蛋白质的错误折叠与疾病

Protein Misfolding and Disease.

蛋白质是生物体内一切功能的执行者 .人体内的任何功能 ,从催化化学反应到抵御外来侵略都是蛋白质作用的结果 .蛋白质折叠是生命活动的最基本过程 ,近年发现蛋白质的错误折叠可以导致一些疾病 .蛋白质的错误折叠与疾病的关系已成为分子生物学新的研究前沿 .介绍了细胞内保证蛋白质正常功能的“质量控制”系统 ,重点讨论了翻译后的质量控制。

Proteins take center stage in directing the working of living cells. Every conceivable role within human bodies is played by proteins, from catalysis chemical reactions to defence against alien attack. A variety of quality control mechanisms that operate in the endoplasmic reticulum in downstream compart ments of the secretary pathway to ensure the fidelity and regulation of protein expression during cell life and differentiation were introduced. The posttrans lational quality control and its relation with protein misfolding were discussed in details. A number of diseases related with protein misfolding were introduced and the principles of therapy were discussed.