Journal
JOURNAL OF BIOLOGICAL CHEMISTRY
Volume 287, Issue 7, Pages 4426-4433Publisher
ELSEVIER
DOI: 10.1074/jbc.R111.270678
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Funding
- Deutsche Forschungsgemeinschaft
- Landesschwerpunkt Rheinland-Pfalz on Membrane Transport
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99% of all mitochondrial proteins are synthesized in the cytosol, from where they are imported into mitochondria. In contrast to matrix proteins, many proteins of the intermembrane space (IMS) lack presequences and are imported in an oxidation-driven reaction by the mitochondrial disulfide relay. Incoming polypeptides are recognized and oxidized by the IMS-located receptor Mia40. Reoxidation of Mia40 is facilitated by the sulfhydryl oxidase Erv1 and the respiratory chain. Although structurally unrelated, the mitochondrial disulfide relay functionally resembles the Dsb (disufide bond) system of the bacterial periplasm, the compartment from which the IMS was derived 2 billion years ago.
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