title: Alteration of free calcium levels and acylphosphatase muscular isoenzyme in cultured dystrophic skin fibroblasts.
abstract: Levels of free intracellular calcium have been measured on two cell lines of cultured human fibroblasts carrying the genetic lesions occurring in Duchenne and Becker dystrophies. Both cell lines elicited a markedly higher content of the cation (98 nM and 57 nM, respectively) than control fibroblasts (35 nM). Differences toward controls were statistically significant (p < 0.01). Dystrophic fibroblasts were also found to possess a significantly reduced amount by about 50% of muscular acylphosphatase isoenzyme as compared to normal cells. As acylphosphatase was demonstrated to be involved in the regulation of Ca2+-ATPase activity from different sources, a hypothesis was formulated that could explain the disruption of calcium homeostasis as an effect of the altered acylphosphatase activity.
authors.affiliation: Department of Biochemical Sciences, Università di Firenze, Italy.
authors.forename: D
authors.initials: D
authors.lastname: Degl'Innocenti
authors.forename: A
authors.initials: A
authors.lastname: Pieri
authors.forename: A
authors.initials: A
authors.lastname: Berti
authors.forename: G
authors.initials: G
authors.lastname: Liguri
authors.forename: C
authors.initials: C
authors.lastname: Cecchi
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; D002460:Cell Line; D002478:Cells, Cultured; D005347:Fibroblasts; D006801:Humans; D007527:Isoenzymes / Q000378:metabolism*; D008297:Male; D018482:Muscle, Skeletal / Q000201:enzymology; D009136:Muscular Dystrophies / Q000378:metabolism*; D012016:Reference Values; D012867:Skin / Q000378:metabolism*; D000097024:Acylphosphatase
chemical_list: D017766:Acid Anhydride Hydrolases; D002118:Calcium; D007527:Isoenzymes; D000097024:Acylphosphatase