期刊
CURRENT OPINION IN CELL BIOLOGY
卷 34, 期 -, 页码 101-112出版社
CURRENT BIOLOGY LTD
DOI: 10.1016/j.ceb.2015.06.003
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类别
资金
- Wellcome Trust [095209, 092076]
- National Institutes of Health [R01AR048997, R01HD070713, R56NS059352]
- Muscular Dystrophy Association [MDA 294537]
- Los Angeles Thoracic and Cardiovascular Foundation
Human 'laminopathy' diseases result from mutations in genes encoding nuclear lamins or nuclear envelope (NE) transmembrane proteins (NETs). These diseases present a seeming paradox: the mutated proteins are widely expressed yet pathology is limited to specific tissues. New findings suggest tissue-specific pathologies arise because these widely expressed proteins act in various complexes that include tissue-specific components. Diverse mechanisms to achieve NE tissue-specificity include tissue-specific regulation of the expression, mRNA splicing, signaling, NE-localization and interactions of potentially hundreds of tissue-specific NETs. New findings suggest these NETs underlie tissue-specific NE roles in cytoskeletal mechanics, cell-cycle regulation, signaling, gene expression and genome organization. This view of the NE as 'specialized' in each cell type is important to understand the tissue-specific pathology of NE-linked diseases.
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