Cardiovascular Research Institute, Biochemistry and Biophysics Department, University of California San Francisco, California 94158, USA.
Nat Rev Mol Cell Biol. 2012 Nov;13(11):687-99. doi: 10.1038/nrm3461.
Nuclear pore complexes (NPCs) are multiprotein aqueous channels that penetrate the nuclear envelope connecting the nucleus and the cytoplasm. NPCs consist of multiple copies of roughly 30 different proteins known as nucleoporins (NUPs). Due to their essential role in controlling nucleocytoplasmic transport, NPCs have traditionally been considered as structures of ubiquitous composition. The overall structure of the NPC is indeed conserved in all cells, but new evidence suggests that the protein composition of NPCs varies among cell types and tissues. Moreover, mutations in various nucleoporins result in tissue-specific diseases. These findings point towards a heterogeneity in NPC composition and function. This unexpected heterogeneity suggests that cells use a combination of different nucleoporins to assemble NPCs with distinct properties and specialized functions.
核孔复合体(NPC)是一种多蛋白水通道,贯穿核膜,连接细胞核和细胞质。NPC 由大约 30 种不同的蛋白质组成,这些蛋白质被称为核孔蛋白(NUPs)。由于 NPC 在控制核质运输方面的重要作用,它们通常被认为是具有普遍组成的结构。NPC 的整体结构在所有细胞中确实是保守的,但新的证据表明,NPC 的蛋白质组成在细胞类型和组织之间存在差异。此外,各种核孔蛋白的突变会导致组织特异性疾病。这些发现表明 NPC 的组成和功能存在异质性。这种出乎意料的异质性表明,细胞使用不同的核孔蛋白组合来组装具有不同特性和特定功能的 NPC。