生物
线粒体核糖体
内膜转移酶
线粒体
核糖体
细胞生物学
生物发生
内膜
线粒体内膜
酿酒酵母
线粒体载体
线粒体膜转运蛋白
生物化学
呼吸链
转运蛋白
膜蛋白
酵母
细菌外膜
核糖核酸
基因
膜
大肠杆菌
作者
Ann E. Frazier,Rebecca D. Taylor,David U. Mick,Bettina Warscheid,Nadine Stoepel,Helmut E. Meyer,Michael T. Ryan,Bernard Guiard,Peter Rehling
标识
DOI:10.1083/jcb.200505060
摘要
Saccharomyces cerevisiae Mdm38 and Ylh47 are homologues of human Letm1, a protein implicated in Wolf-Hirschhorn syndrome. We analyzed the function of Mdm38 and Ylh47 in yeast mitochondria to gain insight into the role of Letm1. We find that mdm38Delta mitochondria have reduced amounts of certain mitochondrially encoded proteins and low levels of complex III and IV and accumulate unassembled Atp6 of complex V of the respiratory chain. Mdm38 is especially required for efficient transport of Atp6 and cytochrome b across the inner membrane, whereas Ylh47 plays a minor role in this process. Both Mdm38 and Ylh47 form stable complexes with mitochondrial ribosomes, similar to what has been reported for Oxa1, a central component of the mitochondrial export machinery. Our results indicate that Mdm38 functions as a component of an Oxa1-independent insertion machinery in the inner membrane and that Mdm38 plays a critical role in the biogenesis of the respiratory chain by coupling ribosome function to protein transport across the inner membrane.
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