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 language||English (US)|
|Number of pages||7|
|State||Published - Jan 1 1979|
ASJC Scopus subject areas
- Clinical Neurology