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Titolo:
QUANTITATIVE AND QUALITATIVE ALTERATIONS OF DYSTROPHIN ARE EXPRESSED IN MUSCLE-CELL CULTURES OF XP21 MUSCULAR-DYSTROPHY PATIENTS (DUCHENNE AND BECKER TYPE)
Autore:
MONGINI T; DORIGUZZI C; PALMUCCI L; CHIADOPIAT L;
Indirizzi:
UNIV TURIN,NEUROL CLIN 2,VIA CHERASCO 15 I-10126 TURIN ITALY UNIV TURIN,PAOLO PEIROLO CTR NEUROMUSC DIS,DIV NEUROL 2 TURIN ITALY
Titolo Testata:
European journal of clinical investigation
fascicolo: 4, volume: 26, anno: 1996,
pagine: 322 - 324
SICI:
0014-2972(1996)26:4<322:QAQAOD>2.0.ZU;2-G
Fonte:
ISI
Lingua:
ENG
Soggetto:
MYOTUBES;
Keywords:
BECKER; DUCHENNE; DYSTROPHIN; MUSCLE CELL CULTURES; WESTERN BLOTTING; XP21 MUSCULAR DYSTROPHY;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
17
Recensione:
Indirizzi per estratti:
Citazione:
T. Mongini et al., "QUANTITATIVE AND QUALITATIVE ALTERATIONS OF DYSTROPHIN ARE EXPRESSED IN MUSCLE-CELL CULTURES OF XP21 MUSCULAR-DYSTROPHY PATIENTS (DUCHENNE AND BECKER TYPE)", European journal of clinical investigation, 26(4), 1996, pp. 322-324

Abstract

The authors studied skeletal muscle cell cultures from four control subjects, two patients with Duchenne muscular dystrophy, two Duchenne carriers and three patients with Becker muscular dystrophy with different phenotypes. Western blotting was performed on well-differentiated myotubes and compared with the results obtained in muscle tissue. In all cultures the band of dystrophin closely corresponded to the one observed in muscle tissue: both quantitative and qualitative defects were observed. This confirms the early expression of the Xp21 gene defect in uninnervated muscle cultures and supports the usefulness of muscle cultures both in diagnostic procedure and as a model to study the disease.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 25/09/20 alle ore 06:22:21