线粒体功能异常与朊病毒病的关系

Correlation of prion disease and mitochondrial dysfunction

朊病毒病也称传播性海绵状脑病(TSEs),是一类罕见的致死性神经退行性疾病,包括人的克雅病(CJD)、库鲁病(Kuru)、家族型致死性失眠症(FFI)、牛海绵状脑病(BSE)和羊瘙痒病(Scrapie)等。朊病毒病临床上表现为进行性共济失调、震颤、认知障碍,最终导致死亡,病理特征为中枢神经系统出现海绵样变、神经元丢失和神经胶质细胞增生等特征性改变。

Prion disease(transmissible spongiform encephalopathies,TSEs)is a fatal neuro degenerative disease.One possible pathogenesis in the development of TSEs is the transformation of normal prion protein to an abnormal form via misfolding and the accumulation of abnormal prion protein in central nervous system which eventually lead to neuronal cell death.But the mechanism of abnormal prion protein leading to neurodegenerative disease is still unclear.Mitochondrial dysfunction is a phenomenon that appears at the beginning of neuronal damage during the process of prion disease,and mitochondrial damage is observed prior to synapses dysfunction.Therefore,mitochondrial structural changes and functional abnormalities may be an important pathologic marker in the early stage of prion diseases.