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Titolo:
Role for alpha-dystrobrevin in the pathogenesis of dystrophin-dependent muscular dystrophies
Autore:
Grady, RM; Grange, RW; Lau, KS; Maimone, MM; Nichol, MC; Stull, JT; Sanes, JR;
Indirizzi:
Washington Univ, Sch Med, Dept Pediat, St Louis, MO 63110 USA Washington Univ St Louis MO USA 63110 Dept Pediat, St Louis, MO 63110 USA Univ Texas, SW Med Ctr, Dept Physiol, Dallas, TX 75235 USA Univ Texas Dallas TX USA 75235 ed Ctr, Dept Physiol, Dallas, TX 75235 USA Washington Univ, Sch Med, Dept Mol Biol & Pharmacol, St Louis, MO 63110 USA Washington Univ St Louis MO USA 63110 & Pharmacol, St Louis, MO 63110 USA SUNY Syracuse, Dept Anat & Cell Biol, Syracuse, NY 13210 USA SUNY Syracuse Syracuse NY USA 13210 t & Cell Biol, Syracuse, NY 13210 USA Washington Univ, Sch Med, Dept Anat & Neurobiol, St Louis, MO 63110 USA Washington Univ St Louis MO USA 63110 & Neurobiol, St Louis, MO 63110 USA
Titolo Testata:
NATURE CELL BIOLOGY
fascicolo: 4, volume: 1, anno: 1999,
pagine: 215 - 220
SICI:
1465-7392(199908)1:4<215:RFAITP>2.0.ZU;2-S
Fonte:
ISI
Lingua:
ENG
Soggetto:
NITRIC-OXIDE SYNTHASE; TORPEDO ELECTRIC ORGAN; SKELETAL-MUSCLE; BETA-DYSTROBREVIN; SODIUM-CHANNELS; DEFICIENT MICE; MDX MOUSE; PROTEIN; COMPLEX; FAMILY;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
49
Recensione:
Indirizzi per estratti:
Indirizzo: Grady, RM Washington Univ, Sch Med, Dept Pediat, 660 S Euclid Ave, St Louis, MO 63110 USA Washington Univ 660 S Euclid Ave St Louis MO USA 63110 63110 USA
Citazione:
R.M. Grady et al., "Role for alpha-dystrobrevin in the pathogenesis of dystrophin-dependent muscular dystrophies", NAT CELL BI, 1(4), 1999, pp. 215-220

Abstract

A dystrophin-containing glycoprotein complex (DGC) links the basal lamina surrounding each muscle fibre to the fibre's cytoskeleton, providing both structural support and a scaffold for signalling molecules. Mutations in genes encoding several DGC components disrupt the complex and lead to musculardystrophy. Here we show that mice deficient in alpha-dystrobrevin, a cytoplasmic protein of the DGC, exhibit skeletal and cardiac myopathies. Analysis of double and triple mutants indicates that alpha-dystrobrevin acts largely through the DGC, Structural components of the DGC are retained in the absence of alpha-dystrobrevin, but a DGC-associated signalling protein, nitric oxide synthase, is displaced from the membrane and nitric-oxide-mediated signalling is impaired. These results indicate that both signalling and structural functions of the DGC are required for muscle stability, and implicate alpha-dystrobrevin in the former.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 03/12/20 alle ore 14:45:16