title: Oxidative protein biogenesis and redox regulation in the mitochondrial intermembrane space.
abstract: Mitochondria are organelles that play a central role in cellular metabolism, as they are responsible for processes such as iron/sulfur cluster biogenesis, respiration and apoptosis. Here, we describe briefly the various protein import pathways for sorting of mitochondrial proteins into the different subcompartments, with an emphasis on the targeting to the intermembrane space. The discovery of a dedicated redox-controlled pathway in the intermembrane space that links protein import to oxidative protein folding raises important questions on the redox regulation of this process. We discuss the salient features of redox regulation in the intermembrane space and how such mechanisms may be linked to the more general redox homeostasis balance that is crucial not only for normal cell physiology but also for cellular dysfunction.
authors.affiliation: Institute of Molecular, Cell and Systems Biology, College of Medical, Veterinary and Life Sciences, University of Glasgow, Glasgow, UK.
authors.forename: Phanee
authors.initials: P
authors.lastname: Manganas
authors.affiliation: Institute of Molecular, Cell and Systems Biology, College of Medical, Veterinary and Life Sciences, University of Glasgow, Glasgow, UK.
authors.forename: Lisa
authors.initials: L
authors.lastname: MacPherson
authors.affiliation: Institute of Molecular, Cell and Systems Biology, College of Medical, Veterinary and Life Sciences, University of Glasgow, Glasgow, UK. Kostas.Tokatlidis@glasgow.ac.uk.
authors.forename: Kostas
authors.identifier: http://orcid.org/0000-0001-6295-8183
authors.initials: K
authors.lastname: Tokatlidis
mesh_terms: D004220:Disulfides / Q000378:metabolism; D006801:Humans; D051336:Mitochondrial Membranes / Q000378:metabolism*; D024101:Mitochondrial Proteins / Q000378:metabolism*; D010084:Oxidation-Reduction; D014176:Protein Biosynthesis; D021381:Protein Transport
chemical_list: D004220:Disulfides; D024101:Mitochondrial Proteins
keywords: Intermembrane space; Mitochondria; Oxidative folding; Protein import; Redox regulation