分子伴侣与错误折叠疾病

近年来研究发现,蛋白质错误折叠可以导致一些疾病。蛋白质错误折叠形成非天然构象,并相互聚集。这些聚集体不仅丧失了原有的蛋白质功能,还对细胞有一定毒性。分子伴侣与错误折叠疾病间的关系正逐渐被了解。分子伴侣可识别并阻止蛋白质的错误折叠,其基因突变会引起一些人类疾病。升高的分子伴侣水平可抑制一些变异蛋白的神经毒性,这将有助于疾病的药物治疗。

Molecular chaperones and misfolding diseases

Diverse human disorders arise from protenis misfolding.The abrerrantly folded proteins reach non-native states and aggregate.Misfolded polypeptides not only loose their normal function,they may also form toxic species.Recent findings have increased our understanding of the molecular chaperone mechanism of protein conformational disorders.Chaperones recognize and counteract the misfolding process.Mutations of chaperones lead to several rare syndromes.Increased chaperones expression can suppress the neurotoxicity of these mutated proteins,which contribute viable therapeutic strategies.