Lipoamide dehydrogenase

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Abstract
The activity of lipoamide dehydrogenase was abnormally heat-labile in homogenized platelets from seven patients with a recessive ataxia conforming to the syndrome of Friedreich ataxia or clinical variants. Taken together, the abnormality and previous findings of low activity and abnormal kinetic properties are compatible with a change in the conformation of the enzyme in these patients.

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