核纤层蛋白与Hutchinson-Gilford早老症的研究进展

Research progress on lamin and Hutchinson-Gilford progeria syndrome

细胞骨架核纤层蛋白(lamin)表达、修饰的异常与衰老密切相关。Hutchinson-Gilford早老症(HGPS)是一种由编码核纤层蛋白基因(LMNA)突变引起的罕见、严重的早老性疾病。其病程与健康人体的衰老过程极为类似,故对HGPS的研究有助于人们了解衰老的机制,研发抗衰老的药物。现综述了近年来关于核纤层蛋白、HGPS的发病机制及治疗的相关研究,旨在为更多相关基础与临床方面研究提供帮助。

Many studies have shown that abnormal expression and modification of lamin are closely related to aging.Hutchinson-Gilford progeria syndrome(HGPS)is a rare and severe premature aging disease caused by mutations in the gene encoding nuclear envelope proteins of A-type lamins(LMNA). The pathogenesis of HGPS is similar to the aging process of normal individuals, thus research on HGPS will be helpful for understanding the mechanisms of senescence and developing anti-aging drugs.This paper reviews recent advances in lamin and the pathogenesis and treatment of HGPS, in order to provide a reference for further basic and clinical research on HGPS.