Dystrophin-associated proteins in muscular dystrophy.
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.
National Institute of Neuroscience, NCNP, Tokyo, Japan.
E
E
Ozawa
M
M
Yoshida
A
A
Suzuki
Y
Y
Mizuno
Y
Y
Hagiwara
S
S
Noguchi
D016189:Dystrophin / Q000378:metabolism*; D006801:Humans; D007797:Laminin / Q000378:metabolism; D008562:Membrane Glycoproteins / Q000378:metabolism*; D009136:Muscular Dystrophies / Q000378:metabolism*
D016189:Dystrophin; D007797:Laminin; D008562:Membrane Glycoproteins