Bridger Joanna M, Foeger Nicole, Kill Ian R, Herrmann Harald
Centre for Cell and Chromosome Biology, Division of Biosciences, Brunel University, London, UK.
FEBS J. 2007 Mar;274(6):1354-61. doi: 10.1111/j.1742-4658.2007.05694.x.
The inner face of the nuclear envelope of metazoan cells is covered by a thin lamina consisting of a one-layered network of intermediate filaments interconnecting with a complex set of transmembrane proteins and chromatin associating factors. The constituent proteins, the lamins, have recently gained tremendous recognition, because mutations in the lamin A gene, LMNA, are the cause of a complex group of at least 10 different diseases in human, including the Hutchinson-Gilford progeria syndrome. The analysis of these disease entities has made it clear that besides cytoskeletal functions, the lamina has an important role in the "behaviour" of the genome and is, probably as a consequence of this function, intimately involved in cell fate decisions. Furthermore, these functions are related to the involvement of lamins in organizing the position and functional state of interphase chromosomes as well as to the occurrence of lamins and lamina-associated proteins within the nucleoplasm. However, the structural features of these lamins and the nature of the factors that assist them in genome organization present an exciting challenge to modern biochemistry and cell biology.
后生动物细胞核膜的内表面覆盖着一层薄的核纤层，它由中间丝的单层网络组成，中间丝与一组复杂的跨膜蛋白和染色质相关因子相互连接。其组成蛋白核纤层蛋白最近获得了极大的认可，因为核纤层蛋白A基因（LMNA）的突变是导致人类至少10种不同复杂疾病的原因，包括哈钦森-吉尔福德早衰综合征。对这些疾病实体的分析表明，除了细胞骨架功能外，核纤层在基因组的“行为”中具有重要作用，并且可能由于这一功能，密切参与细胞命运的决定。此外，这些功能与核纤层蛋白参与组织间期染色体的位置和功能状态以及核纤层蛋白和核纤层相关蛋白在核质中的存在有关。然而，这些核纤层蛋白的结构特征以及协助它们进行基因组组织的因子的性质，对现代生物化学和细胞生物学提出了令人兴奋的挑战。
