title: Mitochondrial ABC transporters.
abstract: In contrast to bacteria, mitochondria contain only a few ATP binding cassette (ABC) transporters in their inner membrane. The known mitochondrial ABC proteins fall into two major classes that, in the yeast Saccharomyces cerevisiae, are represented by the half-transporter Atm1p and the two closely homologous proteins Mdl1p and Mdl2p. In humans two Atm1p orthologues (ABC7 and MTABC3) and two proteins homologous to Mdll/2p have been localized to mitochondria. The Atm1p-like proteins perform an important function in mitochondrial iron homeostasis and in the maturation of Fe/S proteins in the cytosol. Mutations in ABC7 are causative of hereditary X-linked sideroblastic anemia and cerebellar ataxia (XLSA/A). MTABC3 may be a candidate gene for the lethal neonatal syndrome. The function of the mitochondrial Mdl1/2p-like proteins is not clear at present with the notable exception of murine ABC-me that may transport intermediates of heme biosynthesis from the matrix to the cytosol in erythroid tissues.
authors: Institut für Zytobiologie und Zytopathologie der Philipps-Universität Marburg, Germany. Lill@mailer.uni-marburg.de
authors: R
authors: R
authors: Lill
authors: G
authors: G
authors: Kispal
mesh_terms: D018528:ATP-Binding Cassette Transporters / Q000378:metabolism*; D000818:Animals; D053487:DEAD-box RNA Helicases; D005057:Eukaryotic Cells; D005656:Fungal Proteins / Q000378:metabolism; D006801:Humans; D007501:Iron / Q000378:metabolism; D008322:Mammals; D008928:Mitochondria / Q000378:metabolism*; D020365:RNA Helicases; D012441:Saccharomyces cerevisiae / Q000378:metabolism*; D029701:Saccharomyces cerevisiae Proteins / Q000235:genetics / Q000378:metabolism; D017384:Sequence Deletion
chemical_list: C107991:ABCB6 protein, human; C113037:ABCB7 protein, human; C091561:ATM1 protein, S cerevisiae; D018528:ATP-Binding Cassette Transporters; D005656:Fungal Proteins; C490069:MDL1 protein, S cerevisiae; C490070:MDL2 protein, S cerevisiae; D029701:Saccharomyces cerevisiae Proteins; C106609:YBT1 protein, S cerevisiae; D007501:Iron; C498428:DDX39A protein, human; D053487:DEAD-box RNA Helicases; D020365:RNA Helicases