Quinolinic acid catabolism is increased in cerebellum of patients with dominantly inherited olivopontocerebellar atrophy.

We measured the activities of the enzymes responsible for the metabolism of the excitotoxin quinolinic acid, 3-hydroxyanthranilate oxygenase and quinolinic acid phosphoribosyltransferase, in autopsied brain of 11 patients with olivopontocerebellar atrophy. In cerebellar cortex, severe Purkinje cell loss was evident but with relative preservation of granule cells. As compared with the control subjects (n = 14), mean activity of 3-hydroxyanthranilate oxygenase was normal in cerebellar cortex from the patients with olivopontocerebellar atrophy, whereas quinolinic acid phosphoribosyltransferase activity was markedly increased (+92%, p less than 0.02). No significant changes in enzyme activities were found in samples from occipital cortex. Increased quinolinic acid phosphoribosyltransferase activity may represent a mechanism, in the degenerating cerebellum, to protect quinolinic acid-sensitive granule cells in patients with olivopontocerebellar atrophy.
AuthorsS J Kish, F Du, D A Parks, Y Robitaille, M J Ball, L Schut, O Hornykiewicz, R Schwarcz
JournalAnnals of neurology (Ann Neurol) Vol. 29 Issue 1 Pg. 100-4 (Jan 1991) ISSN: 0364-5134 [Print] UNITED STATES
PMID1825460 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • Quinolinic Acids
  • Oxidoreductases
  • 3-hydroxyanthranilic acid oxidase
  • Pentosyltransferases
  • nicotinate-nucleotide diphosphorylase (carboxylating)
  • Quinolinic Acid
  • Adult
  • Cerebellum (enzymology, metabolism)
  • Humans
  • Immunohistochemistry
  • Neurons (chemistry, metabolism)
  • Olivopontocerebellar Atrophies (enzymology, genetics, metabolism)
  • Oxidoreductases (analysis, metabolism)
  • Pentosyltransferases (analysis, metabolism)
  • Quinolinic Acid
  • Quinolinic Acids (metabolism)

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