Dystrophin-associated proteins in muscular dystrophy.

Abstract

Dystrophin-associated proteins (DAPs) are classified into a few groups, namely, those comprising of dystroglycan complex, sarcoglycan complex, syntrophin complex and others. Subsarcolemmal actin filaments are connected to laminin in the basement membrane through dystrophin and the dystroglycan complex. This system may function to protect muscle fibers from mechanical damage. Furthermore, the sarcoglycan complex is associated with the system. Defects in the components of the protection system or the sarcoglycan complex or both are characteristically found in various muscular dystrophies. The roles of the syntrophin complex are meagerly understood. In this review, the possible roles of laminin, DAPs and dystrophin in each dystrophy are explained.

• Authors: E Ozawa, M Yoshida, A Suzuki, Y Mizuno, Y Hagiwara, S Noguchi
• Journal: Human molecular genetics (Hum Mol Genet) Vol. 4 Spec No Pg. 1711-6 ( 1995) ISSN: 0964-6906 [Print] England
• PMID: 8541869 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)

Chemical References

• Dystrophin
• Laminin
• Membrane Glycoproteins

Topics

• Dystrophin (metabolism)
• Humans
• Laminin (metabolism)
• Membrane Glycoproteins (metabolism)
• Muscular Dystrophies (metabolism)