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Titolo:
Muscle degeneration without mechanical injury in sarcoglycan deficiency
Autore:
Hack, AA; Cordier, L; Shoturma, DI; Lam, MY; Sweeney, HL; McNally, EM;
Indirizzi:
Univ Chicago, Dept Med, Cardiol Sect, Chicago, IL 60637 USA Univ Chicago Chicago IL USA 60637 ed, Cardiol Sect, Chicago, IL 60637 USA Univ Chicago, Dept Mol Genet & Cell Biol, Chicago, IL 60637 USA Univ Chicago Chicago IL USA 60637 enet & Cell Biol, Chicago, IL 60637 USA Univ Penn, Sch Med, Dept Physiol, Philadelphia, PA 19104 USA Univ Penn Philadelphia PA USA 19104 t Physiol, Philadelphia, PA 19104 USA
Titolo Testata:
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
fascicolo: 19, volume: 96, anno: 1999,
pagine: 10723 - 10728
SICI:
0027-8424(19990914)96:19<10723:MDWMII>2.0.ZU;2-0
Fonte:
ISI
Lingua:
ENG
Soggetto:
DYSTROPHIN-ASSOCIATED PROTEIN; DUCHENNE MUSCULAR-DYSTROPHY; TORPEDO ELECTRIC ORGAN; GLYCOPROTEIN COMPLEX; MDX MOUSE; GAMMA-SARCOGLYCAN; INTRACELLULAR CALCIUM; EXTRACELLULAR-MATRIX; EPSILON-SARCOGLYCAN; BETA-DYSTROBREVIN;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
61
Recensione:
Indirizzi per estratti:
Indirizzo: McNally, EM Univ Chicago, Dept Med, Cardiol Sect, 5841 S Maryland Ave,MC 6088, Chicago, IL 60637 USA Univ Chicago 5841 S Maryland Ave,MC 6088 ChicagoIL USA 60637
Citazione:
A.A. Hack et al., "Muscle degeneration without mechanical injury in sarcoglycan deficiency", P NAS US, 96(19), 1999, pp. 10723-10728

Abstract

In humans, mutations in the genes encoding components of the dystrophin-glycoprotein complex cause muscular dystrophy. Specifically, primary mutations in the genes encoding alpha-, beta-, gamma-, and delta-sarcoglycan have been identified in humans with limb-girdle muscular dystrophy. Mice lacking gamma-sarcoglycan develop progressive muscular dystrophy similar to human muscular dystrophy. Without gamma-sarcoglycan, beta- and delta-sarcoglycan are unstable at the muscle membrane and alpha-sarcoglycan is severely reduced. The expression and localization of dystrophin, dystroglycan, and laminin-alpha 2, a mechanical link between the actin cytoskeleton and the extracellular matrix, appears unaffected by the loss of sarcoglycan. We assessed the functional integrity of this mechanical link and found that isolated muscles lacking gamma-sarcoglycan showed normal resistance to mechanical straininduced by eccentric muscle contraction. Sarcoglycan-deficient muscles also showed normal peak isometric and tetanic force generation. Furthermore, there was no evidence for contraction-induced injury in mice lacking gamma-sarcoglycan that were subjected to an extended, rigorous exercise regimen. These data demonstrate that mechanical weakness and contraction-induced muscle injury are not required for muscle degeneration and the dystrophic process. Thus, a nonmechanical mechanism, perhaps involving some unknown signaling function, likely is responsible for muscular dystrophy where sarcoglycanis deficient.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 03/12/20 alle ore 15:59:31