Gruenbaum Yosef, Margalit Ayelet, Goldman Robert D, Shumaker Dale K, Wilson Katherine L
Department of Genetics, The Institute of Life Sciences, The Hebrew University of Jerusalem, Givat Ram, Jerusalem, 91904 Israel.
Nat Rev Mol Cell Biol. 2005 Jan;6(1):21-31. doi: 10.1038/nrm1550.
Many nuclear proteins form lamin-dependent complexes, including LEM-domain proteins, nesprins and SUN-domain proteins. These complexes have roles in chromatin organization, gene regulation and signal transduction. Some link the nucleoskeleton to cytoskeletal structures, ensuring that the nucleus and centrosome assume appropriate intracellular positions. These complexes provide new insights into cell architecture, as well as a foundation for the understanding of the molecular mechanisms that underlie the human laminopathies - clinical disorders that range from Emery-Dreifuss muscular dystrophy to the accelerated ageing seen in Hutchinson-Gilford progeria syndrome.
许多核蛋白形成依赖于核纤层的复合物,包括LEM结构域蛋白、核膜蛋白和SUN结构域蛋白。这些复合物在染色质组织、基因调控和信号转导中发挥作用。一些复合物将核骨架与细胞骨架结构相连,确保细胞核和中心体在细胞内占据适当位置。这些复合物为细胞结构提供了新的见解,也为理解导致人类核纤层蛋白病的分子机制奠定了基础,核纤层蛋白病是一系列临床疾病,从埃默里-德雷福斯肌营养不良症到哈钦森-吉尔福德早衰综合征中出现的加速衰老。