title: Sodium 4-phenylbutyrate reduces myofiber damage in a mouse model of Duchenne muscular dystrophy.
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.
authors.affiliation: a Department of Health Care Sciences, Physical Therapy Program, Wayne State University, 259 Mack Ave., Rm. 4440, Detroit, MI 48201, USA.
authors.forename: Morium
authors.initials: M
authors.lastname: Begam
authors.affiliation: a Department of Health Care Sciences, Physical Therapy Program, Wayne State University, 259 Mack Ave., Rm. 4440, Detroit, MI 48201, USA.
authors.forename: Valerie M
authors.initials: VM
authors.lastname: Abro
authors.affiliation: b Program in Molecular Medicine, University of Maryland School of Medicine, 685 W. Baltimore St., Rm. 580, Baltimore, MD 21201, USA.
authors.forename: Amber L
authors.initials: AL
authors.lastname: Mueller
authors.affiliation: a Department of Health Care Sciences, Physical Therapy Program, Wayne State University, 259 Mack Ave., Rm. 4440, Detroit, MI 48201, USA.
authors.forename: Joseph A
authors.initials: JA
authors.lastname: Roche
mesh_terms: D000818:Animals; D006614:Hindlimb; D056572:Histone Deacetylase Inhibitors / Q000008:administration & dosage / Q000627:therapeutic use*; D007274:Injections, Intraperitoneal; D007869:Leg Injuries / Q000517:prevention & control; D008262:Macrophage Activation / Q000187:drug effects*; D008297:Male; D018101:Mice, Inbred mdx; D009119:Muscle Contraction / Q000187:drug effects; D018482:Muscle, Skeletal / Q000187:drug effects* / Q000276:immunology / Q000293:injuries / Q000473:pathology; D020388:Muscular Dystrophy, Duchenne / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D026201:Musculoskeletal Manipulations / Q000009:adverse effects*; D009210:Myofibrils / Q000187:drug effects* / Q000276:immunology / Q000473:pathology; D010654:Phenylbutyrates / Q000008:administration & dosage / Q000627:therapeutic use*; D019415:Torque
chemical_list: D056572:Histone Deacetylase Inhibitors; D010654:Phenylbutyrates; C075773:4-phenylbutyric acid
keywords: animal model(s); lésion musculaire; modèle animal; muscle damage; muscle physiology; muscle squelettique; physiologie musculaire; physiotherapy; physiothérapie; skeletal muscle