title: Structures and functions of mitochondrial ABC transporters.
abstract: A small number of physiologically important ATP-binding cassette (ABC) transporters are found in mitochondria. Most are half transporters of the B group forming homodimers and their topology suggests they function as exporters. The results of mutant studies point towards involvement in iron cofactor biosynthesis. In particular, ABC subfamily B member 7 (ABCB7) and its homologues in yeast and plants are required for iron-sulfur (Fe-S) cluster biosynthesis outside of the mitochondria, whereas ABCB10 is involved in haem biosynthesis. They also play a role in preventing oxidative stress. Mutations in ABCB6 and ABCB7 have been linked to human disease. Recent crystal structures of yeast Atm1 and human ABCB10 have been key to identifying substrate-binding sites and transport mechanisms. Combined with in vitro and in vivo studies, progress is being made to find the physiological substrates of the different mitochondrial ABC transporters.
authors.affiliation: Department of Biological Chemistry and Crop Protection, Rothamsted Research, West Common, Harpenden, AL5 2JQ, U.K.
authors.forename: Theresia A
authors.initials: TA
authors.lastname: Schaedler
authors.affiliation: Structural Genomics Consortium, Nuffield Department of Clinical Medicine, University of Oxford, Oxford, OX3 7DQ, U.K.
authors.forename: Belinda
authors.initials: B
authors.lastname: Faust
authors.affiliation: Structural Genomics Consortium, Nuffield Department of Clinical Medicine, University of Oxford, Oxford, OX3 7DQ, U.K.
authors.forename: Chitra A
authors.initials: CA
authors.lastname: Shintre
authors.affiliation: Structural Genomics Consortium, Nuffield Department of Clinical Medicine, University of Oxford, Oxford, OX3 7DQ, U.K.
authors.forename: Elisabeth P
authors.initials: EP
authors.lastname: Carpenter
authors.affiliation: LOEWE center for synthetic microbiology (SYNMIKRO) and Philipps University, D-35043 Marburg, Germany.
authors.forename: Vasundara
authors.initials: V
authors.lastname: Srinivasan
authors.affiliation: Department of Pharmacology, University of Cambridge, Tennis Court Road, Cambridge, CB2 1PD, U.K.
authors.forename: Hendrik W
authors.initials: HW
authors.lastname: van Veen
authors.affiliation: John Innes Centre and University of East Anglia, Colney Lane, Norwich, NR4 7UH, U.K. janneke.balk@jic.ac.uk.
authors.forename: Janneke
authors.initials: J
authors.lastname: Balk
mesh_terms: D018528:ATP-Binding Cassette Transporters / Q000145:classification / Q000235:genetics / Q000378:metabolism*; D000818:Animals; D018360:Crystallography, X-Ray; D006801:Humans; D008928:Mitochondria / Q000378:metabolism*; D024101:Mitochondrial Proteins / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D009154:Mutation; D010802:Phylogeny; D010940:Plant Proteins / Q000737:chemistry / Q000235:genetics / Q000378:metabolism; D012441:Saccharomyces cerevisiae / Q000737:chemistry / Q000235:genetics / Q000378:metabolism
chemical_list: D018528:ATP-Binding Cassette Transporters; D024101:Mitochondrial Proteins; D010940:Plant Proteins
keywords: ATP-binding cassette (ABC) transporter; glutathione; haem; iron; mitochondria; oxidative stress