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Titolo:
Biochemical evidence for association of dystrobrevin with the sarcoglycan-sarcospan complex as a basis for understanding sarcoglycanopathy
Autore:
Yoshida, M; Hama, H; Ishikawa-Sakurai, M; Imamura, M; Mizuno, Y; Araishi, K; Wakabayashi-Takai, E; Noguchi, S; Sasaoka, T; Ozawa, E;
Indirizzi:
NCNP, Natl Inst Neurosci, Dept Cell Biol, Tokyo 1878502, Japan NCNP Tokyo Japan 1878502 Neurosci, Dept Cell Biol, Tokyo 1878502, Japan Japan Sci & Technol Corp, PREST, Inheritance & Variat Grp, Kawaguchi, Saitama 3320012, Japan Japan Sci & Technol Corp Kawaguchi Saitama Japan 3320012 a 3320012, Japan
Titolo Testata:
HUMAN MOLECULAR GENETICS
fascicolo: 7, volume: 9, anno: 2000,
pagine: 1033 - 1040
SICI:
0964-6906(20000412)9:7<1033:BEFAOD>2.0.ZU;2-Y
Fonte:
ISI
Lingua:
ENG
Soggetto:
NITRIC-OXIDE SYNTHASE; DYSTROPHIN-ASSOCIATED PROTEINS; DUCHENNE MUSCULAR-DYSTROPHY; GLYCOPROTEIN-COMPLEX; SKELETAL-MUSCLE; CARDIOMYOPATHIC HAMSTER; MOLECULAR-ORGANIZATION; BETA-DYSTROBREVIN; DEFICIENT MICE; BINDING SITE;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
41
Recensione:
Indirizzi per estratti:
Indirizzo: Yoshida, M NCNP, Natl Inst Neurosci, Dept Cell Biol, 4-1-1 Ogawahigashi Chou, Tokyo 1878502, Japan NCNP 4-1-1 Ogawahigashi Chou Tokyo Japan 1878502 878502, Japan
Citazione:
M. Yoshida et al., "Biochemical evidence for association of dystrobrevin with the sarcoglycan-sarcospan complex as a basis for understanding sarcoglycanopathy", HUM MOL GEN, 9(7), 2000, pp. 1033-1040

Abstract

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 ofthis complex revealed that the N-terminal half of dystrobrevin participates in this association. It is thus considered that the sarcoglycan-sarcospancomplex is linked to the signaling protein neuronal nitric oxide synthase via alpha-syntrophin associated with dystrobrevin.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 30/11/20 alle ore 10:10:59