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Lipoamide dehydrogenase

Rapid heat inactivation in platelets of patients with recessively inherited ataxia

From the Department of Neurology. Reed Neurological and Jerry Lewis Neuromuscular Research Centers (Drs. Kark, Budelli, and Reckeri, and Department of Biomathematics (Dr. Forsythel, UCLA School of Medicine, and the Departments of Neurology and Microbiology (Dr. Weinerl, University of southern California School of Medicine, Los Angeles, CA.

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.

Address correspondence and reprint requests to Dr. Kark, Reed Neurological Research Center, UCLA School of Medicine, Los Angeles, CA 90024.

This work was supported in part by USPHS grants Nos. NS-15245, HD-04612, and RR-865, and by grants from the Muscular Dystrophy Association and the Easter Seal Research Foundation; and by donations from the Louis B. Mayer Foundation, Mrs. Marjorie L. Johnson, the Friedreich's Ataxia Group In America, and the National Ataxia Foundation.

Accepted for publication April 21, 1980.

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