神经变性疾病是一类病因未明、起病隐匿,慢性进行性发展的神经系统疾病,其病理学改变包括异常折叠蛋白沉积、神经元变性及脱髓鞘改变。越来越多文献报道,这类异常折叠蛋白与朊蛋白在分子结构与生物学行为上具有相似性,朊蛋白可能是影响神经变性疾病发生和发展的原因。本文就神经变性疾病的朊蛋白样作用机制作出探讨。 Neurodegenerative diseases are a group of nervous system diseases with unknown etiology, occult onset and chronic progressive development. The pathological changes include abnormal fold protein deposition, degeneration of neurons and demyelination. More and more reported in the literature, these abnormal protein and prion protein structure and biological behavior in the similarity, prion protein may affect the occurrence and development of neurodegenerative diseases. This paper discusses the mechanism of prion-like neurodegenerative diseases.