Journal
CELL REPORTS
Volume 12, Issue 10, Pages 1644-1655Publisher
CELL PRESS
DOI: 10.1016/j.celrep.2015.08.009
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Funding
- Deutsche Forschungsgemeinschaft
- Gottingen Graduate School for Neurosciences and Molecular Biosciences
- Excellence Initiative of the German Federal and State Governments Grant, BIOSS Centre for Biological Signalling Studies [EXC 294]
- European Research Council (AdG) [339580]
- Max Planck Society
- European Research Council (ERC) [339580] Funding Source: European Research Council (ERC)
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Cytochrome c oxidase, the terminal enzyme of the respiratory chain, is assembled from mitochondria- and nuclear-encoded subunits. The MITRAC complex represents the central assembly intermediate during this process as it receives imported subunits and regulates mitochondrial translation of COX1 mRNA. The molecular processes that promote and regulate the progression of assembly downstream of MITRAC are still unknown. Here, we identify MITRAC7 as a constituent of a late form of MITRAC and as a COX1-specific chaperone. MITRAC7 is required for cytochrome c oxidase biogenesis. Surprisingly, loss of MITRAC7 or an increase in its amount causes selective cytochrome c oxidase deficiency in human cells. We demonstrate that increased MITRAC7 levels stabilize and trap COX1 in MITRAC, blocking progression in the assembly process. In contrast, MITRAC7 deficiency leads to turnover of newly synthesized COX1. Accordingly, MITRAC7 affects the biogenesis pathway by stabilizing newly synthesized COX1 in assembly intermediates, concomitantly preventing turnover.
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