title: Mitochondrial disulfide relay: redox-regulated protein import into the intermembrane space.
abstract: 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.
authors.affiliation: Department of Cell Biology, University of Kaiserslautern, Erwin-Schrödinger-Strasse 13, 67663 Kaiserslautern, Germany. hannes.herrmann@biologie.uni-kl.de
authors.forename: Johannes M
authors.initials: JM
authors.lastname: Herrmann
authors.forename: Jan
authors.initials: J
authors.lastname: Riemer
mesh_terms: D000818:Animals; D001419:Bacteria / Q000378:metabolism*; D001426:Bacterial Proteins / Q000378:metabolism*; D003579:Cytochrome Reductases / Q000378:metabolism*; D004220:Disulfides / Q000378:metabolism*; D004579:Electron Transport / Q000502:physiology; D019143:Evolution, Molecular; D006801:Humans; D008928:Mitochondria / Q000378:metabolism*; D033681:Mitochondrial Membrane Transport Proteins / Q000378:metabolism*; D000090622:Mitochondrial Precursor Protein Import Complex Proteins; D010084:Oxidation-Reduction; D050862:Oxidoreductases Acting on Sulfur Group Donors; D019897:Periplasm / Q000378:metabolism*; D021381:Protein Transport / Q000502:physiology
chemical_list: D001426:Bacterial Proteins; C507282:CHCHD4 protein, human; D004220:Disulfides; D033681:Mitochondrial Membrane Transport Proteins; D000090622:Mitochondrial Precursor Protein Import Complex Proteins; D003579:Cytochrome Reductases; C500897:GFER protein, human; D050862:Oxidoreductases Acting on Sulfur Group Donors