Tafforeau Lionel
Laboratoire de biologie cellulaire, institut de recherche en biosciences, université de Mons, place du Parc 20, 7000 Mons, Belgique.
Med Sci (Paris). 2015 Jun-Jul;31(6-7):622-8. doi: 10.1051/medsci/20153106015. Epub 2015 Jul 7.
Ribosomes are cellular ribonucleoprotein particles required for a fundamental mechanism, translation of the genetic information into proteins. Ribosome biogenesis is a highly complex pathway involving many maturation steps: ribosomal RNA (rRNA) synthesis, rRNA processing, pre-rRNA modifications, its assembly with ribosomal proteins in the nuceolus, export of the subunit precursors to the nucleoplasm and the cytoplasm. Ribosome biogenesis has mainly being investigated in yeast during these last 25 years. However, recent works have shown that, despite many similarities between yeast and human ribosome structure and biogenesis, human pre-rRNA processing is far more complex than in yeast. In order to better understand diseases related to a malfunction in ribosome synthesis, the ribosomopathies, research should be conducted directly in human cells and animal models.
核糖体是细胞中的核糖核蛋白颗粒,是将遗传信息转化为蛋白质这一基本机制所必需的。核糖体生物合成是一条高度复杂的途径,涉及许多成熟步骤:核糖体RNA(rRNA)合成、rRNA加工、前体rRNA修饰、其在核仁中与核糖体蛋白组装、亚基前体输出到核质和细胞质。在过去25年里,核糖体生物合成主要是在酵母中进行研究的。然而,最近的研究表明,尽管酵母和人类核糖体结构及生物合成之间有许多相似之处,但人类前体rRNA加工比酵母中的要复杂得多。为了更好地理解与核糖体合成功能异常相关的疾病,即核糖体病,应该直接在人类细胞和动物模型中开展研究。