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散发性帕金森病蛋白酶体功能障碍及其所致的路易(小)体形成
引用本文:李兴安,张应玖,常 明,王丹萍,刘 韬,胡林森.散发性帕金森病蛋白酶体功能障碍及其所致的路易(小)体形成[J].生物化学与生物物理进展,2008,35(5):502-511.
作者姓名:李兴安  张应玖  常 明  王丹萍  刘 韬  胡林森
作者单位:1. 吉林大学第一医院神经内科,长春,130021;吉林大学分子酶学工程教育部重点实验室,长春,130021
2. 吉林大学分子酶学工程教育部重点实验室,长春,130021
3. 吉林大学第一医院神经内科,长春,130021
4. 吉林大学生命科学学院,长春,130021
摘    要:散发性帕金森病(sporadic Parkinson's disease, sPD)的主要病理特征之一是中脑黑质致密部(substantia nigra pars compacta, SNpc)残存多巴胺能神经元内核周路易(小)体(Lewy body, LB)形成.LB发生的具体原因和确切过程有待进一步阐释.来自遗传学、尸体解剖和实验科学的报道提示,蛋白酶体功能障碍及其所致的LB形成可能是按照聚集体形成途径(process of aggresomes)进行的.在聚集体形成途径过程中,异常蛋白质聚集基本上经历了非纤维化分子聚集过程(molecular crowding)以及后续的纤维化聚集过程(fibrilation of aggregation).其间,蛋白酶体功能障碍(dysfunction of proteasome)、内质网相关降解丧失(loss of endoplasmic reticulum associated degradation)、非纤维化聚集物(nonfibrilar aggregates)、聚集体(aggresomes)及至纤维化LB (fibrilar LB)等构成了sPD病变过程的主要事件.这提示在sPD病变过程中,蛋白酶体功能障碍及其所致的LB形成过程实质上是细胞信号的转导过程,其间涉及了众多的蛋白质分子.

关 键 词:散发性帕金森病  蛋白酶体功能障碍  内质网相关降解丧失  聚集物  聚集体  路易(小)体  信号转导  散发性帕金森病  蛋白酶体  功能障碍  路易  Parkinson  body  Formation  Proteasome  Dysfunction  细胞信号  形成过程  事件  构成  aggregates  聚集物  loss  endoplasmic  reticulum  associated  degradation  降解
收稿时间:2007/9/14 0:00:00
修稿时间:2007年9月14日

Dysfunction of Proteasome and Formation of Lewy body in Sporadic Parkinson's Disease
LI Xian-An,ZHANG Ying-Jiu,CHANG Ming,WANG Dan-Ping,LIU Tao and HU Lin-Sen.Dysfunction of Proteasome and Formation of Lewy body in Sporadic Parkinson''s Disease[J].Progress In Biochemistry and Biophysics,2008,35(5):502-511.
Authors:LI Xian-An  ZHANG Ying-Jiu  CHANG Ming  WANG Dan-Ping  LIU Tao and HU Lin-Sen
Institution:Laboratory for Proteomics, Department of Neurology, The First Hospital, Jilin University, Changchun 130021, China; Key Laboratory for Molecular Enzymology and Engineering, The Ministry of Education (Jilin University), Changchun 130021, China;Key Laboratory for Molecular Enzymology and Engineering, The Ministry of Education (Jilin University), Changchun 130021, China;Laboratory for Proteomics, Department of Neurology, The First Hospital, Jilin University, Changchun 130021, China;Laboratory for Proteomics, Department of Neurology, The First Hospital, Jilin University, Changchun 130021, China;College of Life Science, Jilin University, Changchun 130021, China;Laboratory for Proteomics, Department of Neurology, The First Hospital, Jilin University, Changchun 130021, China
Abstract:Lewy body (LB) in the substantia nigra pars compacta (SNpc) is one of the cardinal pathological features in sporadic Parkinson's disease (sPD). Both pathogeny and pathomechanism of LB have been set forth. However, genetic, postmortem and experimental evidences demonstrate that impaired proteasomes and concomitant LB could result in the concept of process of aggresomes, by which aggregation reaction of abnormal proteins is mainly proposed as molecular crowding of non-fibrillar proteins followed by fibrillation of aggregated proteins, and in which dysfunction of proteasomes, loss of endoplasmic reticulum-associated degradation (ERAD), aggregates, aggresomes and LB are attractive occurrences in sPD. This suggests that dysfunction of proteasome and concomitant formation of LB in sPD is basically associated with cellular process of signal transduction involved in a variety of proteins.
Keywords:sporadic Parkinson's disease(sPD)  dysfunction  of  proteasomes  loss  of  endoplasmic  reticulum- associated  degradation (ERAD)  aggregates  aggresomes  Lewy body  signal transduction
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