title: In vivo treatment with the NF-κB inhibitor ursodeoxycholic acid (UDCA) improves tension development in the isolated mdx costal diaphragm.
abstract: BACKGROUND
  Previous experiments have indicated that in vivo administration of ursodeoxycholic acid (UDCA) inhibits nuclear NF-κB activation and has beneficial effects on the structure and function of dystrophic (mdx) muscle. We examined the effect of UDCA on tension development in dystrophic muscle.
  
  
  METHODS
  Isometric tension development was examined in costal diaphragms that were freshly isolated from vehicle and UDCA treated mdx mice. Percent recovery scores were obtained by directly comparing these measurements to those obtained from age-matched nondystrophic mice.
  
  
  RESULTS
  Vehicle treated mdx mice exhibited significantly reduced optimal muscle lengths (lo ) and specific twitch and tetanic tensions compared with age-matched nondystrophic mice. UDCA treated preparations exhibited significantly improved tension development with a 33% recovery score.
  
  
  CONCLUSIONS
  Because UDCA is used in treating certain clinical disorders, these results provide a rationale for human clinical trials using this and related drugs for treatment of Duchenne and related muscular dystrophies.
authors.affiliation: Department of Physiology, Midwestern University Glendale, Glendale, Arizona, 85308.
authors.forename: C George
authors.initials: CG
authors.lastname: Carlson
authors.affiliation: Department of Physiology, Midwestern University Glendale, Glendale, Arizona, 85308.
authors.forename: Ross
authors.initials: R
authors.lastname: Potter
authors.affiliation: Department of Physiology, Midwestern University Glendale, Glendale, Arizona, 85308.
authors.forename: Vivien
authors.initials: V
authors.lastname: Yu
authors.affiliation: Department of Physiology, Midwestern University Glendale, Glendale, Arizona, 85308.
authors.forename: Kevin
authors.initials: K
authors.lastname: Luo
authors.affiliation: Department of Physiology, Midwestern University Glendale, Glendale, Arizona, 85308.
authors.forename: Jesse
authors.initials: J
authors.lastname: Lavin
authors.affiliation: Department of Physiology, Midwestern University Glendale, Glendale, Arizona, 85308.
authors.forename: Cory
authors.initials: C
authors.lastname: Nielsen
mesh_terms: D000818:Animals; D001703:Biophysics; D003964:Diaphragm / Q000187:drug effects* / Q000503:physiopathology; D004195:Disease Models, Animal; D004558:Electric Stimulation; D004791:Enzyme Inhibitors / Q000627:therapeutic use*; D051379:Mice; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D009119:Muscle Contraction / Q000187:drug effects; D009136:Muscular Dystrophies / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology*; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use*
chemical_list: D004791:Enzyme Inhibitors; D014580:Ursodeoxycholic Acid
keywords: Duchenne muscular dystrophy; NF-kappa B inhibitors; mdx mouse; muscle weakness; ursodeoxycholic acid