title: Oxidative folding in the mitochondrial intermembrane space: A regulated process important for cell physiology and disease.
abstract: Mitochondria are fundamental organelles with a complex internal architecture that fulfill important diverse functions including iron-sulfur cluster assembly and cell respiration. Intense work for more than 30 years has identified the key protein import components and the pathways involved in protein targeting and assembly. More recently, oxidative folding has been discovered as one important mechanism for mitochondrial proteostasis whilst several human disorders have been linked to this pathway. We describe the molecular components of this pathway in view of their putative redox regulation and we summarize available evidence on the connections of these pathways to human disorders.
authors.affiliation: Institute of Molecular Cell and Systems Biology, College of Medical, Veterinary and Life Sciences, Davidson Building, University of Glasgow, Glasgow, G12 8QQ, UK.
authors.forename: Afroditi
authors.initials: A
authors.lastname: Chatzi
authors.affiliation: Institute of Molecular Cell and Systems Biology, College of Medical, Veterinary and Life Sciences, Davidson Building, University of Glasgow, Glasgow, G12 8QQ, 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, Davidson Building, University of Glasgow, Glasgow, G12 8QQ, UK; Department of Materials Science and Technology, University of Crete, Heraklion, Crete, Greece; Institute of Molecular Biology and Biotechnology, Foundation for Research and Technology, Heraklion, Crete, Greece. Electronic address: Kostas.Tokatlidis@glasgow.ac.uk.
authors.forename: Kostas
authors.initials: K
authors.lastname: Tokatlidis
mesh_terms: D001692:Biological Transport / Q000502:physiology; D002468:Cell Physiological Phenomena; D006801:Humans; D008928:Mitochondria / Q000378:metabolism / Q000502:physiology*; D028361:Mitochondrial Diseases / Q000378:metabolism / Q000503:physiopathology; D051336:Mitochondrial Membranes / Q000378:metabolism / Q000502:physiology*; D024101:Mitochondrial Proteins / Q000737:chemistry / Q000378:metabolism / Q000502:physiology*; D008954:Models, Biological; D010084:Oxidation-Reduction; D017510:Protein Folding
chemical_list: D024101:Mitochondrial Proteins
keywords: Erv1; Mia40; Mitochondrial intermembrane space; Mitochondrial protein import; Oxidative protein folding; Thiol-disulfide exchange