Friedreich ataxia: Ii. Normal kinetics of lipoamide dehydrogenase

David A. Stumpf; Janice K. Parks

Friedreich ataxia: Ii. Normal kinetics of lipoamide dehydrogenase

David A. Stumpf, Janice K. Parks

Neurology

Research output: Contribution to journal › Article › peer-review

46 Scopus citations

Abstract

Lipoamide dehydrogenase (LAD) kinetic values, K^m and V^max, 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)
Pages (from-to)	820-826
Number of pages	7
Journal	Neurology
Volume	29
Issue number	6
State	Published - Jan 1 1979

ASJC Scopus subject areas

Clinical Neurology

Cite this

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title = "Friedreich ataxia: Ii. Normal kinetics of lipoamide dehydrogenase",

abstract = "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.",

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AU - Parks, Janice K.

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N2 - 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.

AB - 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.

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Friedreich ataxia: Ii. Normal kinetics of lipoamide dehydrogenase

Abstract

ASJC Scopus subject areas

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