3-HYDROXYANTHRANILATE OXYGENASE ACTIVITY IS INCREASED IN THE BRAINS OF HUNTINGTON DISEASE VICTIMS
3-HYDROXYANTHRANILATE OXYGENASE ACTIVITY IS INCREASED IN THE BRAINS OF HUNTINGTON DISEASE VICTIMS
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DOI：
10.1073/pnas.85.11.4079
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发表时间：
1988-06-01
影响因子：
11.1
通讯作者：
WHETSELL, WO
中科院分区：
文献类型：
--
作者：
SCHWARCZ, R;OKUNO, E;WHETSELL, WO
脑中色氨酸代谢物喹啉酸的过量已被假设与亨廷顿病的发病机制有关。喹啉酸的直接生物合成酶，3-羟基邻氨基苯甲酸加氧酶（EC 1.13.11.6），现已在人脑组织中检测到。与对照脑相比，亨廷顿病脑中3-羟基邻氨基苯甲酸加氧酶的活性增加。这种增加在纹状体中特别明显，已知纹状体在亨廷顿病中表现出最显著的神经细胞损失。因此，亨廷顿病脑具有不成比例的高能力来产生内源性“兴奋毒素”喹啉酸。这一发现可能与亨廷顿病相关的临床、神经病理和生化特征有关。
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.