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Reduced Formation of Hippuric Acid After Oral Benzoic Acid in Friedreich’s Ataxia

Published online by Cambridge University Press:  18 September 2015

A. Barbeau ,
R. Bouchard ,
T. Cloutier  and
J.P. Bouchard
A. Barbeau*
Affiliation:
Clinical Research Institute of Montreal, Québec
R. Bouchard
Affiliation:
l’Hôpital del’Enfant-Jèsus, Québec
T. Cloutier
Affiliation:
Clinical Research Institute of Montreal, Québec
J.P. Bouchard
Affiliation:
l’Hôpital del’Enfant-Jèsus, Québec
*
Clinical Research Institute of Montreal, 110 Pine Avenue West, Montreal, Quebec, Canada H2W 1R7
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We have observed a markedly decreased formation of hippuric acid after benzoic acid load in patients with typical Friedreich’s Ataxia compared to normal control subjects. Since there is evidence for normal or even enhanced tauro-conjugation in the bile of patients with this disease, with a decreased G/T ratio, it is unlikely that co-factor or enzyme concentrations are the cause of this defect. We postulate decreased availability of the enzyme for glycine conjugation either to bile acids in the usual situation or to benzoic acid in the artefactual test condition. This could be due to the enzyme’s preference for an increased amount of taurine substrate in the liver. The relationship of this observation to the other biochemical changes observed in Friedreich’s Ataxia must still be established.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 9 , Issue 2 , May 1982 , pp. 217 - 220
Copyright
Copyright © Canadian Neurological Sciences Federation 1982

References

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