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Effect of a Valine Load Test on Plasma Alpha-Keto Acids in Friedreich Ataxia

Published online by Cambridge University Press:  18 September 2015

A. Barbeau ,
M. Bertrand ,
R. Bouchard ,
G.L. Gauthier  and
J.P. Bouchard
A. Barbeau*
Affiliation:
Clinical Research Institute of Montreal
M. Bertrand
Affiliation:
The Department of Chemistry, University of Montreal
R. Bouchard
Affiliation:
Hôpital de l’Enfant-Jèsus, Quebec City
G.L. Gauthier
Affiliation:
The Department of Chemistry, University of Montreal
J.P. Bouchard
Affiliation:
Hôpital de l’Enfant-Jèsus, Quebec City
*
Clinical Research Institute of Montreal, 110 Pine Avenue West, Montréal, Quebec, Canada, H2W 1R7
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To test the physiological significance in vivo of our previous in vitro finding of reduced valine dehydrogenase (VDH) activity inpatients with Friedreich’s Ataxia, we subjected ataxic patients and controls to an oral valine load test (1.0g) and measured the levels of branched chain α-keto acids in the plasma for 24 hours. We demonstrated a significantly higher peak for α-keto isovaleric acid in Friedreich’s Ataxia and a general trend towards higher than control values in all other α-keto acids measured, and at all times in the experiment. These changes are compatible with the postulated defect in regulation of the activity of VDH in this illness, but because of their small amplitude, they also indicate that a VDH – deficiency is not the genetic defect in Friedreich’s A taxia.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 9 , Issue 2 , May 1982 , pp. 239 - 242
Copyright
Copyright © Canadian Neurological Sciences Federation 1982

References

REFERENCES

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