Szczepanowska, Karolina ORCID: 0000-0003-4689-2350, Maiti, Priyanka ORCID: 0000-0003-4207-0341, Kukat, Alexandra, Hofsetz, Eduard ORCID: 0000-0001-5830-4566, Nolte, Hendrik, Senft, Katharina, Becker, Christina, Ruzzenente, Benedetta ORCID: 0000-0001-7366-114X, Hornig-Do, Hue-Tran, Wibom, Rolf, Wiesner, Rudolf J., Krueger, Marcus and Trifunovic, Aleksandra (2016). CLPP coordinates mitoribosomal assembly through the regulation of ERAL1 levels. Embo J., 35 (23). S. 2566 - 2584. HOBOKEN: WILEY-BLACKWELL. ISSN 1460-2075

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Abstract

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.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Szczepanowska, KarolinaUNSPECIFIEDorcid.org/0000-0003-4689-2350UNSPECIFIED
Maiti, PriyankaUNSPECIFIEDorcid.org/0000-0003-4207-0341UNSPECIFIED
Kukat, AlexandraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hofsetz, EduardUNSPECIFIEDorcid.org/0000-0001-5830-4566UNSPECIFIED
Nolte, HendrikUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Senft, KatharinaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Becker, ChristinaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ruzzenente, BenedettaUNSPECIFIEDorcid.org/0000-0001-7366-114XUNSPECIFIED
Hornig-Do, Hue-TranUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wibom, RolfUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wiesner, Rudolf J.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Krueger, MarcusUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Trifunovic, AleksandraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-254722
DOI: 10.15252/embj.201694253
Journal or Publication Title: Embo J.
Volume: 35
Number: 23
Page Range: S. 2566 - 2584
Date: 2016
Publisher: WILEY-BLACKWELL
Place of Publication: HOBOKEN
ISSN: 1460-2075
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
MAMMALIAN MITOCHONDRIAL RIBOSOME; TRANSFER-RNA SYNTHETASE; PERRAULT SYNDROME; HEARING-LOSS; MTDNA TRANSCRIPTION; GROWTH-RETARDATION; PROTEASE; MUTATIONS; TRANSLATION; REVEALSMultiple languages
Biochemistry & Molecular Biology; Cell BiologyMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/25472

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