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Titolo:
EXPRESSION OF GENES (CAPN3, SGCA, SGCB, AND TTN) INVOLVED IN PROGRESSIVE MUSCULAR-DYSTROPHIES DURING EARLY HUMAN-DEVELOPMENT
Autore:
FOUGEROUSSE F; DURAND M; SUEL L; POURQUIE O; DELEZOIDE AL; ROMERO NB; ABITBOL M; BECKMANN JS;
Indirizzi:
GENETHON,URA 1922,1 RUE INT,BP 60 F-91002 EVRY FRANCE GENETHON,URA 1922 F-91002 EVRY FRANCE IBDM F-13288 MARSEILLE FRANCE HOP NECKER ENFANTS MALAD,SERV HISTOL EMBRYOL CYTOGENET F-75015 PARIS FRANCE HOP ROBERT DEBRE,SERV PATHOL NEUROMUSCULAIRE F-75019 PARIS FRANCE FAC NECKER,CERTO F-75015 PARIS FRANCE
Titolo Testata:
Genomics
fascicolo: 2, volume: 48, anno: 1998,
pagine: 145 - 156
SICI:
0888-7543(1998)48:2<145:EOG(SS>2.0.ZU;2-Q
Fonte:
ISI
Lingua:
ENG
Soggetto:
CALCIUM-DEPENDENT PROTEASE; BETA-SARCOGLYCAN; MESSENGER-RNA; MUTATIONS; MUSCLE; DISTINCT; PROTEINS; COMPLEX; ADHALIN; CALPAIN;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
28
Recensione:
Indirizzi per estratti:
Citazione:
F. Fougerousse et al., "EXPRESSION OF GENES (CAPN3, SGCA, SGCB, AND TTN) INVOLVED IN PROGRESSIVE MUSCULAR-DYSTROPHIES DURING EARLY HUMAN-DEVELOPMENT", Genomics, 48(2), 1998, pp. 145-156

Abstract

The developmental expression pattern of four human genes, three of which are involved in progressive muscular dystrophies, was investigated. The rationale for these experiments is that these patterns might provide useful information on the pathophysiology underlying these myopathies. Despite the presence of overlapping clinical signs, the spatiotemporal expression profiles of the corresponding genes differed widely. Transcripts of alpha-sarcoglycan (SGCA) were visible as soon as myotomes were formed, and constitute, together with titin transcripts, precocious muscular system landmarks. beta-sarcoglycan (SGCB) was initially transcribed in a ubiquitous manner, and, toward the second part of the embryonic period, became specific to striated muscle, heart, and the central nervous system. Whereas titin (TTN) transcription and translation seem to be coupled, for the sarcoglycans, translation seemed restricted to skeletal muscle. Calpain3 (CAPN3) RNA was found in only skeletal muscles during the fetal period. It was, however, present earlier in the whole heart, where it selectively disappeared, Finally, evidence for differentially spliced calpain3 variants in smooth muscles wasalso seen. The expression profiles of these genes is suggestive of their having a role during myogenesis, knowledge of which could be pertinent to the understanding of the pathophysiology of the associated diseases. (C) 1998 Academic Press.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 30/05/20 alle ore 13:07:32