Catalogo Articoli (Spogli Riviste)

OPAC HELP

Titolo:
Confocal analysis of the dystrophin protein complex in muscular dystrophy
Autore:
Draviam, R; Billington, L; Senchak, A; Hoffman, EP; Watkins, SC;
Indirizzi:
Univ Pittsburgh, Sch Med, Dept Cell Biol & Physiol, Pittsburgh, PA 15261 USA Univ Pittsburgh Pittsburgh PA USA 15261 Physiol, Pittsburgh, PA 15261 USA Childrens Natl Med Ctr, Duchenne Muscular Dystrophy Res Ctr, Pittsburgh, PA USA Childrens Natl Med Ctr Pittsburgh PA USA phy Res Ctr, Pittsburgh, PA USA Childrens Natl Med Ctr, Med Genet Res Ctr, Washington, DC 20010 USA Childrens Natl Med Ctr Washington DC USA 20010 , Washington, DC 20010 USA
Titolo Testata:
MUSCLE & NERVE
fascicolo: 2, volume: 24, anno: 2001,
pagine: 262 - 272
SICI:
0148-639X(200102)24:2<262:CAOTDP>2.0.ZU;2-0
Fonte:
ISI
Lingua:
ENG
Soggetto:
GLYCOPROTEIN COMPLEX; SARCOGLYCAN COMPLEX; ELECTRON-MICROSCOPY; BETA-SARCOGLYCAN; MUSCLE; MUTATIONS; DUCHENNE; MEMBRANE; DEFICIENCY; SKELETAL;
Keywords:
confocal microscopy; dystroglycan; dystrophin; merosin; muscular dystrophy;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
50
Recensione:
Indirizzi per estratti:
Indirizzo: Watkins, SC Univ Pittsburgh, Sch Med, Dept Cell Biol & Physiol, S225 Biomed Sci Tower,Pittsburgh, PA 15261 USA Univ Pittsburgh S225 Biomed Sci Tower Pittsburgh PA USA 15261
Citazione:
R. Draviam et al., "Confocal analysis of the dystrophin protein complex in muscular dystrophy", MUSCLE NERV, 24(2), 2001, pp. 262-272

Abstract

The dystrophin protein complex (DPC), composed of at least 10 proteins that associate with dystrophin, is critical for the maintenance of normal muscle fiber structure and physiology. In this study, we used immunohistochemistry and confocal microscopy to examine the relative abundance and distribution of several of these proteins in muscle biopsies taken from patients with various muscular dystrophies. The optical sectioning capability of confocal microscopy allowed us to comprehensively analyze the semiquantitative expression of components of the DPC. Alpha-sarcoglycan-deficient patients displayed a marked reduction in membrane immunostaining of the sarcoglycan complex. Gamma-sarcoglycan-deficient patients showed variable decreased immunostaining of the sarcoglycan complex proteins. When beta -sarcoglycan was expressed appropriately at the sarcolemma of gamma -sarcoglycan-deficient patients, intracellular labeling of beta -sarco-glycan was also present. Beta-sarcoglycan-deficient patients showed poor localization of extracellular matrix proteins in addition to a complete absence of the sarcoglycans. Merosin-deficient patients showed relatively normal immunostaining levels of all other members of the DPC. Finally, dystrophin-deficient patients showed little or no change in the expression of extracellular matrix proteins; however, some sarcoglycans were significantly decreased. These data allowed us tosuggest unique fundamental interactions between the members of the DPC. (C) 2001 John Wiley & Sons, Inc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 29/11/20 alle ore 10:28:22