Scheper Gert C, van der Knaap Marjo S, Proud Christopher G
Department of Child Neurology/Center for Neurogenomics and Cognitive Research, Vrije Universiteit Medical Center, De Boelelaan 1117, 1081HV Amsterdam, The Netherlands.
Nat Rev Genet. 2007 Sep;8(9):711-23. doi: 10.1038/nrg2142. Epub 2007 Jul 31.
The list of genetic diseases caused by mutations that affect mRNA translation is rapidly growing. Although protein synthesis is a fundamental process in all cells, the disease phenotypes show a surprising degree of heterogeneity. Studies of some of these diseases have provided intriguing new insights into the functions of proteins involved in the process of translation; for example, evidence suggests that several have other functions in addition to their roles in translation. Given the numerous proteins involved in mRNA translation, it is likely that further inherited diseases will turn out to be caused by mutations in genes that are involved in this complex process.
由影响mRNA翻译的突变所导致的遗传疾病列表正在迅速增加。尽管蛋白质合成是所有细胞中的一个基本过程,但疾病表型却表现出惊人程度的异质性。对其中一些疾病的研究为参与翻译过程的蛋白质功能提供了有趣的新见解;例如,有证据表明,其中几种蛋白质除了在翻译中发挥作用外,还具有其他功能。鉴于参与mRNA翻译的蛋白质众多,很可能会发现更多的遗传性疾病是由参与这一复杂过程的基因突变引起的。