A型核纤层蛋白的研究进展

Research Progress in A-type Lamins

A型核纤层蛋白由LMNA基因编码,为核纤层的主要成分,呈动态网状结构,位于核膜下层,起重要的机械支持作用,直接或间接与染色质相互作用,在维持染色质结构、转录、DNA复制和细胞凋亡等方面发挥重要作用。LMNA基因及其编码蛋白lamin A/C异常能引起一组人类遗传病,称为核纤层蛋白病。为深入了解A型核纤层蛋白的正常生理功能及其在相关核纤层蛋白病中的作用,本文就A型核纤层蛋白的结构分类、修饰组装、动力学、相互作用蛋白及相关核纤层蛋白病等方面进行综述。

A-type lamins are encoded by LMNA and a major component of the nuclear lamina, a dynamic meshwork located just under the nuclear envelope, providing essential mechanical support. In addition, A-type lamins associate with chromatin both directly and indirectly and have been suggested to play important roles in chromatin organization, transcription, DNA replication, and apoptosis. Mutations of LMNA or its encoding proteins can cause a series of human hereditary diseases which are collectively referred to as laminopathies. To approach functions of A-type lamins and their roles in laminopathies, this review summarizes several parts of A-type lamins, including its structure, type, modification, assembly, dynam- ics, interaction proteins and laminopathies.