Expression of truncated utrophin leads to major functional improvements in dystrophin-deficient muscles of mice.
Nat Med
; 3(11): 1216-21, 1997 Nov.
Article
em En
| MEDLINE
| ID: mdl-9359695
ABSTRACT
Dystrophin-deficient mice (mdx) expressing a truncated (trc) utrophin transgene show amelioration of the dystrophic phenotype. Here we report a multifunctional study demonstrating that trcutrophin expression leads to major improvements of the mechanical performance of muscle (that is, force development, mechanical resistance to forced lengthenings and maximal spontaneous activity) and of the maintenance of the intracellular calcium homeostasis. These are two essential functions of muscle fibers, known to be impaired in mdx mouse muscles and Duchenne muscular dystrophy (DMD) patients. Our results bring strong support to the hypothesis that muscle wasting in dystrophin-deficient DMD patients could be prevented by upregulation of utrophin.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Distrofina
/
Proteínas do Citoesqueleto
/
Proteínas de Membrana
/
Contração Muscular
/
Músculos
Limite:
Animals
Idioma:
En
Revista:
Nat Med
Assunto da revista:
BIOLOGIA MOLECULAR
/
MEDICINA
Ano de publicação:
1997
Tipo de documento:
Article
País de afiliação:
Bélgica