Busch, Jakob D., Cipullo, Miriam, Atanassov, Ilian, Bratic, Ana, Ramos, Eduardo Silva, Schoendorf, Thomas, Li, Xinping, Pearce, Sarah F., Milenkovic, Dusanka, Rorbach, Joanna ORCID: 0000-0002-2891-2840 and Larsson, Nils-Goeran (2019). MitoRibo-Tag Mice Provide a Tool for In Vivo Studies of Mitoribosome Composition. Cell Reports, 29 (6). S. 1728 - 1748. CAMBRIDGE: CELL PRESS. ISSN 2211-1247

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Abstract

Mitochondria harbor specialized ribosomes (mitoribosomes) necessary for the synthesis of key membrane proteins of the oxidative phosphorylation (OXPHOS) machinery located in the mitochondrial inner membrane. To date, no animal model exists to study mitoribosome composition and mitochondrial translation coordination in mammals in vivo. Here, we create MitoRibo-Tag mice as a tool enabling affinity purification and proteomics analyses of mitoribosomes and their interactome in different tissues. We also define the composition of an assembly intermediate formed in the absence of MTERF4, necessary for a late step in mitoribosomal biogenesis. We identify the orphan protein PUSL1, which interacts with a large subunit assembly intermediate, and demonstrate that it is an inner-membrane-associated mitochondrial matrix protein required for efficient mitochondrial translation. This work establishes MitoRibo-Tag mice as a powerful tool to study mitoribosomes in vivo, enabling future studies on the mitoribosome interactome under different physiological states, as well as in disease and aging.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Busch, Jakob D.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Cipullo, MiriamUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Atanassov, IlianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bratic, AnaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ramos, Eduardo SilvaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schoendorf, ThomasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Li, XinpingUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Pearce, Sarah F.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Milenkovic, DusankaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Rorbach, JoannaUNSPECIFIEDorcid.org/0000-0002-2891-2840UNSPECIFIED
Larsson, Nils-GoeranUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-128173
DOI: 10.1016/j.celrep.2019.09.080
Journal or Publication Title: Cell Reports
Volume: 29
Number: 6
Page Range: S. 1728 - 1748
Date: 2019
Publisher: CELL PRESS
Place of Publication: CAMBRIDGE
ISSN: 2211-1247
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
MAMMALIAN MITOCHONDRIAL RIBOSOME; RAT-LIVER MITOCHONDRIA; LARGE SUBUNIT; PROTEIN-COMPOSITION; TRANSFER-RNAS; COMPLEX; TRANSLATION; BIOGENESIS; IDENTIFICATION; ORGANIZATIONMultiple languages
Cell BiologyMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/12817

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