期刊
NUCLEUS
卷 7, 期 3, 页码 233-248出版社
TAYLOR & FRANCIS INC
DOI: 10.1080/19491034.2016.1183848
关键词
chromatin; emerin; farnesylation; Hutchinson-Gilford progeria syndrome; laminopathies; LAP2; LBR; mechanotransduction; metabolism; muscles; nuclear envelope; nuclear lamina
类别
资金
- NIH [RO1 GM094513-01]
- Spanish Ministry of Economy and Competitiveness [BFU2013-42709P]
- European Regional Development Fund
- NATIONAL INSTITUTE OF GENERAL MEDICAL SCIENCES [R01GM094513] Funding Source: NIH RePORTER
- British Heart Foundation [RG/11/14/29056] Funding Source: researchfish
The nuclear lamina (NL) is a structural component of the nuclear envelope and makes extensive contacts with integral nuclear membrane proteins and chromatin. These interactions are critical for many cellular processes, such as nuclear positioning, perception of mechanical stimuli from the cell surface, nuclear stability, 3-dimensional organization of chromatin and regulation of chromatin-binding proteins, including transcription factors. The NL is present in all nucleated metazoan cells but its composition and interactome differ between tissues. Most likely, this contributes to the broad spectrum of disease manifestations in humans with mutations in NL-related genes, ranging from muscle dystrophies to neurological disorders, lipodystrophies and progeria syndromes. We review here exciting novel insight into NL function at the cellular level, in particular in chromatin organization and mechanosensation. We also present recent observations on the relation between the NL and metabolism and the special relevance of the NL in muscle tissues. Finally, we discuss new therapeutic approaches to treat NL-related diseases.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据