瑞典卡罗林斯卡医学院的研究人员日前宣布，他们发现了一种线粒体功能控制机制，这一成果将对认识和治疗遗传疾病和老年病有十分重要的意义。
线粒体通常被称为“细胞的发电机”，线粒体内部的核糖体蛋白能够将食物中的能量转化成新的蛋白质，从而被人体加以吸收利用。
研究人员在新一期国际学术期刊《细胞—代谢》 Cell Metabolism 上发表论文说，通过老鼠实验发现，一种名为MTERF4的蛋白质非常关键，它与另一种名为NSUN4的蛋白质组合成一个复合体后，能控制线粒体核糖体的形成与功能。而在缺少MTERF4蛋白质的情况下，线粒体内的核糖体将无法形成，也无从产生核糖体蛋白，这导致老鼠肌体的能量制造能力下降。
项目研究负责人拉尔松教授说，有多种遗传疾病和老年病与线粒体功能衰退相关，因此彻底了解线粒体发挥作用的机理对治疗这些疾病有十分重要的意义。（生物谷Bioon.com）
生物谷推荐原文出处：
Cell metabolism DOI： 10.1016/j.cmet.2011.04.002
MTERF4 Regulates Translation by Targeting the Methyltransferase NSUN4 to the Mammalian Mitochondrial Ribosome
Yolanda Camara, Jorge Asin-Cayuela, Chan Bae Park, Metodi D. Metodiev, Yonghong Shi, Benedetta Ruzzenente, Christian Kukat, Bianca Habermann, Rolf Wibom, Kjell Hultenby, Thomas Franz, Hediye Erdjument-Bromage, Paul Tempst, B. Martin Hallberg, Claes M. Gustafsson, and Nils-Goran Larsson
SummaryPrecise control of mitochondrial DNA gene expression is critical for regulation of oxidative phosphorylation capacity in mammals. The MTERF protein family plays a key role in this process, and its members have been implicated in regulation of transcription initiation and site-specific transcription termination. We now demonstrate that a member of this family, MTERF4, directly controls mitochondrial ribosomal biogenesis and translation. MTERF4 forms a stoichiometric complex with the ribosomal RNA methyltransferase NSUN4 and is necessary for recruitment of this factor to the large ribosomal subunit. Loss of MTERF4 leads to defective ribosomal assembly and a drastic reduction in translation. Our results thus show that MTERF4 is an important regulator of translation in mammalian mitochondria.
