Work

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

Year: 1988

Type: article

Abstract: 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 (EC 1.13.11.6), 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.

Source: Proceedings of the National Academy of Sciences

DOI: 10.1073/pnas.85.11.4079

Authors: R Schwarcz, E Okuno, R J White, E D Bird, W O Whetsell

Institutions: University of Maryland, Baltimore, Harvard University, Vanderbilt University

Language: English

Topic: Genetic Neurodegenerative Diseases

Subfield: Cellular and Molecular Neuroscience

Field: Neuroscience

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: green

Funders: Hereditary Disease Foundation, U.S. Public Health Service, National Institute of Mental Health, National Institute of Neurological Disorders and Stroke