title: Mitochondrial ABC proteins in health and disease.
abstract: ABC transporters represent one of the largest families of membrane proteins that are found in all three phyla of life. Mitochondria comprise up to four ABC systems, ABCB7/ATM1, ABCB10/MDL1, ABCB8 and ABCB6. These half-transporters, which assemble into homodimeric complexes, are involved in a number of key cellular processes, e.g. biogenesis of cytosolic iron-sulfur clusters, heme biosynthesis, iron homeostasis, multidrug resistance, and protection against oxidative stress. Here, we summarize recent advances and emerging themes in our understanding of how these ABC systems in the inner and outer mitochondrial membrane fulfill their functions in important (patho) physiological processes, including neurodegenerative and hematological disorders.
authors.affiliation: Institute of Biochemistry, Biocenter, Goethe-University, Max-von-Laue-Str. 9, D-60348 Frankfurt a.M., Germany.
authors.forename: Ariane
authors.initials: A
authors.lastname: Zutz
authors.forename: Simone
authors.initials: S
authors.lastname: Gompf
authors.forename: Hermann
authors.initials: H
authors.lastname: Schägger
authors.forename: Robert
authors.initials: R
authors.lastname: Tampé
mesh_terms: D018528:ATP-Binding Cassette Transporters / Q000737:chemistry* / Q000235:genetics / Q000378:metabolism*; D000255:Adenosine Triphosphate / Q000378:metabolism; D000818:Animals; D006418:Heme / Q000096:biosynthesis; D006706:Homeostasis; D006801:Humans; D006868:Hydrolysis; D007501:Iron / Q000378:metabolism; D024101:Mitochondrial Proteins / Q000737:chemistry* / Q000235:genetics / Q000378:metabolism*; D008958:Models, Molecular; D010802:Phylogeny; D011499:Protein Processing, Post-Translational
chemical_list: D018528:ATP-Binding Cassette Transporters; D024101:Mitochondrial Proteins; D006418:Heme; D000255:Adenosine Triphosphate; D007501:Iron