Brain proteasomal function in sporadic Parkinson's disease and related disorders
Brain proteasomal function in sporadic Parkinson's disease and related disorders
复制标题
DOI：
10.1002/ana.10207
复制
发表时间：
2002-06-01
影响因子：
11.2
通讯作者：
Briand, Y
中科院分区：
文献类型：
--
作者：
Furukawa, Y;Vigouroux, S;Briand, Y
由于家族性帕金森病中报道了与泛素-蛋白酶体系统相关的遗传缺陷，因此我们对蛋白酶体进行了评估。散发性帕金森氏病尸检的脑功能。我们发现，在帕金森氏病的五个脑区(包括纹状体)中，蛋白酶体特有的一部分蛋白酶体多肽酶活性被保留，这些区域没有观察到神经元丢失。帕金森氏病患者纹状体两个蛋白酶体亚基的蛋白水平正常，但在疾病对照组(多系统萎缩)纹状体蛋白水平轻度降低。我们的大脑数据表明，蛋白酶体本身的催化活性和降解能力的系统性、全球性障碍不太可能解释帕金森氏症的原因。
Because genetic defects relating to the ubiquitin-proteasome system were reported in familial parkinsonism, we evaluated proteasomal. function in autopsied brains with sporadic Parkinson's disease. We found that proteasome peptidase activities in a fraction specific to the proteasome were preserved in five brain areas (including the striatum) of Parkinson's disease where neuronal loss is not observed. Striatal protein levels of two proteasome subunits were normal in Parkinson's disease but reduced mildly in disease controls (multiple system atrophy). Our brain data suggest that a systemic, global disturbance in the catalytic activity and degradation ability of the proteasome itself is unlikely to explain the cause of Parkinson's disease.