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  • Title: Skeletal muscle NAD+(P) and NADP+-dependent malic enzyme in Friedreich's ataxia.
    Author: Bottacchi E, Di Donato S.
    Journal: Neurology; 1983 Jun; 33(6):712-6. PubMed ID: 6682515.
    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.
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