| First Author | Matsumura K | Year | 1992 |
| Journal | Nature | Volume | 360 |
| Issue | 6404 | Pages | 588-91 |
| PubMed ID | 1461282 | Mgi Jnum | J:3426 |
| Mgi Id | MGI:51939 | Doi | 10.1038/360588a0 |
| Citation | Matsumura K, et al. (1992) Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle. Nature 360(6404):588-91 |
| abstractText | Dystrophin is associated with a complex of muscle membrane (sarcolemmal) glycoproteins that provide a linkage to the extracellular matrix protein, laminin. The absence of dystrophin leads to a dramatic reduction of the dystrophin-associated proteins (156DAG, 59DAP, 50DAG, 43DAG and 35DAG) in the sarcolemma of patients with Duchenne muscular dystrophy and mdx mice. Here we demonstrate that dystrophin-related protein (DRP, utrophin), an autosomal homologue of dystrophin, is associated with an identical or antigenically similar complex of sarcolemmal proteins and that DRP and the dystrophin/DRP-associated proteins colocalize to the neuromuscular junction in Duchenne muscular dystrophy and mdx muscle. The DRP and dystrophin/DRP-associated proteins are found throughout the sarcolemma in small-calibre skeletal muscles and cardiac muscle of adult mdx mice. Because these muscles show minimal pathological changes, our results could provide a basis for the upregulation of DRP as a potential therapeutic approach. |
