von Mikecz Anna
Institut für umweltmedizinische Forschung, Heinrich-Heine-Universität Düsseldorf, Auf'm Hennekamp 50, 40225 Düsseldorf, Germany.
J Cell Sci. 2006 May 15;119(Pt 10):1977-84. doi: 10.1242/jcs.03008.
In eukaryotes, thousands of genes have to be organized and expressed in the cell nucleus. Conformational and kinetic instability of nuclear structure and components appear to enable cells to use the encoded information selectively. The ubiquitin-proteasome system is active in distinct nuclear domains and plays a major role controlling the initial steps of gene expression, DNA repair and nuclear quality-control mechanisms. Recent work indicates that a tuned balance of ubiquitylation and proteasome-dependent protein degradation of nuclear proteins is instrumental in nuclear function and, when deregulated, leads to the development of diseases such as polyQ disorders and other neurodegenerative conditions.
在真核生物中，细胞核内有成千上万个基因需要进行组织和表达。核结构及组分的构象和动力学不稳定性似乎使细胞能够选择性地利用编码信息。泛素-蛋白酶体系统在不同的核区域发挥作用，在控制基因表达、DNA修复及核质量控制机制的起始步骤中起主要作用。最近的研究表明，核蛋白泛素化和蛋白酶体依赖性蛋白降解之间的平衡调节对核功能至关重要，一旦失衡，就会导致多聚谷氨酰胺疾病和其他神经退行性疾病等病症的发生。
