: In Duchenne muscular dystrophy (DMD), the loss of dystrophin component dystrophin-glycoprotein complex (DGC) compromises plasma membrane integrity in skeletal muscle, resulting extensive muscle degeneration. addition, many DMD patients exhibit brain deficits which cellular etiology remains poorly understood. We recently found that dystroglycan, a receptor DGC binds intracellularly to dystrophin, regulates development oligodendrocytes, myelinating glial cells brain. investigated whether contributes oligodendroglial function and myelination. oligodendrocytes express up three isoforms, conjunction with classic components, are developmentally regulated during differentiation response extracellular matrix engagement. mdx mice, model lacking expression largest isoform, have delayed myelination inappropriate oligodendrocyte progenitor proliferation cerebral cortex. When we prevented all isoforms cultured using RNA interference, later stages maturation were significantly delayed, similar phenotypes developing find is expressed influences developmental myelination, provides new insight into potential contributors dysfunction associated DMD.