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malic enzyme/ataksia
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Mitochondrial malic enzyme in Friedreich's ataxia: failure to demonstrate reduced activity in cultured fibroblasts.
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Mitochondrial and cytosolic malic enzymes were assayed radiochemically in fibroblasts from six patients suffering from Friedreich's ataxia in order to verify earlier reports of abnormalities in these enzymes. No abnormalities could be detected in the activities of either enzyme. On cellulose acetate
Friedreich's ataxia: malic enzyme activity in cellular fractions of cultured skin fibroblasts.
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We have measured the activity of malic enzyme NADP+ dependent in the nuclear, mitochondrial, lysosomal and cytosolic fractions of cultured skin fibroblasts from twelve patients with Friedreich's ataxia and nine control subjects. Hexosaminidase, cytochrome-C-oxidase, lactate dehydrogenase and malic
Skeletal muscle NAD+(P) and NADP+-dependent malic enzyme in Friedreich's ataxia.
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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
Normal mitochondrial malic enzyme levels in Friedreich's ataxia fibroblasts.
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Normal levels of mitochondrial malic enzyme were found in fibroblasts from three patients with Friedreich's ataxia.
Normal fibroblast mitochondrial malic enzyme activity in Friedreich's ataxia.
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Mitochondrial and cytoplasmic malic isoenzymes were assayed fluorometrically in digitonin-fractionated fibroblasts from three patients with Friedreich's ataxia (FA). Normal activity was found, failing to verify an earlier report of reduced fibroblast mitochondrial malic enzyme activity in FA. The
Friedreich ataxia: III. Mitochondrial malic enzyme deficiency.
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Polarographic assays of oxidative phosphorylation in muscle mitochondria indicated abnormal pyruvate-malate metabolism in Friedreich ataxia (FA). Pursuing this clue, more specific assays were performed. Mitochondrial malic enzyme (MEm; malate: NADP+ oxidoreductase) specific activity was 10% of
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