title: The peroxisomal ABC transporter family.
abstract: This review describes the current state of knowledge about the ABCD family of peroxisomal half adenosine-triphosphate-binding cassette (ABC) transporters. ABCDs are predicted to be present in a variety of eukaryotic organisms, although at present, only ABCDs in the yeast Saccharomyces cerevisiae, the plant Arabidopsis thaliana, and different mammalian species have been identified and characterized to any significant extent. The functional role of none of these ABCDs has been established definitively and awaits successful reconstitution of ABCDs, either as homo- or heterodimers into liposomes, followed by transport studies. Data obtained in S. cerevisiae suggest that the two ABCDs, which have been identified in this organism, form a heterodimer, which actually transports acyl coenzyme A esters across the peroxisomal membrane. In mammals, four ABCDs have been identified, of which one [adrenoleukodystrophy protein (ALDP)] has been implicated in the transport of the coenzyme A esters of very-long-chain fatty acids. Mutations in the gene (ABCD1) encoding ALDP are the cause of a severe X-linked disease, called X-linked adrenoleukodystrophy. The availability of mutant mice in which Abcd1, Abcd2, or Abcd3 have been disrupted will help to resolve the true role of the peroxisomal half-ABC transporters.
authors.affiliation: Department of Clinical Chemistry and Pediatrics, Emma Children's Hospital, Laboratory Genetic Metabolic Diseases, University of Amsterdam, Academic Medical Center, Amsterdam, The Netherlands. r.j.wanders@amc.uva.nl
authors.forename: Ronald J A
authors.initials: RJ
authors.lastname: Wanders
authors.forename: Wouter F
authors.initials: WF
authors.lastname: Visser
authors.forename: Carlo W T
authors.initials: CW
authors.lastname: van Roermund
authors.forename: Stephan
authors.initials: S
authors.lastname: Kemp
authors.forename: Hans R
authors.initials: HR
authors.lastname: Waterham
mesh_terms: D000074063:ATP Binding Cassette Transporter, Subfamily D, Member 1; D018528:ATP-Binding Cassette Transporters / Q000502:physiology*; D000251:Adenosine Triphosphatases; D000326:Adrenoleukodystrophy / Q000235:genetics / Q000628:therapy; D000818:Animals; D029681:Arabidopsis Proteins / Q000502:physiology; D004195:Disease Models, Animal; D005227:Fatty Acids / Q000378:metabolism; D005786:Gene Expression Regulation; D006801:Humans; D051379:Mice; D020675:Peroxisomes / Q000502:physiology*; D010802:Phylogeny; D012333:RNA, Messenger / Q000378:metabolism; D012441:Saccharomyces cerevisiae / Q000378:metabolism; D029701:Saccharomyces cerevisiae Proteins / Q000378:metabolism / Q000502:physiology
chemical_list: C115572:ABCD1 protein, human; C063305:ABCD3 protein, human; C489273:ABCD4 protein, human; D000074063:ATP Binding Cassette Transporter, Subfamily D, Member 1; D018528:ATP-Binding Cassette Transporters; D029681:Arabidopsis Proteins; D005227:Fatty Acids; C098338:PXA1 protein, S cerevisiae; C103561:PXA2 protein, S cerevisiae; D012333:RNA, Messenger; D029701:Saccharomyces cerevisiae Proteins; D000251:Adenosine Triphosphatases; C456843:At4g39850 protein, Arabidopsis