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Skeletal muscle NAD⁺(P) and NADP+-dependent malic enzyme in Friedreich's ataxia

Laboratory of Neurometabolic Diseases, Istituto Neurologico C. Besta, Milan, Italy.

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.

Address correspondence and reprint requests to Dr. Di Donato. Laboratory of Neurometabolic Diseases, Istituto Neurologico "C. Besta", via Celoria 11, 20133 Milan, Italy.

This research was carried out under a grant of the Muscular Dystrophy Association, USA, and research grant No. CT 81.00146.04 of the Consiglio Nazionale delle Ricerche, Italy.

Accepted for publication September 20, 1982.