title: Oxidative stress and calcium homeostasis in dystrophic skin fibroblasts.
abstract: Muscular dystrophy is a genetic disease that affects primarily skeletal muscle. The dystrophin absence has been related to the degeneration of muscle fibres. Indirect evidences suggest that oxidative stress may play a role in the pathogenesis of the disease, but the significance and precise extent of this contribution is poorly understood. In this paper we show that Becker Muscular Dystrophy (BMD) and Duchenne Muscular Dystrophy (DMD) skin fibroblasts are more susceptible to H2O2 treatment than are fibroblasts from unaffected persons. In particular, we found that, in growing DMD skin fibroblasts, the oxidative treatment resulted in significantly reduced growing capacity. We also investigated the concentrations of intracellular calcium during H2O2 treatment. The intracellular free calcium concentration increased by 22%, 35%, and 40% in unaffected, BMD, and DMD fibroblasts, respectively. However, the increase of the intracellular free calcium concentration is not related, as previously hypothesized, to a reduction of acylphosphatase concentrations, which seem to be unaffected by the H2O2 treatment, but rather to reduced enzyme activity.
authors.affiliation: Department of Biochemical Sciences, University of Florence, Firenze, Italy.
authors.forename: D
authors.initials: D
authors.lastname: Degl'Innocenti
authors.forename: A
authors.initials: A
authors.lastname: Pieri
authors.forename: F
authors.initials: F
authors.lastname: Rosati
authors.forename: G
authors.initials: G
authors.lastname: Ramponi
mesh_terms: D017766:Acid Anhydride Hydrolases / Q000378:metabolism; D000293:Adolescent; D002118:Calcium / Q000378:metabolism*; D002455:Cell Division / Q000187:drug effects; D002470:Cell Survival / Q000187:drug effects; D002478:Cells, Cultured; D004305:Dose-Response Relationship, Drug; D005347:Fibroblasts / Q000187:drug effects / Q000201:enzymology / Q000378:metabolism* / Q000473:pathology; D006706:Homeostasis / Q000502:physiology*; D006801:Humans; D006861:Hydrogen Peroxide / Q000633:toxicity; D007527:Isoenzymes / Q000378:metabolism; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000201:enzymology / Q000378:metabolism* / Q000473:pathology; D018384:Oxidative Stress / Q000502:physiology*; D012867:Skin / Q000187:drug effects / Q000201:enzymology / Q000378:metabolism* / Q000473:pathology; D013778:Tetrazolium Salts; D013844:Thiazoles; D000097024:Acylphosphatase
chemical_list: D017766:Acid Anhydride Hydrolases; D002118:Calcium; D006861:Hydrogen Peroxide; D007527:Isoenzymes; D013778:Tetrazolium Salts; D013844:Thiazoles; D000097024:Acylphosphatase; C022616:thiazolyl blue