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MITRAC15/COA1 promotes mitochondrial translation in a ND2 ribosome-nascent chain complex.


ABSTRACT: The mitochondrial genome encodes for thirteen core subunits of the oxidative phosphorylation system. These proteins assemble with imported proteins in a modular manner into stoichiometric enzyme complexes. Assembly factors assist in these biogenesis processes by providing co-factors or stabilizing transient assembly stages. However, how expression of the mitochondrial-encoded subunits is regulated to match the availability of nuclear-encoded subunits is still unresolved. Here, we address the function of MITRAC15/COA1, a protein that participates in complex I biogenesis and complex IV biogenesis. Our analyses of a MITRAC15 knockout mutant reveal that MITRAC15 is required for translation of the mitochondrial-encoded complex I subunit ND2. We find that MITRAC15 is a constituent of a ribosome-nascent chain complex during ND2 translation. Chemical crosslinking analyses demonstrate that binding of the ND2-specific assembly factor ACAD9 to the ND2 polypeptide occurs at the C-terminus and thus downstream of MITRAC15. Our analyses demonstrate that expression of the founder subunit ND2 of complex I undergoes regulation. Moreover, a ribosome-nascent chain complex with MITRAC15 is at the heart of this process.

SUBMITTER: Wang C 

PROVIDER: S-EPMC6945058 | biostudies-literature | 2020 Jan

REPOSITORIES: biostudies-literature

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MITRAC15/COA1 promotes mitochondrial translation in a ND2 ribosome-nascent chain complex.

Wang Cong C   Richter-Dennerlein Ricarda R   Pacheu-Grau David D   Liu Fan F   Zhu Ying Y   Dennerlein Sven S   Rehling Peter P  

EMBO reports 20191113 1


The mitochondrial genome encodes for thirteen core subunits of the oxidative phosphorylation system. These proteins assemble with imported proteins in a modular manner into stoichiometric enzyme complexes. Assembly factors assist in these biogenesis processes by providing co-factors or stabilizing transient assembly stages. However, how expression of the mitochondrial-encoded subunits is regulated to match the availability of nuclear-encoded subunits is still unresolved. Here, we address the fun  ...[more]

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