Alteration of free calcium levels and acylphosphatase muscular isoenzyme in cultured dystrophic skin fibroblasts.
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.
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Department of Biochemical Sciences, Università di Firenze, Italy.
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Berti
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Liguri
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Cecchi
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Ramponi
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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
D017766:Acid Anhydride Hydrolases; D002118:Calcium; D007527:Isoenzymes; D000097024:Acylphosphatase