亨廷顿舞蹈病和阿尔茨海默型痴呆症患者受影响脑区内质网相关酶的缺失。

Loss of endoplasmic reticulum-associated enzymes in affected brain regions in Huntington's disease and Alzheimer-type dementia.

作者信息

Cross A J, Crow T J, Johnson J A, Dawson J M, Peters T J

出版信息

J Neurol Sci. 1985 Dec;71(2-3):137-43. doi: 10.1016/0022-510x(85)90055-3.

Abstract

Post-mortem brain samples from patients with either Huntington's disease, Alzheimer-type dementia or appropriate controls were assayed for endoplasmic reticulum enzymes, NADPH-cytochrome c reductase, neutral alpha-glucosidase, inosine diphosphatase, alpha-mannosidase and glucose-6-phosphatase and for Golgi enzymes, fucosyl- and galactosyl-transferases. In Alzheimer-type dementia there was a selective decrease in alpha-glucosidase activity in the temporal cortex. In Huntington's disease there was a selective decrease of putamen alpha-glucosidase and fucosyl-transferase activities. It is suggested that these changes reflect highly specific alterations in glycoprotein synthesis and processing and may contribute to the underlying pathology of these disorders.

摘要

对患有亨廷顿舞蹈症、阿尔茨海默型痴呆症的患者或合适的对照者的脑尸检样本进行内质网酶、NADPH - 细胞色素c还原酶、中性α - 葡萄糖苷酶、肌苷二磷酸酶、α - 甘露糖苷酶和葡萄糖 - 6 - 磷酸酶以及高尔基体酶、岩藻糖基转移酶和半乳糖基转移酶的检测。在阿尔茨海默型痴呆症中，颞叶皮质的α - 葡萄糖苷酶活性有选择性降低。在亨廷顿舞蹈症中，壳核的α - 葡萄糖苷酶和岩藻糖基转移酶活性有选择性降低。提示这些变化反映了糖蛋白合成与加工中的高度特异性改变，可能与这些疾病的潜在病理过程有关。