Endosome-lysosomes, ubiquitin and neurodegeneration.
Endosome-lysosomes, ubiquitin and neurodegeneration.
复制标题
内体-溶酶体、泛素和神经变性。
DOI：
--
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发表时间：
1996
影响因子：
--
通讯作者：
M. Landon
中科院分区：
文献类型：
--
作者：
R. Mayer;C. Tipler;J. Arnold;L. Laszlo;A. Al;J. Lowe;M. Landon
在泛素免疫化学和免疫金电子显微镜出现之前，神经退行性疾病之间尚无已知的细胞内分子共性。主要检测泛素蛋白缀合物的抗体的应用表明，所有人类和动物特发性和传染性慢性神经退行性疾病（包括阿尔茨海默病（AD）、路易体病（LBD）、肌萎缩侧索硬化症（ALS）、克雅氏病（CJD）和痒病）都与某种形式的含有泛素蛋白缀合物的神经元内包涵体相关。此外，阿尔茨海默病、克雅氏病和羊痒病等疾病的特征是淀粉样蛋白沉积，这是由于膜蛋白不完全分解而产生的，这可能与细胞骨架重组有关。尽管我们对这些疾病的了解不断增加，但它们在很大程度上仍然无法治愈。最近，人们的注意力集中在不同类型淀粉样蛋白的产生机制以及可能参与内体-溶酶体系统的细胞内，这些细胞器对泛素蛋白缀合物呈免疫阳性。这些细胞器可能是膜蛋白展开和部分降解以产生淀粉样物质或其前体的“生物反应器”位点，淀粉样物质或其前体随后从细胞中排出，或从死细胞中释放，并作为病理实体累积。疾病过程的这些共同特征为治疗干预提供了新的方向。
Before the advent of ubiquitin immunochemistry and immunogold electron microscopy, there was no known intracellular molecular commonality between neurodegenerative diseases. The application of antibodies which primarily detect ubiquitin protein conjugates has shown that all of the human and animal idiopathic and transmissible chronic neurodegenerative diseases, (including Alzheimer's disease (AD), Lewy body disease (LBD), amyotrophic lateral sclerosis (ALS), Creutzfeldt-Jakob disease (CJD) and scrapie) are related by some form of intraneuronal inclusion which contains ubiquitin protein conjugates. In addition, disorders such as Alzheimer's disease, CJD and sheep scrapie, are characterised by deposits of amyloid, arising through incomplete breakdown of membrane proteins which may be associated with cytoskeletal reorganisation. Although our knowledge about these diseases is increasing, they remain largely untreatable. Recently, attention has focused on the mechanisms of production of different types of amyloid and the likely involvement within cells of the endosome-lysosome system, organelles which are immuno-positive for ubiquitin protein conjugates. These organelles may be 'bioreactor' sites for the unfolding and partial degradation of membrane proteins to generate the amyloid materials or their precursors which subsequently become expelled from the cell, or are released from dead cells, and accumulate as pathological entities. Such common features of the disease processes give new direction to therapeutic intervention.
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DOI：
10.1016/s0021-9258(18)41985-0
发表时间：
1992-08
期刊：
The Journal of biological chemistry
影响因子：
--
作者：
D. Borchelt;A. Taraboulos;S. Prusiner
通讯作者：
D. Borchelt;A. Taraboulos;S. Prusiner
受感染培养细胞细胞质囊泡中痒病朊病毒蛋白的超微结构定位。
DOI：
--
发表时间：
1991
期刊：
Laboratory investigation; a journal of technical methods and pathology
影响因子：
--
作者：
McKinley,MP;Taraboulos,A;Kenaga,L;Serban,D;Stieber,A;DeArmond,SJ;Prusiner,SB;Gonatas,N
通讯作者：
Gonatas,N
DOI：
10.1073/pnas.88.24.10998
发表时间：
1991-12-01
影响因子：
11.1
作者：
CATALDO, AM;PASKEVICH, PA;NIXON, RA
通讯作者：
NIXON, RA
正常人脑中的泛素免疫反应结构。
DOI：
--
发表时间：
1990
期刊：
Laboratory investigation; a journal of technical methods and pathology
影响因子：
--
作者：
Dickson,DW;Wertkin,A;Kress,Y;Ksiezak-Reding,H;Yen,SH
通讯作者：
Yen,SH
影响因子：
2.9
作者：
JARRETT, JT;BERGER, EP;LANSBURY, PT
通讯作者：
LANSBURY, PT