Dziewulska Dorota, Rafałowska Janina
Department of Neurology, Medical University of Warsaw, Banacha 1a, 02-097 Warsaw, Poland.
Folia Neuropathol. 2005;43(2):51-63.
Neurodegenerative disorders are characterized by accumulation of "toxic", pathologic proteins in brain cells. Mutations in genes coding these proteins result in conformational disturbances of the protein structure and their accumulation and aggregation manifesting at the level of light microscope as various intracellular inclusions. This paper is an attempt of approach cellular mechanisms underlying neurodegenerative disorders with special attention to mechanisms of protein elimination.
神经退行性疾病的特征是脑细胞中“有毒”病理蛋白的积累。编码这些蛋白的基因突变导致蛋白质结构的构象紊乱,其积累和聚集在光学显微镜水平表现为各种细胞内包涵体。本文试图探讨神经退行性疾病潜在的细胞机制,特别关注蛋白质清除机制。