Sodium 4-phenylbutyrate reduces myofiber damage in a mouse model of Duchenne muscular dystrophy.
Appl Physiol Nutr Metab
; 41(10): 1108-1111, 2016 Oct.
Article
em En
| MEDLINE
| ID: mdl-27628198
ABSTRACT
We performed a placebo-controlled pre-clinical study to determine if sodium 4-phenylbutyrate (4PB) can reduce contraction-induced myofiber damage in the mdx mouse model of Duchenne muscular dystrophy (DMD). At 72 h post-eccentric contractions, 4PB significantly increased contractile torque and reduced myofiber damage and macrophage infiltration. We conclude that 4PB, which is approved by Health Canada (Pheburane) and the United States Food and Drug Administration (Buphenyl) for urea cycle disorders, might modify disease severity in patients with DMD.
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Base de dados:
MEDLINE
Assunto principal:
Fenilbutiratos
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Músculo Esquelético
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Distrofia Muscular de Duchenne
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Manipulações Musculoesqueléticas
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Inibidores de Histona Desacetilases
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Ativação de Macrófagos
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Miofibrilas
Idioma:
En
Ano de publicação:
2016
Tipo de documento:
Article