title: The paradox of muscle hypertrophy in muscular dystrophy.
abstract: Mutations in the dystrophin gene cause Duchenne and Becker muscular dystrophy in humans and syndromes in mice, dogs, and cats. Affected humans and dogs have progressive disease that leads primarily to muscle atrophy. Mdx mice progress through an initial phase of muscle hypertrophy followed by atrophy. Cats have persistent muscle hypertrophy. Hypertrophy in humans has been attributed to deposition of fat and connective tissue (pseudohypertrophy). Increased muscle mass (true hypertrophy) has been documented in animal models. Muscle hypertrophy can exaggerate postural instability and joint contractures. Deleterious consequences of muscle hypertrophy should be considered when developing treatments for muscular dystrophy.
authors.affiliation: Department of Pathology and Laboratory Medicine, School of Medicine, University of North Carolina-Chapel Hill, Chapel Hill, NC 27599, USA. joe_kornegay@med.unc.edu
authors.forename: Joe N
authors.initials: JN
authors.lastname: Kornegay
authors.forename: Martin K
authors.initials: MK
authors.lastname: Childers
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Bogan
authors.forename: Janet R
authors.initials: JR
authors.lastname: Bogan
authors.forename: Peter
authors.initials: P
authors.lastname: Nghiem
authors.forename: Jiahui
authors.initials: J
authors.lastname: Wang
authors.forename: Zheng
authors.initials: Z
authors.lastname: Fan
authors.forename: James F
authors.initials: JF
authors.lastname: Howard
authors.forename: Scott J
authors.initials: SJ
authors.lastname: Schatzberg
authors.forename: Jennifer L
authors.initials: JL
authors.lastname: Dow
authors.forename: Robert W
authors.initials: RW
authors.lastname: Grange
authors.forename: Martin A
authors.initials: MA
authors.lastname: Styner
authors.forename: Eric P
authors.initials: EP
authors.lastname: Hoffman
authors.forename: Kathryn R
authors.initials: KR
authors.lastname: Wagner
mesh_terms: D000818:Animals; D003286:Contracture / Q000209:etiology / Q000503:physiopathology; D004195:Disease Models, Animal; D016189:Dystrophin / Q000235:genetics / Q000378:metabolism; D006801:Humans; D006984:Hypertrophy / Q000209:etiology* / Q000378:metabolism / Q000503:physiopathology; D007738:Kyphosis / Q000209:etiology; D053580:Muscle Strength; D018482:Muscle, Skeletal / Q000473:pathology* / Q000503:physiopathology; D009136:Muscular Dystrophies / Q000150:complications / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D055435:Myostatin / Q000037:antagonists & inhibitors
chemical_list: D016189:Dystrophin; D055435:Myostatin