Malgaroli Antonio, Vallar Lucia, Zimarino Vincenzo
Scientific Institute San Raffaele, University Vita-Salute San Raffaele, Via Olgettina 58 20132 Milan, Italy.
Curr Opin Neurobiol. 2006 Jun;16(3):270-4. doi: 10.1016/j.conb.2006.05.009. Epub 2006 May 19.
In neuronal cells, proteins are synthesized on ribosomes from the genetic information encoded in DNA. In some instances translation takes place at the neuronal cell soma but in other it occurs at distal location, such as in a dendritic spine. Folding is usually initiated before the completion of protein synthesis and its outcome strictly depends on the local environment in which the nascent protein is submerged. Incompletely folded proteins and, more importantly, misfolded proteins are under the surveillance of several quality control systems that re-establish the correct conformation or initiate protein degradation. Regulation and maintenance of these systems is a vital issue for neuronal and glial cells, and impairments at different levels leads to neurodegenerative diseases.
在神经元细胞中，蛋白质由DNA编码的遗传信息在核糖体上合成。在某些情况下，翻译发生在神经元细胞体，但在其他情况下则发生在远端位置，如树突棘。折叠通常在蛋白质合成完成之前就开始了，其结果严格取决于新生蛋白质所处的局部环境。未完全折叠的蛋白质，更重要的是错误折叠的蛋白质，受到几种质量控制系统的监测，这些系统会重新建立正确的构象或启动蛋白质降解。这些系统的调节和维持对神经元和神经胶质细胞来说是一个至关重要的问题，不同层面的损伤会导致神经退行性疾病。
