title: Muscle metabolic alterations assessed by 31-phosphorus magnetic resonance spectroscopy in mild Becker muscular dystrophy.
abstract: Although the molecular defect causing Becker muscular dystrophy (BMD) has been identified, the biochemical mechanisms that lead to muscle necrosis remain unclear. Exercise-related muscle metabolism in 9 mildly affected BMD patients was assessed by muscle 31-phosphorus magnetic resonance spectroscopy ((31)P MRS) during an incremental workload. Compared with normal controls, BMD patients showed deregulation of resting pH and intramuscular membrane breakdown. We also observed increased reliance upon anaerobic metabolism during sustained submaximal contraction and maintenance of oxidative function during recovery.
authors.affiliation: Department of Developmental Neuroscience and MR Laboratory, IRCCS Stella Maris, Viale del Tirreno 331, 56128 Calambrone, Pisa, Italy. michela.tosetti@inpe.unipi.it
authors.forename: Michela
authors.initials: M
authors.lastname: Tosetti
authors.forename: Stefania
authors.initials: S
authors.lastname: Linsalata
authors.forename: Roberta
authors.initials: R
authors.lastname: Battini
authors.forename: Leda
authors.initials: L
authors.lastname: Volpi
authors.forename: Claudio
authors.initials: C
authors.lastname: Cini
authors.forename: Otello
authors.initials: O
authors.lastname: Presciutti
authors.forename: Francesco
authors.initials: F
authors.lastname: Muntoni
authors.forename: Giovanni
authors.initials: G
authors.lastname: Cioni
authors.forename: Gabriele
authors.initials: G
authors.lastname: Siciliano
mesh_terms: D000328:Adult; D004734:Energy Metabolism / Q000502:physiology; D005080:Exercise Test / Q000379:methods; D006801:Humans; D009682:Magnetic Resonance Spectroscopy / Q000379:methods*; D008297:Male; D018482:Muscle, Skeletal / Q000000981:diagnostic imaging* / Q000378:metabolism*; D020388:Muscular Dystrophy, Duchenne / Q000000981:diagnostic imaging* / Q000378:metabolism*; D010759:Phosphorus Isotopes; D011877:Radionuclide Imaging; D016474:Weight-Bearing / Q000502:physiology; D055815:Young Adult
chemical_list: D010759:Phosphorus Isotopes