Normal fibroblast mitochondrial malic enzyme activity in Friedreich's ataxia

R. J. Fernandez, Francisco Civantos, E. Tress, W. A. Maltese, D. C. De Vivo

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

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 previously reported disturbance in fibroblast mitochondrial malic enzyme may be an epiphenomenon in patients with the FA phenotype. Further studies are necessary to define the primary genetic defect in this inherited disorder.

Original languageEnglish
Pages (from-to)869-872
Number of pages4
JournalNeurology
Volume36
Issue number6
StatePublished - Sep 10 1986
Externally publishedYes

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Friedreich Ataxia
Fibroblasts
Enzymes
Digitonin
Isoenzymes
Phenotype

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Fernandez, R. J., Civantos, F., Tress, E., Maltese, W. A., & De Vivo, D. C. (1986). Normal fibroblast mitochondrial malic enzyme activity in Friedreich's ataxia. Neurology, 36(6), 869-872.

Normal fibroblast mitochondrial malic enzyme activity in Friedreich's ataxia. / Fernandez, R. J.; Civantos, Francisco; Tress, E.; Maltese, W. A.; De Vivo, D. C.

In: Neurology, Vol. 36, No. 6, 10.09.1986, p. 869-872.

Research output: Contribution to journalArticle

Fernandez, RJ, Civantos, F, Tress, E, Maltese, WA & De Vivo, DC 1986, 'Normal fibroblast mitochondrial malic enzyme activity in Friedreich's ataxia', Neurology, vol. 36, no. 6, pp. 869-872.
Fernandez RJ, Civantos F, Tress E, Maltese WA, De Vivo DC. Normal fibroblast mitochondrial malic enzyme activity in Friedreich's ataxia. Neurology. 1986 Sep 10;36(6):869-872.
Fernandez, R. J. ; Civantos, Francisco ; Tress, E. ; Maltese, W. A. ; De Vivo, D. C. / Normal fibroblast mitochondrial malic enzyme activity in Friedreich's ataxia. In: Neurology. 1986 ; Vol. 36, No. 6. pp. 869-872.
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