核纤层蛋白与核纤层病的研究进展  

Nuclear Lamins and Laminopathies

作　　者：郑伟[1] 曾晶[1] 徐进[1] 

机构地区：[1]宁波大学医学院,宁波315211

出　　处：《中国细胞生物学学报》2012年第12期1287-1293,共7页Chinese Journal of Cell Biology

基　　金：国家自然科学基金(No.30901216);浙江省自然科学基金(No.LY12B07002);宁波市科技创新团队(No.2011B82014)资助项目~~

摘　　要：核纤层蛋白(lamin)是中间纤维蛋白家族的重要成员,其多聚体组成的网格状结构紧贴于核膜内侧,在维持细胞核的正常及有丝分裂过程中发挥着重要的作用。近年来,大量研究表明编码核纤层蛋白的基因尤其是lamin A编码基因(LMNA)突变会引起一系列的疾病,即核纤层病(lami-nopathy)。该文就核纤层蛋白和核纤层病的关系进行综述,有助于读者了解核纤层蛋白的重要性,也为核纤层病的治疗提供线索。Nuclear lamins are type V intermediate filament proteins that polymerize to meshwork on the inner aspect of the inner nuclear membrane. Long known to be essential for maintaining nuclear structure and play- ing an important role during mitosis, research over the past dozen years has shown that mutations in genes encod- ing nuclear lamins, particularly LMNA encoding the A-type lamins, cause a broad range of diverse diseases, often referred to as laminopathies. To help readers understand the importance of lamin and also provide clues for the treatment of laminopathies, this review summarize current state of knowledge about the functions of lamins and pathogenic mechanisms underlying the laminopathies.

关 键 词：中间纤维 核纤层蛋白 核纤层病 LMNA基因 

分 类 号：R363[医药卫生—病理学]