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Titolo:
ABSENCE OF GAMMA-SARCOGLYCAN (35-DAG) IN AUTOSOMAL RECESSIVE MUSCULAR-DYSTROPHY LINKED TO CHROMOSOME 13Q12
Autore:
JUNG D; LETURCQ F; SUNADA Y; DUCLOS F; TOME FMS; MOOMAW C; MERLINI L; AZIBI K; CHAOUCH M; SLAUGHTER C; FARDEAU M; KAPLAN JC; CAMPBELL KP;
Indirizzi:
UNIV IOWA,COLL MED,HOWARD HUGHES MED INST IOWA CITY IA 52242 UNIV IOWA,COLL MED,HOWARD HUGHES MED INST IOWA CITY IA 52242 UNIV IOWA,COLL MED,DEPT PHYSIOL & BIOPHYS IOWA CITY IA 52242 UNIV PARIS 05,CHU COCHIN,INSERM 129 F-75014 PARIS FRANCE UNIV PARIS 05,BIOCHIM GENET LAB F-75014 PARIS FRANCE INSERM 153 F-75005 PARIS FRANCE UNIV TEXAS,SW MED CTR,HOWARD HUGHES MED INST,BIOPOLYMER FACIL DALLAS TX 75235 IST ORTOPED RIZZOLI BOLOGNA ITALY CHU,HOP BOLOGHINE ALGIERS ALGERIA CHU,HOP BEN AKNOUN ALGIERS ALGERIA
Titolo Testata:
FEBS letters
fascicolo: 1-2, volume: 381, anno: 1996,
pagine: 15 - 20
SICI:
0014-5793(1996)381:1-2<15:AOG(IA>2.0.ZU;2-L
Fonte:
ISI
Lingua:
ENG
Soggetto:
GLYCOPROTEIN COMPLEX; SKELETAL-MUSCLE; DEFICIENCY; SARCOLEMMA; SEQUENCE; PROTEINS;
Keywords:
DYSTROPHIN-ASSOCIATED GLYCOPROTEIN, 35 KDA; GAMMA-SARCOGLYCAN; SEVERE CHILDHOOD AUTOSOMAL MUSCULAR DYSTROPHY; LIMB-GIRDLE MUSCULAR DYSTROPHY; SARCOGLYCAN COMPLEX;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
37
Recensione:
Indirizzi per estratti:
Citazione:
D. Jung et al., "ABSENCE OF GAMMA-SARCOGLYCAN (35-DAG) IN AUTOSOMAL RECESSIVE MUSCULAR-DYSTROPHY LINKED TO CHROMOSOME 13Q12", FEBS letters, 381(1-2), 1996, pp. 15-20

Abstract

We have partially sequenced rabbit skeletal muscle gamma-sarcoglycan an integral component of the dystrophin-glycoprotein complex, Specificantibodies were produced against a gamma-sarcoglycan peptide and usedto examine the expression of gamma-sarcoglycan in skeletal muscle of patients with severe childhood autosomal muscular dystrophy linked to chromosome 13q12 (SCARMD), We show by immunofluorescence and Western blotting that in skeletal muscle from these patients gamma-sarcoglycan is completely absent and alpha- and beta-sarcoglycan are greatly reduced in abundance, whereas other components of the DGC are preserved. Inaddition, we show that in normal muscle alpha-, beta-, and gamma-sarcoglycan constitute a tightly associated sarcolemma complex which can not be disrupted by SDS treatment.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 29/11/20 alle ore 10:17:35