Butterworth J, Yates C M, Simpson J
J Neurochem. 1983 Aug;41(2):440-7. doi: 10.1111/j.1471-4159.1983.tb04761.x.
Involvement of phosphate-activated glutaminase in Huntington's disease and agonal state was investigated in caudate nucleus and frontal cortex from postmortem brains. In Huntington's disease the activities of phosphate-activated glutaminase, glutamic acid decarboxylase, succinic dehydrogenase, choline acetyltransferase, and acetylcholinesterase were significantly reduced in the caudate nucleus, but not in the frontal cortex. The activity of phosphate-activated glutaminase, and to a lesser extent of glutamic acid decarboxylase, was reduced in cases of terminal illness, as compared with cases of sudden death. Succinic dehydrogenase and choline acetyltransferase were reduced only in the few cases of prolonged and severe terminal illness. Enzyme activities of the caudate nucleus were more affected by agonal state than were those of frontal cortex. Results indicate that phosphate-activated glutaminase could be a useful marker of neuronal damage due to agonal state, and that phosphate-activated glutaminase and succinic dehydrogenase are reduced in Huntington's disease.
在死后大脑的尾状核和额叶皮质中，研究了磷酸激活谷氨酰胺酶在亨廷顿舞蹈症及濒死状态中的作用。在亨廷顿舞蹈症中，尾状核中磷酸激活谷氨酰胺酶、谷氨酸脱羧酶、琥珀酸脱氢酶、胆碱乙酰转移酶和乙酰胆碱酯酶的活性显著降低，但额叶皮质中未出现这种情况。与猝死病例相比，在终末期疾病病例中，磷酸激活谷氨酰胺酶的活性降低，谷氨酸脱羧酶的活性在较小程度上也降低。仅在少数长期严重终末期疾病病例中，琥珀酸脱氢酶和胆碱乙酰转移酶降低。尾状核的酶活性比额叶皮质的酶活性受濒死状态的影响更大。结果表明，磷酸激活谷氨酰胺酶可能是濒死状态导致神经元损伤的一个有用标志物，并且在亨廷顿舞蹈症中磷酸激活谷氨酰胺酶和琥珀酸脱氢酶降低。
