title: Overexpression of semicarbazide-sensitive amine oxidase in human myopathies.
abstract: Oxidative stress has been implicated in the pathogenesis of several muscle diseases. Semicarbazide-sensitive amine oxidase (SSAO) metabolizes oxidative deamination of primary aromatic and aliphatic amines. In the oxidative reactions, amine substrates are converted into the aldehyde, followed by the production of ammonia and H(2)O(2). Although normal levels in muscle are very low, SSAO is expressed in almost all mammalian tissues. In this study, we examined the possible implication of SSAO as an additional source of oxidative stress in the pathogenesis of muscle disorders. The expression of SSAO was examined immunohistochemically in muscle biopsy specimens from patients with inclusion-body myositis (IBM; n = 5), desmin-related myopathy (DRM; n = 3), dermatomyositis (n = 3), granulomatous (sarcoid) myopathy (n = 2), muscle denervation-reinnervation (n = 3), and rhabdomyolysis (n = 2), as well as from control subjects (n = 3). Strong SSAO immunoreactivity was present in vacuolated and nonvacuolated fibers in IBM, in abnormal fibers in DRM, and in degenerating and regenerating fibers in dermatomyositis and rhabdomyolysis. In addition, SSAO overexpression was observed in muscle fibers adjacent to granulomas in sarcoid myopathy. These results suggest that SSAO is a source of oxidative stress in diseased human skeletal muscle and that it contributes to oxidative stress-induced damage in various inflammatory and other myopathies. Alternatively, the expression of SSAO in muscle fibers may be a consequence of muscle fiber injury.
authors.affiliation: Institut de Neuropatologia, Hospital Universitari de Bellvitge, 08907 Hospitalet de Llobregat, Barcelona, Spain. 25169mop@comb.es
authors.forename: Montse
authors.initials: M
authors.lastname: Olivé
authors.forename: Mercè
authors.initials: M
authors.lastname: Unzeta
authors.forename: Dolores
authors.initials: D
authors.lastname: Moreno
authors.forename: Isidro
authors.initials: I
authors.lastname: Ferrer
mesh_terms: D000368:Aged; D006631:Amine Oxidase (Copper-Containing) / Q000096:biosynthesis* / Q000235:genetics; D005260:Female; D015971:Gene Expression Regulation, Enzymologic / Q000502:physiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D018485:Muscle Fibers, Skeletal / Q000201:enzymology* / Q000473:pathology; D009135:Muscular Diseases / Q000201:enzymology* / Q000235:genetics / Q000473:pathology; D009220:Myositis / Q000201:enzymology / Q000235:genetics / Q000473:pathology; D012206:Rhabdomyolysis / Q000201:enzymology / Q000235:genetics / Q000473:pathology
chemical_list: D006631:Amine Oxidase (Copper-Containing)