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Muscular dystrophy meets protein biochemistry, the mother of invention.
J Clin Invest ; 127(3): 798-800, 2017 Mar 01.
Article em En | MEDLINE | ID: mdl-28218619
Muscular dystrophies result from a defect in the linkage between the muscle fiber cytoskeleton and the basement membrane (BM). Congenital muscular dystrophy type MDC1A is caused by mutations in laminin α2 that either reduce its expression or impair its ability to polymerize within the muscle fiber BM. Defects in this BM lead to muscle fiber damage from the force of contraction. In this issue of the JCI, McKee and colleagues use a laminin polymerization-competent, designer chimeric BM protein in vivo to restore function of a polymerization-defective laminin, leading to normalized muscle structure and strength in a mouse model of MDC1A. Delivery of such a protein to patients could ameliorate many aspects of their disease.
Assuntos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Laminina / Fibras Musculares Esqueléticas / Força Muscular / Contração Muscular / Distrofias Musculares / Mutação Limite: Animals / Humans Idioma: En Revista: J Clin Invest Ano de publicação: 2017 Tipo de documento: Article

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Laminina / Fibras Musculares Esqueléticas / Força Muscular / Contração Muscular / Distrofias Musculares / Mutação Limite: Animals / Humans Idioma: En Revista: J Clin Invest Ano de publicação: 2017 Tipo de documento: Article