title: 3-Hydroxyanthranilate oxygenase activity is increased in the brains of Huntington disease victims.
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.
authors.affiliation: Maryland Psychiatric Research Center, University of Maryland School of Medicine, Baltimore 21228.
authors.forename: R
authors.initials: R
authors.lastname: Schwarcz
authors.forename: E
authors.initials: E
authors.lastname: Okuno
authors.forename: R J
authors.initials: RJ
authors.lastname: White
authors.forename: E D
authors.initials: ED
authors.lastname: Bird
authors.forename: W O
authors.initials: WO
authors.lastname: Whetsell
mesh_terms: D050561:3-Hydroxyanthranilate 3,4-Dioxygenase; D001921:Brain / Q000201:enzymology*; D001931:Brain Mapping; D002421:Caudate Nucleus / Q000201:enzymology; D049308:Dioxygenases; D006801:Humans; D006816:Huntington Disease / Q000201:enzymology*; D007700:Kinetics; D010105:Oxygenases / Q000378:metabolism*; D017378:Quinolinic Acid; D011805:Quinolinic Acids / Q000378:metabolism
chemical_list: D011805:Quinolinic Acids; D010105:Oxygenases; D049308:Dioxygenases; D050561:3-Hydroxyanthranilate 3,4-Dioxygenase; D017378:Quinolinic Acid