CLPP coordinates mitoribosomal assembly through the regulation of ERAL1 levels

被引:119
作者
Szczepanowska, Karolina [1 ,2 ]
Maiti, Priyanka [1 ,2 ]
Kukat, Alexandra [1 ,2 ]
Hofsetz, Eduard [1 ,2 ]
Nolte, Hendrik [1 ,3 ]
Senft, Katharina [1 ,2 ]
Becker, Christina [1 ,2 ]
Ruzzenente, Benedetta [4 ]
Hornig-Do, Hue-Tran [1 ,5 ]
Wibom, Rolf [6 ]
Wiesner, Rudolf J. [1 ,5 ]
Krueger, Marcus [1 ,3 ]
Trifunovic, Aleksandra [1 ,2 ]
机构
[1] Cologne Excellence Cluster Cellular Stress Respon, Cologne, Germany
[2] Univ Cologne, Fac Med, Inst Mitochondrial Dis & Aging, Cologne, Germany
[3] Univ Cologne, Inst Genet, Cologne, Germany
[4] Max Planck Inst Biol Aging, Cologne, Germany
[5] Univ Cologne, Inst Vegetat Physiol, Cologne, Germany
[6] Karolinska Inst, Dept Lab Med, Stockholm, Sweden
基金
欧洲研究理事会;
关键词
CLPP; ERAL1; mitochondrial ribosome assembly; OXPHOS deficiency; MAMMALIAN MITOCHONDRIAL RIBOSOME; TRANSFER-RNA SYNTHETASE; PERRAULT SYNDROME; HEARING-LOSS; MTDNA TRANSCRIPTION; GROWTH-RETARDATION; PROTEASE; MUTATIONS; TRANSLATION; REVEALS;
D O I
10.15252/embj.201694253
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Despite being one of the most studied proteases in bacteria, very little is known about the role of ClpXP in mitochondria. We now present evidence that mammalian CLPP has an essential role in determining the rate of mitochondrial protein synthesis by regulating the level of mitoribosome assembly. Through a proteomic approach and the use of a catalytically inactive CLPP, we produced the first comprehensive list of possible mammalian ClpXP substrates involved in the regulation of mitochondrial translation, oxidative phosphorylation, and a number of metabolic pathways. We further show that the defect in mitoribosomal assembly is a consequence of the accumulation of ERAL1, a putative 12S rRNA chaperone, and novel ClpXP substrate. The presented data suggest that the timely removal of ERAL1 from the small ribosomal subunit is essential for the efficient maturation of the mitoribosome and a normal rate of mitochondrial translation.
引用
收藏
页码:2566 / 2583
页数:18
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