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Deficiency of dystrophin-associated proteins in Duchenne muscular dystrophy patients lacking COOH-terminal domains of dystrophin.

Dystrophin, the protein product of the Duchenne muscular dystrophy (DMD) gene, is a cytoskeletal protein tightly associated with a large oligomeric complex of sarcolemmal glycoproteins including dystroglycan, which provides a linkage to the extracellular matrix component, laminin. In DMD, the absenc...

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Udgivet i:J Clin Invest
Principais autores: Matsumura, K, Tomé, F M, Ionasescu, V, Ervasti, J M, Anderson, R D, Romero, N B, Simon, D, Récan, D, Kaplan, J C, Fardeau, M
Format: Artigo
Sprog:Inglês
Udgivet: American Society for Clinical Investigation 1993
Fag:
Research Article
Online adgang:https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC294925/
https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/8349821/
https://ncbi.nlm.nih.govhttps://doi.org/10.1172/JCI116661
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