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Titolo:
DISRUPTION OF MUSCLE ARCHITECTURE AND MYOCARDIAL DEGENERATION IN MICELACKING DESMIN
Autore:
MILNER DJ; WEITZER G; TRAN D; BRADLEY A; CAPETANAKI Y;
Indirizzi:
BAYLOR COLL MED,DEPT CELL BIOL,1 BAYLOR PLAZA HOUSTON TX 77030 BAYLOR COLL MED,DEPT CELL BIOL HOUSTON TX 77030 BAYLOR COLL MED,HOWARD HUGHES MED INST HOUSTON TX 77030 BAYLOR COLL MED,INST MOL GENET HOUSTON TX 77030
Titolo Testata:
The Journal of cell biology
fascicolo: 5, volume: 134, anno: 1996,
pagine: 1255 - 1270
SICI:
0021-9525(1996)134:5<1255:DOMAAM>2.0.ZU;2-K
Fonte:
ISI
Lingua:
ENG
Soggetto:
EPIDERMOLYSIS-BULLOSA SIMPLEX; INTERMEDIATE FILAMENT ORGANIZATION; AMYOTROPHIC-LATERAL-SCLEROSIS; CHICKEN SKELETAL-MUSCLE; SMOOTH-MUSCLE; MONONUCLEATED MYOBLASTS; HUMAN KERATIN-14; MOUSE MODEL; Z-DISC; EXPRESSION;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
99
Recensione:
Indirizzi per estratti:
Citazione:
D.J. Milner et al., "DISRUPTION OF MUSCLE ARCHITECTURE AND MYOCARDIAL DEGENERATION IN MICELACKING DESMIN", The Journal of cell biology, 134(5), 1996, pp. 1255-1270

Abstract

Desmin, the muscle specific intermediate filament (IF) protein encoded by a single gene, is expressed in all muscle tissues. In mature striated muscle, desmin IFs surround the Z-discs, interlink them together and integrate the contractile apparatus with the sarcolemma and the nucleus. To investigate the function of desmin in all three muscle typesin vivo, we generated desmin null mice through homologous recombination. Surprisingly, desmin null mice are viable and fertile. However, these mice demonstrated a multisystem disorder involving cardiac, skeletal, and smooth muscle. Histological and electron microscopic analysis in both heart and skeletal muscle tissues revealed severe disruption of muscle architecture and degeneration. Structural abnormalities included loss of lateral alignment of myofibrils and abnormal mitochondrialorganization. The consequences of these abnormalities were most severe in the heart, which exhibited progressive degeneration and necrosis of the myocardium accompanied by extensive calcification. Abnormalities of smooth muscle included hypoplasia and degeneration. The present data demonstrate the essential role of desmin in the maintenance of myofibril, myofiber, and whole muscle tissue structural and functional integrity, and show that the absence of desmin leads to muscle degeneration.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 22/09/20 alle ore 19:28:38