3-Hydroxyanthranilate oxygenase activity is increased in the brains of Huntington disease victims

Journal Article · Wed Jun 01 00:00:00 EDT 1988 · Proceedings of the National Academy of Sciences of the United States of America; (USA)

DOI:https://doi.org/10.1073/pnas.85.11.4079· OSTI ID:5565372

Schwarcz, R; Okuno, E; White, R J; Bird, E D; Whetsell, Jr, W O ^[1]

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1. Univ. of Maryland School of Medicine, Baltimore (USA)

An excess of the tryptophan metabolite quinolinic acid in the brain has been hypothetically related to the pathogenesis of Huntington disease. Quinolinate's immediate biosynthetic enzyme, 3-hydroxyanthranilate oxygenase, has now been detected in human brain tissue. The activity of 3-hydroxyanthranilate oxygenase is increased in Huntington disease brains as compared to control brains. The increment is particularly pronounced in the striatum, which is known to exhibit the most prominent nerve-cell loss in Huntington disease. Thus, the Huntington disease brain has a disproportionately high capability to produce the endogenous excitotoxin quinolinic acid. This finding may be of relevance for clinical, neuropathologic, and biochemical features associated with Huntington disease.

• OSTI ID: 5565372
• Journal Information: Proceedings of the National Academy of Sciences of the United States of America; (USA), Journal Name: Proceedings of the National Academy of Sciences of the United States of America; (USA) Vol. 85:11; ISSN 0027-8424; ISSN PNASA
• Country of Publication: United States
• Language: English

Related Subjects

550201* -- Biochemistry-- Tracer Techniques
59 BASIC BIOLOGICAL SCIENCES
AMINO ACIDS
AROMATICS
AZAARENES
AZOLES
BIOCHEMISTRY
BODY
BRAIN
CARBOXYLIC ACIDS
CENTRAL NERVOUS SYSTEM
CHEMISTRY
DISEASES
ENZYME ACTIVITY
ENZYMES
HEREDITARY DISEASES
HETEROCYCLIC ACIDS
HETEROCYCLIC COMPOUNDS
INDOLES
METABOLITES
NERVOUS SYSTEM
NERVOUS SYSTEM DISEASES
ORGANIC ACIDS
ORGANIC COMPOUNDS
ORGANIC NITROGEN COMPOUNDS
ORGANS
OXIDOREDUCTASES
OXYGENASES
PATHOGENESIS
PATIENTS
PYRROLES
TRYPTOPHAN