Biochemical evidence for association of dystrobrevin with the sarcoglycan-sarcospan complex as a basis for understanding sarcoglycanopathy

被引:117
作者
Yoshida, M
Hama, H
Ishikawa-Sakurai, M
Imamura, M
Mizuno, Y
Araishi, K
Wakabayashi-Takai, E
Noguchi, S
Sasaoka, T
Ozawa, E
机构
[1] NCNP, Natl Inst Neurosci, Dept Cell Biol, Tokyo 1878502, Japan
[2] Japan Sci & Technol Corp, PREST, Inheritance & Variat Grp, Kawaguchi, Saitama 3320012, Japan
关键词
D O I
10.1093/hmg/9.7.1033
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
The sarcoglycan complex is composed of four membrane-spanning dystrophin-associated proteins (DAPs) and is essential for skeletal muscle survival, since the absence or markedly reduced expression of this complex due to mutation of any one of the sarcoglycan genes causes a group of muscular dystrophies, collectively termed sarcoglycanopathy, Although one of the putative functions of the sarcoglycan complex is its participation in signaling processes, detailed studies have been scarce. Very recently, it was shown that gene knockout mice for a DAP, alpha-dystrobrevin, exhibit a dystrophic phenotype, possibly due to defects in muscle cell signaling, To clarify the putative function of the sarcoglycan complex, it is essential to determine whether or not there is a link between it and the intracellular signaling molecules, To elucidate this, we developed new methods for preparing various DAP complexes containing the sarcoglycan complex from the purified dystrophin-DAP complex, It was suggested from one of the complexes prepared that the sarcoglycan-sarcospan complex (the sarcoglycan complex associated with sarcospan) is associated with syntrophin and/or dystrobrevin. Further analysis of this complex revealed that the N-terminal half of dystrobrevin participates in this association. It is thus considered that the sarcoglycan-sarcospan complex is linked to the signaling protein neuronal nitric oxide synthase via alpha-syntrophin associated with dystrobrevin.
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页码:1033 / 1040
页数:8
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