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Serum and Platelet Lipoamide Dehydrogenase in Friedreich's Ataxia

Published online by Cambridge University Press:  18 September 2015

A. Filla
Affiliation:
Department of Neurobiology, Clinical Research Institute of Montreal, Hôpital Sainte-Justine, Montreal; and Centre Hospitalier Universitaire de Sherbrooke
R. F. Butterworth
Affiliation:
Department of Neurobiology, Clinical Research Institute of Montreal, Hôpital Sainte-Justine, Montreal; and Centre Hospitalier Universitaire de Sherbrooke
G. Geoffroy
Affiliation:
Department of Neurobiology, Clinical Research Institute of Montreal, Hôpital Sainte-Justine, Montreal; and Centre Hospitalier Universitaire de Sherbrooke
B. Lemieux
Affiliation:
Department of Neurobiology, Clinical Research Institute of Montreal, Hôpital Sainte-Justine, Montreal; and Centre Hospitalier Universitaire de Sherbrooke
A. Barbeau*
Affiliation:
Department of Neurobiology, Clinical Research Institute of Montreal, Hôpital Sainte-Justine, Montreal; and Centre Hospitalier Universitaire de Sherbrooke
*
Clinical Research Institute of Montreal, 110 Pine Avenue West, Montreal, Quebec, Canada H2W 1R7
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Summary:

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Pyruvate dehydrogenase (PDH), α -keto gluturate dehydrogenase (α -KGDH) and lipoamide dehydrogenase (LAD) were measured in platelets of II patients with typical Friedreich's ataxia and 10 normal control subjects. Serum LAD was also evaluated in the same patients. No statistically significant changes were found in platelets for the group as a whole, although some patients had low values (more than one standard deviation below control mean). Serum LAD was significantly reduced in the patients with Friedreich's ataxia. This was not due to associated diabetes.

Type
Quebec Cooperative Study of Friedreich's Ataxia
Copyright
Copyright © Canadian Neurological Sciences Federation 1978

References

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