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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

REFERENCES

Barbeau, A. (1979) Friedreich’s Ataxia 1979: an overview. Can. J. Neurol. Sci. 6: 311–319.CrossRefGoogle ScholarPubMed
Barbeau, A. (1980) Friedreich’s Ataxia 1980: an overview of the physiopathology. Can. J. Neurol. Sci. 7:455–468.CrossRefGoogle ScholarPubMed
Barbeau, A., Breton, G., Lemieux, B. and Butterworth, R.F. (1976) Bilirubin Metabolism in Friedreich’s Ataxia – Preliminary investigation. Can. J. Neurol. Sci. 3:365–372.CrossRefGoogle ScholarPubMed
Barbeau, A., and Huxtable, R.J. editors (1978) Taurine and neurological disorders, Raven Press, New York, 1978, pp. 1–468.Google Scholar
Barbeau, A., Patenaude, F., Nadon, G., Charbonneau, M. and Cloutier, T. (1982) A possible genetic pattern of taurine urinary excretion in Friedreich’s Ataxia. Can. J. Neurol. Sci. (This issue).CrossRefGoogle Scholar
Bertrand, M., Bouchard, R., Gauthier, G.L., Bouchard, J.P. and Barbeau, A. (1982) Quantitative metabolic profiling of α-keto acids in Friedreich’s Ataxia. Can. J. Neurol. Sci. (This issue).CrossRefGoogle ScholarPubMed
Bouchard, J.P., Barbeau, A., Bouchard, R., Paquet, M. and Bouchard, R.W. (1979) A cluster of Friedreich’s Ataxia in Rimouski, Quebec. Can. J. Neurol. Sci. 6: 205–208.CrossRefGoogle ScholarPubMed
Campanella, G., Filla, A., De Falco, F., Mansi, D., Durivage, A., and Barbeau, A. (1980) Friedreich’s Ataxia in the south of Italy: A clinical and biochemical survey of 23 patients. Can. J. Neurol. Sci. 7:351–357.CrossRefGoogle ScholarPubMed
Geoffroy, G., Barbeau, A., Breton, G., Lemieux, B., Aube, M., Leger, C., and Bouchard, J.B. (1976) Clinical description and Roentgenologic evaluation of patients with Friedreich’s Ataxia. Can. J. Neurol. Sci. 3: 279–286.CrossRefGoogle ScholarPubMed
Gottfries, A., Schersten, T. and Ekdahl, P.H. (1966) The capacity of human liver homogenates to synthesize taurocholic and glycocholic acid in vitro- an experimental study in patients with presumably normal liver function. Scand. J. Clin, and Lab. Invest. 18: 643–653.CrossRefGoogle Scholar
Hamel, E., Bedard, D., Laviolette, F., Butterworth, R.F. and Barbeau, A. (1978) Familial hyperbilirubinemia in Friedreich’s Ataxia. Can. J. Neurol. Sci. 5:101–105.CrossRefGoogle ScholarPubMed
Hofmann, A.F. (1976) The enterohepatic circulation of bile acids inman. Adv. Int. Med. 21:501–534.Google Scholar
Huxtable, R. and Barbeau, A. editors (1976) Taurine, Raven Press, New York, 1976. pp. 1–397.Google ScholarPubMed
Killengerg, P.G. and Jordan, J.T. (1978) Purification and characterization of bile acid-CoA: amino acid N-Acyltransferase from rat liver. J. Biol. Chem. 253: 1005–1010.CrossRefGoogle Scholar
Lemieux, B., Barbeau, A., Beroniade, V., Shapcott, D., Breton, G., Geoffroy, G., and Melancon, S. (1976) Amino acid metabolism in Friedreich’s Ataxia. Can. J. Neurol. Sci. 3: 373–378.CrossRefGoogle ScholarPubMed
Morgan, R.O., NagliE, G., Horrobin, D.F. and Barbeau, A. (1979) Erythrocyte protoporphyrin levels in patients with Friedreich’s and other ataxias. Can. J. Neurol. Sci. 6: 227–232.CrossRefGoogle ScholarPubMed
Quick, A.J. (1936) Clinical value of test for hippuric acid in cases of diseases of liver. Arch. Int. Med. 57: 544.CrossRefGoogle Scholar
Sjovall, J. (1959) Dietary glycine and taurine in bile acid conjugation in man. Proc. soc. exp. Biol. 100b; 676–678.CrossRefGoogle Scholar
Stumpf, D.A., Parks, J.K., Eguren, L.A. and Haas, R. (1982) Friedreich’s Ataxia: III. Mitochondrial malic enzyme deficiency. Neurology (Ny) 32: 221–227.CrossRefGoogle ScholarPubMed
Truswell, A.S., Mcveigh, S., Mitchell, W.D. and Brontestewart, B. (1965) Effect in man of feeding taurine on bile acid conjugates and serum cholesterol levels. J. Atheroscler. Res. 5: 526–532.CrossRefGoogle ScholarPubMed
Walker, J.L., Chamberlain, S. and Robinson, N. (1980) Lipids and lipoproteins in Friedreich’s Ataxia. J. Neurol. Neurosurg. Psychiat. 43: 111–117.CrossRefGoogle ScholarPubMed
Weichselbaum, T.E., Probstein, J.G. (1938) Determination of hippuric acid in urine. J. Lab. Clin. Med. 24: 636–639.Google Scholar