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.
Keywords:
animal model(s); lésion musculaire; modèle animal; muscle damage; muscle physiology; muscle squelettique; physiologie musculaire; physiotherapy; physiothérapie; skeletal muscle.
MeSH terms
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Animals
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Hindlimb
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Histone Deacetylase Inhibitors / administration & dosage
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Histone Deacetylase Inhibitors / therapeutic use*
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Injections, Intraperitoneal
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Leg Injuries / prevention & control
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Macrophage Activation / drug effects*
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Male
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Mice, Inbred mdx
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Muscle Contraction / drug effects
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Muscle, Skeletal / drug effects*
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Muscle, Skeletal / immunology
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Muscle, Skeletal / injuries
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Muscle, Skeletal / pathology
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Muscular Dystrophy, Duchenne / drug therapy*
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Muscular Dystrophy, Duchenne / immunology
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Muscular Dystrophy, Duchenne / pathology
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Musculoskeletal Manipulations / adverse effects*
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Myofibrils / drug effects*
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Myofibrils / immunology
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Myofibrils / pathology
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Phenylbutyrates / administration & dosage
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Phenylbutyrates / therapeutic use*
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Torque
Substances
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Histone Deacetylase Inhibitors
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Phenylbutyrates
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4-phenylbutyric acid