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Cell surface and gene expression regulation molecules in dystrophinopathy: mdx vs. Duchenne
Fadic, Ricardo.
Afiliação
  • Fadic, Ricardo; Pontificia Universidad Católica de Chile. Facultad de Medicina. Departamento de Neurología. Unidad Neuromuscular. Santiago. CL
Biol. Res ; 38(4): 375-380, 2005.
Article em En | LILACS | ID: lil-425821
Biblioteca responsável: CL1.1
RESUMO
Duchenne muscular dystrophy (DMD) is secondary to loss-of-function mutations in the dystrophin gene. The causes underlying the progression of DMD, differential muscle involvement, and the discrepancies in phenotypes among species with the same genetic defect are not understood. The mdx mouse, an animal model with dystrophin mutation, has a milder phenotype. This article reviews the available information on expression of signaling-related molecules in DMD and mdx. Extracellular matrix proteoglycans, growth factors, integrins, caveolin-3, and neuronal nitric oxide synthase expression do not show significant differences. Calcineurin is inconsistently activated in mdx, which is associated with lack of cardiomyopathy, compared to the permanent calcineurin activation in mdx/utrophin null mice that have a DMD-like cardiomyopathy. Levels of focal adhesion kinase (FAK) and extracellular regulated kinases (ERKs) differ among mdx and DMD. Further work is needed to identify the point of discrepancy in these signaling molecules' pathways in dystrophynopathies.
Assuntos
Texto completo: 1 Coleções: 01-internacional Base de dados: LILACS Assunto principal: Distrofina / Distrofia Muscular de Duchenne Tipo de estudo: Prognostic_studies Limite: Animals Idioma: En Revista: Biol. Res Assunto da revista: BIOLOGIA Ano de publicação: 2005 Tipo de documento: Article / Project document País de afiliação: Chile País de publicação: Chile
Texto completo: 1 Coleções: 01-internacional Base de dados: LILACS Assunto principal: Distrofina / Distrofia Muscular de Duchenne Tipo de estudo: Prognostic_studies Limite: Animals Idioma: En Revista: Biol. Res Assunto da revista: BIOLOGIA Ano de publicação: 2005 Tipo de documento: Article / Project document País de afiliação: Chile País de publicação: Chile