瑞典卡羅林斯卡醫(yī)學院的研究人員日前宣布，他們發(fā)現(xiàn)了一種線粒體功能控制機制,，這一成果將對認識和治療遺傳疾病和老年病有十分重要的意義,。
線粒體通常被稱為“細胞的發(fā)電機”,，線粒體內部的核糖體蛋白能夠將食物中的能量轉化成新的蛋白質，從而被人體加以吸收利用,。
研究人員在新一期國際學術期刊《細胞—代謝》 Cell Metabolism 上發(fā)表論文說,，通過老鼠實驗發(fā)現(xiàn)，一種名為MTERF4的蛋白質非常關鍵,，它與另一種名為NSUN4的蛋白質組合成一個復合體后,，能控制線粒體核糖體的形成與功能。而在缺少MTERF4蛋白質的情況下,，線粒體內的核糖體將無法形成,，也無從產生核糖體蛋白，這導致老鼠肌體的能量制造能力下降,。
項目研究負責人拉爾松教授說,，有多種遺傳疾病和老年病與線粒體功能衰退相關，因此徹底了解線粒體發(fā)揮作用的機理對治療這些疾病有十分重要的意義,。（生物谷Bioon.com）
生物谷推薦原文出處：
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.
