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Quinolinic acid catabolism is increased in cerebellum of patients with dominantly inherited olivopontocerebellar atrophy
Author(s) -
Kish Stephen J.,
Du Fu,
Parks Deborah A.,
Robitaille Yves,
Ball Melvyn J.,
Schut Lawrence,
Hornykiewicz Oleh,
Schwarcz Robert
Publication year - 1991
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410290119
Subject(s) - quinolinic acid , olivopontocerebellar atrophy , cerebellum , endocrinology , medicine , atrophy , biology , chemistry , biochemistry , neuroscience , degenerative disease , central nervous system disease , amino acid , tryptophan
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 < 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.