Guidetti P, Reddy P H, Tagle D A, Schwarcz R
Maryland Psychiatric Research Center, Baltimore, MD 21228, USA.
Neurosci Lett. 2000 Apr 14;283(3):233-5. doi: 10.1016/s0304-3940(00)00956-3.
Several neuroactive metabolites of the kynurenine pathway of tryptophan degradation have been speculatively linked to the pathophysiology of Huntington's Disease (HD). Here we demonstrate that the levels of two of these metabolites, the free radical generator 3-hydroxykynurenine (3HK) and the neuroprotectant kynurenate (KYNA), are increased in the neostriatum of stage 1 HD patients and in the brain of mice transgenic for full-length mutant huntingtin. In both cases, the elevation in 3HK was far more pronounced, resulting in significant increases in the 3HK/KYNA ratios. These data suggest that abnormal kynurenine pathway metabolism may play a role during the early phases of the neurodegenerative process in HD.
色氨酸降解的犬尿氨酸途径中的几种神经活性代谢产物被推测与亨廷顿舞蹈症（HD）的病理生理学有关。在此我们证明，在1期HD患者的新纹状体以及全长突变亨廷顿蛋白转基因小鼠的大脑中，这两种代谢产物中的两种，即自由基生成剂3-羟基犬尿氨酸（3HK）和神经保护剂犬尿喹啉酸（KYNA）的水平升高。在这两种情况下，3HK的升高更为明显，导致3HK/KYNA比值显著增加。这些数据表明，异常的犬尿氨酸途径代谢可能在HD神经退行性过程的早期阶段起作用。
