Friedreich ataxia: Ii. Normal kinetics of lipoamide dehydrogenase

David A. Stumpf, Janice K. Parks

Research output: Contribution to journalArticlepeer-review

46 Scopus citations


Lipoamide dehydrogenase (LAD) kinetic values, Km and Vmax, were normal in 11 patients with Friedreich ataxia. Fibroblast activities of the pyruvate and α-ketoglutarate dehydrogenase complex, and LAD activities, were also normal. There was no reduction in oxidative decarboxylation of pyruvate, α-ketoglutarate, or several other substrates in intact fibroblasts. Methodologic differences may account for differences of opinion about putative abnormalities of the α-ketoacid dehydrogenase complexes.

Original languageEnglish (US)
Pages (from-to)820-826
Number of pages7
Issue number6
StatePublished - Jan 1 1979

ASJC Scopus subject areas

  • Clinical Neurology


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