期刊
JOURNAL OF CELLULAR AND MOLECULAR MEDICINE
卷 13, 期 6, 页码 1059-1085出版社
WILEY
DOI: 10.1111/j.1582-4934.2009.00676.x
关键词
nuclear lamina; nuclear envelope; chromatin; transcription; filament assembly; stem cells; cancer
资金
- Israel Ministry of Health, the German-Israeli Foundation (GIF)
- USA-Israel Binational Science Foundation (BSF)
- Marie Curie (IRG)
- European Union FP6 (eurolaminopathies)
- Israel Science Foundation (Morasha)
- Muscular Dystrophy Association (MDA)
The nuclear lamina is a proteinaceous structure located underneath the inner nuclear membrane (INM), where it associates with the peripheral chromatin. It contains lamins and lamin-associated proteins, including many integral proteins of the INM, chromatin modifying proteins, transcriptional repressors and structural proteins. A fraction of lamins is also present in the nucleoplasm, where it forms stable complexes and is associated with specific nucleoplasmic proteins. The lamins and their associated proteins are required for most nuclear activities, mitosis and for linking the nucleoplasm to all major cytoskeletal networks in the cytoplasm. Mutations in nuclear lamins and their associated proteins cause about 20 different diseases that are collectively called 'laminopathies'. This review concentrates mainly on lamins, their structure and their roles in DNA replication, chromatin organization, adult stem cell differentiation, aging, tumorogenesis and the lamin mutations leading to laminopathic diseases.
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