Carter C J
Life Sci. 1982 Sep 13;31(11):1151-9. doi: 10.1016/0024-3205(82)90090-x.
Glutamine synthetase activity was measured in seven brain areas post-mortem from control patients, and those with Huntington's disease. The activity of the enzyme was reduced in the frontal and temporal cortex, putamen and cerebellum, but not in the hippocampus, thalamus or olivary nucleus. The results do not suggest a generalised deficiency of glutamine synthetase in Huntington's disease. However, as this enzyme is localised to astrocytic cells, the reduction in activity in areas of neuronal devastation, where the ration of astrocytes to neurones is increased, may reflect a greater functional deficit. The enzyme plays a crucial role in cerebral ammonia assimilation and its inhibition in laboratory animals is known to produce neuronal toxicity. A reduction in its activity in Huntington's disease may well contribute to the neuronal pathology in certain areas.
在对对照组患者以及患有亨廷顿舞蹈症患者的七处脑区进行尸检时，测定了谷氨酰胺合成酶的活性。该酶在额叶和颞叶皮质、壳核及小脑中的活性降低，但在海马体、丘脑或橄榄核中未降低。这些结果并不表明亨廷顿舞蹈症患者普遍缺乏谷氨酰胺合成酶。然而，由于这种酶定位于星形胶质细胞，在神经元破坏区域（星形胶质细胞与神经元的比例增加）活性降低，可能反映出更大的功能缺陷。该酶在脑氨同化中起关键作用，已知在实验动物中抑制它会产生神经元毒性。其在亨廷顿舞蹈症中的活性降低很可能促成了某些区域的神经元病变。
