Functions of the nuclear envelope and lamina in development and disease

被引：30

作者：

Cohen, Tatiana V. ^{[2
]}

Hernandez, Lidia ^{[3
]}

Stewart, Colin L. ^{[1
]}

机构：

[1] Inst Med Biol, Singapore 138668, Singapore

[2] Childrens Natl Med Ctr, Ctr Genet Med, Washington, DC 20010 USA

[3] NCI, Med Oncol Branch CCR, Bethesda, MD 20892 USA

来源：

BIOCHEMICAL SOCIETY TRANSACTIONS | 2008年 / 36卷

关键词：

Emery-Dreifuss muscular dystrophy (EDMD); Hutchinson-Gilford progeria syndrome (HGPS); lamina; nuclear envelope;

D O I：

10.1042/BST0361329

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Recent findings that some 24 inherited diseases and anomalies are caused by defects in proteins of the NE (nuclear envelope) and lamina have resulted in a fundamental reassessment of the functions of the NE and underlying lamina. instead of just regarding the NE and lamina as a molecular filtering device, regulating the transfer of macromolecules between the cytoplasm and nucleus, we now envisage the NE/lamina functioning as a key cellular 'hub' in integrating critical functions that include chromatin organization, transcriptional regulation, mechanical integrity of the cell and signalling pathways, as well as acting as a key component in the organization and function of the cytoskeleton.

引用

页码：1329 / 1334

页数：6

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