神经变性构象病及其分子基础

构象病的概念被广泛用于命名与蛋白质的构象异常相关的疾病。随着生命科学的进步,人们对神经变性疾病发病的分子机制有了较好的认识,发现几乎所有的此类疾病,诸如阿尔采末病（AD）、帕金森病（PD）、亨廷顿病（HD）以及朊蛋白病（PrD）等都具有一个共同的特征,即病变细胞中蓄积有大量错误折叠并易于聚合的蛋白质,这符合构象病的特点,所以又派生了神经变性构象病的新概念。近年来,人们在神经变性构象病的蛋白质错误折叠和聚合以及其细胞毒性方面的认识越来越走向深入,这将对寻找有效的治疗方法起到极大的推动作用。

Neurodegenerative conformational disease and its molecular bases

In recent years,the term "conformational disease" has been used to describe a range of disorders which are linked to misfolding and aberrant structural change in proteins.The molecular bases underlying the pathogenesis of neurodegenerative diseases are gradually being disclosed,and almost all of the diseases such as Alzheimer's disease,Parkinson's disease,Huntington's disease and prion disease are increasingly being realized to have common molecular mechanisms including the accumulation of misfolded or aggregation-prone proteins,thus,they can be termed "neurodegenerative conformational disease".There is now increased understanding of the molecular pathways involved in protein misfolding and aggregation and cellular toxicity in neurodegenerative conformational diseases.These are leading to approaches toward rational therapeutics.