title: Skeletal muscle NAD+(P) and NADP+-dependent malic enzyme in Friedreich's ataxia.
abstract: Malic enzymes were studied in skeletal muscle from seven patients with Friedreich's ataxia (FA) and nine controls. Muscle contained three different malic enzymes. There were two strictly NADP+-dependent enzymes, one in the cytosol and one in mitochondria. These two enzymes are not allosteric. In FA muscle, activity of the mitochondrial NADP+-linked enzyme was significantly low and the cytosol NADP+-linked enzyme was significantly increased. A third malic enzyme, NAD+(P)-dependent, was found in the mitochondrial fraction. That enzyme had allosteric properties, and its activity was about the same in FA and control muscle.
mesh_terms: D000293:Adolescent; D000328:Adult; D003600:Cytosol / Q000032:analysis / Q000201:enzymology; D005260:Female; D005621:Friedreich Ataxia / Q000201:enzymology*; D006801:Humans; D008291:Malate Dehydrogenase / Q000032:analysis* / Q000172:deficiency; D008297:Male; D008875:Middle Aged; D008931:Mitochondria, Muscle / Q000032:analysis / Q000201:enzymology; D009132:Muscles / Q000201:enzymology*; D009249:NADP / Q000032:analysis* / Q000378:metabolism
chemical_list: D009249:NADP; D008291:Malate Dehydrogenase