ABC-family proteins mediated transport (Homo sapiens)

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12, 18, 438, 14-17, 21...4, 29, 303, 34, 46328, 14-17, 21...25, 2811, 19, 23, 40-42, 476, 713, 26, 31, 37, 489, 10, 20, 22, 36...51, 2, 27, 31, 4424, 35, 45mitochondrial matrixperoxisomal matrixABCD1ABCD1 ABCD1ABCD3 cytosolMTABC3 dimer ABCA7-1ApoA1 complex ABCD2ABCD3 ABCG5ABCG8 heterodimer PEX19ABCD1/2/3 transport vesicleABCG4 dimer ABCG1 dimer ABCD1/2/3 dimers ABCD1ABCD2 PiCFTRABCD1/2/3 dimersPiATPPEX19ABCD1/2/3H2OCHOLH2OABCA cholesterol transportersADPsterolsADPH2OABCG4 ABCD3ATPATPADPABCA7-1ApoA1 complexABCG8 PiCHOLABCG4 dimerhemeABCD1 ADPABCA4ATPATPH2OABCCsPEX3ABCG1 dimerPiATPH2OABCA7-dependent phospholipidsABCD2 PiABCG5ABCG8 heterodimerABCA7-dependent phospholipidshemePEX19organic anionporphyrinADPatRALH2OporphyrinH2OABCA8/B1/B5PiPiABCG5 HCO3-cholesterolADPHCO3-PEX19 H2OcholesterolLCFAPiATPABCG1 ATPPiADPCHOLABCA7 organic anionCHOLa xenobioticatRALH2OH2OMTABC3 dimerHeme transporterssterolsPiADPADPATPATPLFCAAPOA1ADPABCB6 a xenobiotic


The ATP-binding cassette (ABC) superfamily of active transporters involves a large number of functionally diverse transmembrane proteins. They transport a variety of compounds through membranes against steep concentration gradients at the cost of ATP hydrolysis. These substrates include amino acids, lipids, inorganic ions, peptides, saccharides, peptides for antigen presentation, metals, drugs, and proteins. The ABC transporters not only move a variety of substrates into and out of the cell, but are also involved in intracellular compartmental transport. Energy derived from the hydrolysis of ATP is used to transport the substrate across the membrane against a concentration gradient. Human genome contains 48 ABC genes; 16 of these have a known function and 14 are associated with a defined human disease (Dean et al. 2001, Borst and Elferink 2002, Rees et al. 2009). Original Pathway at Reactome:

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  1. Park M, Ko SB, Choi JY, Muallem G, Thomas PJ, Pushkin A, Lee MS, Kim JY, Lee MG, Muallem S, Kurtz I.; ''The cystic fibrosis transmembrane conductance regulator interacts with and regulates the activity of the HCO3- salvage transporter human Na+-HCO3- cotransport isoform 3.''; PubMed Europe PMC Scholia
  2. Lukacs GL, Mohamed A, Kartner N, Chang XB, Riordan JR, Grinstein S.; ''Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP.''; PubMed Europe PMC Scholia
  3. Hogue DL, Liu L, Ling V.; ''Identification and characterization of a mammalian mitochondrial ATP-binding cassette membrane protein.''; PubMed Europe PMC Scholia
  4. Ward CL, Omura S, Kopito RR.; ''Degradation of CFTR by the ubiquitin-proteasome pathway.''; PubMed Europe PMC Scholia
  5. DeStefano GM, Kurban M, Anyane-Yeboa K, Dall'Armi C, Di Paolo G, Feenstra H, Silverberg N, Rohena L, López-Cepeda LD, Jobanputra V, Fantauzzo KA, Kiuru M, Tadin-Strapps M, Sobrino A, Vitebsky A, Warburton D, Levy B, Salas-Alanis JC, Christiano AM.; ''Mutations in the cholesterol transporter gene ABCA5 are associated with excessive hair overgrowth.''; PubMed Europe PMC Scholia
  6. Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, Dřevínek P, Griese M, McKone EF, Wainwright CE, Konstan MW, Moss R, Ratjen F, Sermet-Gaudelus I, Rowe SM, Dong Q, Rodriguez S, Yen K, Ordoñez C, Elborn JS, VX08-770-102 Study Group.; ''A CFTR potentiator in patients with cystic fibrosis and the G551D mutation.''; PubMed Europe PMC Scholia
  7. Riordan JR, Rommens JM, Kerem B, Alon N, Rozmahel R, Grzelczak Z, Zielenski J, Lok S, Plavsic N, Chou JL.; ''Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA.''; PubMed Europe PMC Scholia
  8. Soleimani M, Burnham CE.; ''Na+:HCO(3-) cotransporters (NBC): cloning and characterization.''; PubMed Europe PMC Scholia
  9. Frank NY, Margaryan A, Huang Y, Schatton T, Waaga-Gasser AM, Gasser M, Sayegh MH, Sadee W, Frank MH.; ''ABCB5-mediated doxorubicin transport and chemoresistance in human malignant melanoma.''; PubMed Europe PMC Scholia
  10. Wei SJ, Williams JG, Dang H, Darden TA, Betz BL, Humble MM, Chang FM, Trempus CS, Johnson K, Cannon RE, Tennant RW.; ''Identification of a specific motif of the DSS1 protein required for proteasome interaction and p53 protein degradation.''; PubMed Europe PMC Scholia
  11. Pranke IM, Sermet-Gaudelus I.; ''Biosynthesis of cystic fibrosis transmembrane conductance regulator.''; PubMed Europe PMC Scholia
  12. Krishnamurthy PC, Du G, Fukuda Y, Sun D, Sampath J, Mercer KE, Wang J, Sosa-Pineda B, Murti KG, Schuetz JD.; ''Identification of a mammalian mitochondrial porphyrin transporter.''; PubMed Europe PMC Scholia
  13. Borst P, Elferink RO.; ''Mammalian ABC transporters in health and disease.''; PubMed Europe PMC Scholia
  14. Wang N, Lan D, Gerbod-Giannone M, Linsel-Nitschke P, Jehle AW, Chen W, Martinez LO, Tall AR.; ''ATP-binding cassette transporter A7 (ABCA7) binds apolipoprotein A-I and mediates cellular phospholipid but not cholesterol efflux.''; PubMed Europe PMC Scholia
  15. El Khouri E, Le Pavec G, Toledano MB, Delaunay-Moisan A.; ''RNF185 is a novel E3 ligase of endoplasmic reticulum-associated degradation (ERAD) that targets cystic fibrosis transmembrane conductance regulator (CFTR).''; PubMed Europe PMC Scholia
  16. Picciotto MR, Cohn JA, Bertuzzi G, Greengard P, Nairn AC.; ''Phosphorylation of the cystic fibrosis transmembrane conductance regulator.''; PubMed Europe PMC Scholia
  17. Kaminski WE, Piehler A, Püllmann K, Porsch-Ozcürümez M, Duong C, Bared GM, Büchler C, Schmitz G.; ''Complete coding sequence, promoter region, and genomic structure of the human ABCA2 gene and evidence for sterol-dependent regulation in macrophages.''; PubMed Europe PMC Scholia
  18. Shen DW, Fojo A, Chin JE, Roninson IB, Richert N, Pastan I, Gottesman MM.; ''Human multidrug-resistant cell lines: increased mdr1 expression can precede gene amplification.''; PubMed Europe PMC Scholia
  19. Morita SY, Terada T.; ''Molecular mechanisms for biliary phospholipid and drug efflux mediated by ABCB4 and bile salts.''; PubMed Europe PMC Scholia
  20. Ramjeesingh M, Ugwu F, Li C, Dhani S, Huan LJ, Wang Y, Bear CE.; ''Stable dimeric assembly of the second membrane-spanning domain of CFTR (cystic fibrosis transmembrane conductance regulator) reconstitutes a chloride-selective pore.''; PubMed Europe PMC Scholia
  21. Wenzel JJ, Kaminski WE, Piehler A, Heimerl S, Langmann T, Schmitz G.; ''ABCA10, a novel cholesterol-regulated ABCA6-like ABC transporter.''; PubMed Europe PMC Scholia
  22. Small DM.; ''Role of ABC transporters in secretion of cholesterol from liver into bile.''; PubMed Europe PMC Scholia
  23. Ledford H.; ''Drug bests cystic-fibrosis mutation.''; PubMed Europe PMC Scholia
  24. Gloeckner CJ, Mayerhofer PU, Landgraf P, Muntau AC, Holzinger A, Gerber JK, Kammerer S, Adamski J, Roscher AA.; ''Human adrenoleukodystrophy protein and related peroxisomal ABC transporters interact with the peroxisomal assembly protein PEX19p.''; PubMed Europe PMC Scholia
  25. Kaminski WE, Orsó E, Diederich W, Klucken J, Drobnik W, Schmitz G.; ''Identification of a novel human sterol-sensitive ATP-binding cassette transporter (ABCA7).''; PubMed Europe PMC Scholia
  26. Oldfield S, Lowry C, Ruddick J, Lightman S.; ''ABCG4: a novel human white family ABC-transporter expressed in the brain and eye.''; PubMed Europe PMC Scholia
  27. Nasonkin I, Illing M, Koehler MR, Schmid M, Molday RS, Weber BH.; ''Mapping of the rod photoreceptor ABC transporter (ABCR) to 1p21-p22.1 and identification of novel mutations in Stargardt's disease.''; PubMed Europe PMC Scholia
  28. Sun H, Molday RS, Nathans J.; ''Retinal stimulates ATP hydrolysis by purified and reconstituted ABCR, the photoreceptor-specific ATP-binding cassette transporter responsible for Stargardt disease.''; PubMed Europe PMC Scholia
  29. Zhao C, Haase W, Tampé R, Abele R.; ''Peptide specificity and lipid activation of the lysosomal transport complex ABCB9 (TAPL).''; PubMed Europe PMC Scholia
  30. Aubourg P, Mosser J, Douar AM, Sarde CO, Lopez J, Mandel JL.; ''Adrenoleukodystrophy gene: unexpected homology to a protein involved in peroxisome biogenesis.''; PubMed Europe PMC Scholia
  31. Abe-Dohmae S, Ikeda Y, Matsuo M, Hayashi M, Okuhira K, Ueda K, Yokoyama S.; ''Human ABCA7 supports apolipoprotein-mediated release of cellular cholesterol and phospholipid to generate high density lipoprotein.''; PubMed Europe PMC Scholia
  32. Yamano G, Funahashi H, Kawanami O, Zhao LX, Ban N, Uchida Y, Morohoshi T, Ogawa J, Shioda S, Inagaki N.; ''ABCA3 is a lamellar body membrane protein in human lung alveolar type II cells.''; PubMed Europe PMC Scholia
  33. Csere P, Lill R, Kispal G.; ''Identification of a human mitochondrial ABC transporter, the functional orthologue of yeast Atm1p.''; PubMed Europe PMC Scholia
  34. Dean M, Rzhetsky A, Allikmets R.; ''The human ATP-binding cassette (ABC) transporter superfamily.''; PubMed Europe PMC Scholia
  35. Petry F, Kotthaus A, Hirsch-Ernst KI.; ''Cloning of human and rat ABCA5/Abca5 and detection of a human splice variant.''; PubMed Europe PMC Scholia
  36. Sacksteder KA, Jones JM, South ST, Li X, Liu Y, Gould SJ.; ''PEX19 binds multiple peroxisomal membrane proteins, is predominantly cytoplasmic, and is required for peroxisome membrane synthesis.''; PubMed Europe PMC Scholia
  37. Osborne L, Knight R, Santis G, Hodson M.; ''A mutation in the second nucleotide binding fold of the cystic fibrosis gene.''; PubMed Europe PMC Scholia
  38. Kamijo K, Osumi T, Hashimoto T.; ''[PMP70, the 70-kDa peroxisomal membrane protein: a member of the ATP-binding cassette transporters].''; PubMed Europe PMC Scholia
  39. Holzinger A, Kammerer S, Berger J, Roscher AA.; ''cDNA cloning and mRNA expression of the human adrenoleukodystrophy related protein (ALDRP), a peroxisomal ABC transporter.''; PubMed Europe PMC Scholia
  40. Paytubi S, Wang X, Lam YW, Izquierdo L, Hunter MJ, Jan E, Hundal HS, Proud CG.; ''ABC50 promotes translation initiation in mammalian cells.''; PubMed Europe PMC Scholia
  41. Biswas EE, Biswas SB.; ''The C-terminal nucleotide binding domain of the human retinal ABCR protein is an adenosine triphosphatase.''; PubMed Europe PMC Scholia
  42. Vembar SS, Brodsky JL.; ''One step at a time: endoplasmic reticulum-associated degradation.''; PubMed Europe PMC Scholia
  43. Akiyama M, Sugiyama-Nakagiri Y, Sakai K, McMillan JR, Goto M, Arita K, Tsuji-Abe Y, Tabata N, Matsuoka K, Sasaki R, Sawamura D, Shimizu H.; ''Mutations in lipid transporter ABCA12 in harlequin ichthyosis and functional recovery by corrective gene transfer.''; PubMed Europe PMC Scholia
  44. Babenko AP, Gonzalez G, Aguilar-Bryan L, Bryan J.; ''Reconstituted human cardiac KATP channels: functional identity with the native channels from the sarcolemma of human ventricular cells.''; PubMed Europe PMC Scholia
  45. Fang Y, Morrell JC, Jones JM, Gould SJ.; ''PEX3 functions as a PEX19 docking factor in the import of class I peroxisomal membrane proteins.''; PubMed Europe PMC Scholia
  46. Wolters JC, Abele R, Tampé R.; ''Selective and ATP-dependent translocation of peptides by the homodimeric ATP binding cassette transporter TAP-like (ABCB9).''; PubMed Europe PMC Scholia
  47. Chloupková M, Reaves SK, LeBard LM, Koeller DM.; ''The mitochondrial ABC transporter Atm1p functions as a homodimer.''; PubMed Europe PMC Scholia
  48. Morita SY, Kobayashi A, Takanezawa Y, Kioka N, Handa T, Arai H, Matsuo M, Ueda K.; ''Bile salt-dependent efflux of cellular phospholipids mediated by ATP binding cassette protein B4.''; PubMed Europe PMC Scholia
  49. Piehler A, Kaminski WE, Wenzel JJ, Langmann T, Schmitz G.; ''Molecular structure of a novel cholesterol-responsive A subclass ABC transporter, ABCA9.''; PubMed Europe PMC Scholia
  50. Kapoor H, Koolwal A, Singh A.; ''Ivacaftor: a novel mutation modulating drug.''; PubMed Europe PMC Scholia
  51. Klugbauer N, Hofmann F.; ''Primary structure of a novel ABC transporter with a chromosomal localization on the band encoding the multidrug resistance-associated protein.''; PubMed Europe PMC Scholia
  52. Voges D, Zwickl P, Baumeister W.; ''The 26S proteasome: a molecular machine designed for controlled proteolysis.''; PubMed Europe PMC Scholia
  53. Rees DC, Johnson E, Lewinson O.; ''ABC transporters: the power to change.''; PubMed Europe PMC Scholia
  54. Tsuruoka S, Ishibashi K, Yamamoto H, Wakaumi M, Suzuki M, Schwartz GJ, Imai M, Fujimura A.; ''Functional analysis of ABCA8, a new drug transporter.''; PubMed Europe PMC Scholia
  55. Allikmets R, Raskind WH, Hutchinson A, Schueck ND, Dean M, Koeller DM.; ''Mutation of a putative mitochondrial iron transporter gene (ABC7) in X-linked sideroblastic anemia and ataxia (XLSA/A).''; PubMed Europe PMC Scholia
  56. Paytubi S, Morrice NA, Boudeau J, Proud CG.; ''The N-terminal region of ABC50 interacts with eukaryotic initiation factor eIF2 and is a target for regulatory phosphorylation by CK2.''; PubMed Europe PMC Scholia
  57. Tammaro P, Ashcroft FM.; ''A mutation in the ATP-binding site of the Kir6.2 subunit of the KATP channel alters coupling with the SUR2A subunit.''; PubMed Europe PMC Scholia
  58. Engel T, Lorkowski S, Lueken A, Rust S, Schlüter B, Berger G, Cullen P, Assmann G.; ''The human ABCG4 gene is regulated by oxysterols and retinoids in monocyte-derived macrophages.''; PubMed Europe PMC Scholia
  59. Kaminski WE, Piehler A, Schmitz G.; ''Genomic organization of the human cholesterol-responsive ABC transporter ABCA7: tandem linkage with the minor histocompatibility antigen HA-1 gene.''; PubMed Europe PMC Scholia
  60. Jensen TJ, Loo MA, Pind S, Williams DB, Goldberg AL, Riordan JR.; ''Multiple proteolytic systems, including the proteasome, contribute to CFTR processing.''; PubMed Europe PMC Scholia
  61. Vaughan AM, Oram JF.; ''ABCG1 redistributes cell cholesterol to domains removable by high density lipoprotein but not by lipid-depleted apolipoproteins.''; PubMed Europe PMC Scholia
  62. Gelman MS, Kannegaard ES, Kopito RR.; ''A principal role for the proteasome in endoplasmic reticulum-associated degradation of misfolded intracellular cystic fibrosis transmembrane conductance regulator.''; PubMed Europe PMC Scholia
  63. Liu LX, Janvier K, Berteaux-Lecellier V, Cartier N, Benarous R, Aubourg P.; ''Homo- and heterodimerization of peroxisomal ATP-binding cassette half-transporters.''; PubMed Europe PMC Scholia
  64. Kamisako T, Leier I, Cui Y, König J, Buchholz U, Hummel-Eisenbeiss J, Keppler D.; ''Transport of monoglucuronosyl and bisglucuronosyl bilirubin by recombinant human and rat multidrug resistance protein 2.''; PubMed Europe PMC Scholia
  65. Demirel O, Waibler Z, Kalinke U, Grünebach F, Appel S, Brossart P, Hasilik A, Tampé R, Abele R.; ''Identification of a lysosomal peptide transport system induced during dendritic cell development.''; PubMed Europe PMC Scholia
  66. Zhang F, Zhang W, Liu L, Fisher CL, Hui D, Childs S, Dorovini-Zis K, Ling V.; ''Characterization of ABCB9, an ATP binding cassette protein associated with lysosomes.''; PubMed Europe PMC Scholia
  67. Younger JM, Chen L, Ren HY, Rosser MF, Turnbull EL, Fan CY, Patterson C, Cyr DM.; ''Sequential quality-control checkpoints triage misfolded cystic fibrosis transmembrane conductance regulator.''; PubMed Europe PMC Scholia
  68. Graf SA, Haigh SE, Corson ED, Shirihai OS.; ''Targeting, import, and dimerization of a mammalian mitochondrial ATP binding cassette (ABC) transporter, ABCB10 (ABC-me).''; PubMed Europe PMC Scholia
  69. Berge KE, Tian H, Graf GA, Yu L, Grishin NV, Schultz J, Kwiterovich P, Shan B, Barnes R, Hobbs HH.; ''Accumulation of dietary cholesterol in sitosterolemia caused by mutations in adjacent ABC transporters.''; PubMed Europe PMC Scholia
  70. Ousingsawat J, Kongsuphol P, Schreiber R, Kunzelmann K.; ''CFTR and TMEM16A are separate but functionally related Cl- channels.''; PubMed Europe PMC Scholia
  71. Ikeda Y, Abe-Dohmae S, Munehira Y, Aoki R, Kawamoto S, Furuya A, Shitara K, Amachi T, Kioka N, Matsuo M, Yokoyama S, Ueda K.; ''Posttranscriptional regulation of human ABCA7 and its function for the apoA-I-dependent lipid release.''; PubMed Europe PMC Scholia
  72. Zhang F, Hogue DL, Liu L, Fisher CL, Hui D, Childs S, Ling V.; ''M-ABC2, a new human mitochondrial ATP-binding cassette membrane protein.''; PubMed Europe PMC Scholia
  73. Stefková J, Poledne R, Hubácek JA.; ''ATP-binding cassette (ABC) transporters in human metabolism and diseases.''; PubMed Europe PMC Scholia
  74. Mitsuhashi N, Miki T, Senbongi H, Yokoi N, Yano H, Miyazaki M, Nakajima N, Iwanaga T, Yokoyama Y, Shibata T, Seino S.; ''MTABC3, a novel mitochondrial ATP-binding cassette protein involved in iron homeostasis.''; PubMed Europe PMC Scholia
  75. Vulevic B, Chen Z, Boyd JT, Davis W, Walsh ES, Belinsky MG, Tew KD.; ''Cloning and characterization of human adenosine 5'-triphosphate-binding cassette, sub-family A, transporter 2 (ABCA2).''; PubMed Europe PMC Scholia
  76. Deeley RG, Westlake C, Cole SP.; ''Transmembrane transport of endo- and xenobiotics by mammalian ATP-binding cassette multidrug resistance proteins.''; PubMed Europe PMC Scholia
  77. Shulenin S, Nogee LM, Annilo T, Wert SE, Whitsett JA, Dean M.; ''ABCA3 gene mutations in newborns with fatal surfactant deficiency.''; PubMed Europe PMC Scholia
  78. Accurso FJ, Rowe SM, Clancy JP, Boyle MP, Dunitz JM, Durie PR, Sagel SD, Hornick DB, Konstan MW, Donaldson SH, Moss RB, Pilewski JM, Rubenstein RC, Uluer AZ, Aitken ML, Freedman SD, Rose LM, Mayer-Hamblett N, Dong Q, Zha J, Stone AJ, Olson ER, Ordoñez CL, Campbell PW, Ashlock MA, Ramsey BW.; ''Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.''; PubMed Europe PMC Scholia
  79. Ward CL, Kopito RR.; ''Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins.''; PubMed Europe PMC Scholia
  80. Kaminski WE, Wenzel JJ, Piehler A, Langmann T, Schmitz G.; ''ABCA6, a novel a subclass ABC transporter.''; PubMed Europe PMC Scholia
  81. Roerig P, Mayerhofer P, Holzinger A, Gärtner J.; ''Characterization and functional analysis of the nucleotide binding fold in human peroxisomal ATP binding cassette transporters.''; PubMed Europe PMC Scholia


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101456view11:32, 1 November 2018ReactomeTeamreactome version 66
100994view21:11, 31 October 2018ReactomeTeamreactome version 65
100530view19:45, 31 October 2018ReactomeTeamreactome version 64
100077view16:29, 31 October 2018ReactomeTeamreactome version 63
99628view15:01, 31 October 2018ReactomeTeamreactome version 62 (2nd attempt)
99234view12:44, 31 October 2018ReactomeTeamreactome version 62
93773view13:35, 16 August 2017ReactomeTeamreactome version 61
93299view11:19, 9 August 2017ReactomeTeamreactome version 61
86383view09:16, 11 July 2016ReactomeTeamreactome version 56
83823view13:07, 13 December 2015EgonwTypo: LFCA -> LCFA
83165view10:15, 18 November 2015ReactomeTeamVersion54
82708view09:11, 23 October 2015EgonwTypo: LFCA -> LCFA
81529view13:04, 21 August 2015ReactomeTeamVersion53
76998view08:29, 17 July 2014ReactomeTeamFixed remaining interactions
76703view12:07, 16 July 2014ReactomeTeamFixed remaining interactions
76516view11:41, 16 July 2014ReactomeTeamFixed remaining interactions
76029view10:09, 11 June 2014ReactomeTeamRe-fixing comment source
75738view11:22, 10 June 2014ReactomeTeamReactome 48 Update
75088view14:04, 8 May 2014AnweshaFixing comment source for displaying WikiPathways description
74735view08:49, 30 April 2014ReactomeTeamReactome46
44936view12:20, 6 October 2011MartijnVanIerselOntology Term : 'transport pathway' added !
42004view21:49, 4 March 2011MaintBotAutomatic update
39806view05:50, 21 January 2011MaintBotNew pathway

External references


View all...
NameTypeDatabase referenceComment
ABCA cholesterol transportersREACT_17258 (Reactome)
ABCA4ProteinP78363 (Uniprot-TrEMBL)
ABCA7 ProteinQ8IZY2 (Uniprot-TrEMBL)
ABCA7-1 ApoA1 complexComplexREACT_17496 (Reactome)
ABCA7-dependent phospholipidsMetaboliteREACT_15647 (Reactome)
ABCA7-dependent phospholipidsMetaboliteREACT_16108 (Reactome)
ABCA8/B1/B5ProteinREACT_111404 (Reactome)
ABCB6 ProteinQ9NP58 (Uniprot-TrEMBL)
ABCCsProteinREACT_111491 (Reactome)
ABCD1 ProteinP33897 (Uniprot-TrEMBL)
ABCD1/2/3 dimersComplexREACT_111783 (Reactome)
ABCD2 ProteinQ9UBJ2 (Uniprot-TrEMBL)
ABCD3ProteinP28288 (Uniprot-TrEMBL)
ABCG1 ProteinP45844 (Uniprot-TrEMBL)
ABCG1 dimerComplexREACT_14301 (Reactome)
ABCG4 ProteinQ9H172 (Uniprot-TrEMBL)
ABCG4 dimerComplexREACT_111598 (Reactome)
ABCG5 ABCG8 heterodimerComplexREACT_14030 (Reactome)
ABCG5 ProteinQ9H222 (Uniprot-TrEMBL)
ABCG8 ProteinQ9H221 (Uniprot-TrEMBL)
ADPMetaboliteCHEBI:16761 (ChEBI)
APOA1ProteinP02647 (Uniprot-TrEMBL)
ATPMetaboliteCHEBI:15422 (ChEBI)
CFTRProteinP13569 (Uniprot-TrEMBL)
CHOLMetaboliteCHEBI:16113 (ChEBI)
H2OMetaboliteCHEBI:15377 (ChEBI)
HCO3-MetaboliteCHEBI:17544 (ChEBI)
Heme transportersComplexREACT_111880 (Reactome)
LCFAMetaboliteCHEBI:15904 (ChEBI)
LFCAMetaboliteCHEBI:15904 (ChEBI)
MTABC3 dimerComplexREACT_111504 (Reactome)
PEX19 ABCD1/2/3ComplexREACT_15838 (Reactome)
PEX19 ProteinP40855 (Uniprot-TrEMBL)
PEX19ProteinP40855 (Uniprot-TrEMBL)
PEX3ProteinP56589 (Uniprot-TrEMBL)
PiMetaboliteCHEBI:18367 (ChEBI)
a xenobioticMetaboliteCHEBI:35703 (ChEBI)
atRALMetaboliteCHEBI:17898 (ChEBI)
cholesterolMetaboliteCHEBI:16113 (ChEBI)
hemeMetaboliteCHEBI:17627 (ChEBI)
organic anionMetaboliteCHEBI:25696 (ChEBI)
porphyrinMetaboliteCHEBI:8337 (ChEBI)
sterolsMetaboliteREACT_13962 (Reactome)
sterolsMetaboliteREACT_14321 (Reactome)

Annotated Interactions

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SourceTargetTypeDatabase referenceComment
ABCA cholesterol transportersREACT_111067 (Reactome)
ABCA cholesterol transportersREACT_111169 (Reactome)
ABCA4REACT_111189 (Reactome)
ABCA7-1 ApoA1 complexREACT_15367 (Reactome)
ABCA7-dependent phospholipidsArrowREACT_15367 (Reactome)
ABCA7-dependent phospholipidsREACT_15367 (Reactome)
ABCA8/B1/B5REACT_111164 (Reactome)
ABCCsREACT_111162 (Reactome)
ABCD1/2/3 dimersArrowREACT_15396 (Reactome)
ABCD1/2/3 dimersREACT_15322 (Reactome)
ABCG1 dimerREACT_13681 (Reactome)
ABCG4 dimerREACT_111034 (Reactome)
ABCG5 ABCG8 heterodimerREACT_13440 (Reactome)
ADPArrowREACT_111034 (Reactome)
ADPArrowREACT_111067 (Reactome)
ADPArrowREACT_111156 (Reactome)
ADPArrowREACT_111162 (Reactome)
ADPArrowREACT_111164 (Reactome)
ADPArrowREACT_111189 (Reactome)
ADPArrowREACT_13440 (Reactome)
ADPArrowREACT_13681 (Reactome)
ADPArrowREACT_15300 (Reactome)
ADPArrowREACT_15322 (Reactome)
ADPArrowREACT_15367 (Reactome)
ADPArrowREACT_22342 (Reactome)
ATPREACT_111034 (Reactome)
ATPREACT_111067 (Reactome)
ATPREACT_111156 (Reactome)
ATPREACT_111162 (Reactome)
ATPREACT_111164 (Reactome)
ATPREACT_111189 (Reactome)
ATPREACT_13440 (Reactome)
ATPREACT_13681 (Reactome)
ATPREACT_15300 (Reactome)
ATPREACT_15322 (Reactome)
ATPREACT_15367 (Reactome)
ATPREACT_22342 (Reactome)
CFTRREACT_15300 (Reactome)
CHOLArrowREACT_111034 (Reactome)
CHOLArrowREACT_111067 (Reactome)
CHOLREACT_111034 (Reactome)
CHOLREACT_111067 (Reactome)
H2OREACT_111034 (Reactome)
H2OREACT_111067 (Reactome)
H2OREACT_111156 (Reactome)
H2OREACT_111162 (Reactome)
H2OREACT_111164 (Reactome)
H2OREACT_111189 (Reactome)
H2OREACT_13440 (Reactome)
H2OREACT_13681 (Reactome)
H2OREACT_15300 (Reactome)
H2OREACT_15322 (Reactome)
H2OREACT_15367 (Reactome)
H2OREACT_22342 (Reactome)
HCO3-ArrowREACT_15300 (Reactome)
HCO3-REACT_15300 (Reactome)
Heme transportersREACT_22342 (Reactome)
LCFAREACT_15322 (Reactome)
LFCAArrowREACT_15322 (Reactome)
MTABC3 dimerREACT_111156 (Reactome)
PEX19 ABCD1/2/3REACT_15396 (Reactome)
PEX19ArrowREACT_15396 (Reactome)
PEX3ArrowREACT_15396 (Reactome)
PEX3REACT_15396 (Reactome)
PiArrowREACT_111034 (Reactome)
PiArrowREACT_111067 (Reactome)
PiArrowREACT_111156 (Reactome)
PiArrowREACT_111162 (Reactome)
PiArrowREACT_111164 (Reactome)
PiArrowREACT_111189 (Reactome)
PiArrowREACT_13440 (Reactome)
PiArrowREACT_13681 (Reactome)
PiArrowREACT_15300 (Reactome)
PiArrowREACT_15322 (Reactome)
PiArrowREACT_15367 (Reactome)
PiArrowREACT_22342 (Reactome)
REACT_111034 (Reactome) Human ABCG4 shows sequence homology to the Drosophila white gene, the product of which must dimerise to become functionally active. ABCG4 is closely related to ABCG1 with 74% identity and is thus thought to play a role in the efflux of excess cholesterol (Engel et al. 2001). Northern Blot analysis shows that ABCG4 is expressed specifically in brain and the eye (Oldfield et al. 2002).
REACT_111067 (Reactome) ABCA3 plays an important role in the formation of pulmonary surfactant, probably by transporting lipids such as cholesterol (Klugbauer and Hofmann 1996, Yamano et al. 2001). Defects in ABCA3 are the cause of pulmonary surfactant metabolism dysfunction type 3 (SMDP3) [MIM:610921] (Shulenin et al. 2004).
The exact roles of ABCA2 (Vulevic et al. 2001, Kaminski et al. 2001), ABCA6 (Kaminski & Wenzel et al. 2001), ABCA9 (Piehler et al. 2002), ABCA10 (Wenzel et al. 2003) and ABCA12 (Annilo et al. 2002), candidates for ABC lipid transporter-related activities, need to be elucidated. Even thought cholesterol-responsiveness has been noted in experimental systems, contribution of these proteins in regulation or in active transport is not yet clear.
REACT_111156 (Reactome) The human gene ABCB6 encodes a mitochondrial half-type ATP-binding cassette (ABC) protein MTABC3 which is uniquely located on the outer mitochondrial membrane and is functional as a homodimer (Krishnamurthy et al. 2006). It plays a crucial role in heme synthesis by mediating porphyrin uptake into mitochondria (Mitsuhashi et al. 2000, Krishnamurthy et al. 2006).
REACT_111162 (Reactome) The multidrug resistance associated protein (MRPs) subfamily of the ABC transporter family can transport a wide and diverse range of organic anions that can be endogenous compounds and xenobiotics and their metabolites. All human MRPs (except MRP9) can mediate these transport reactions (Deeley et al. 2006).

Separately, specific reactions have also been annotated to describe the roles of ABCC4 in platelet dense granule assembly, of ABCC1 in LTC4 export (an aspect of leukotriene synthesis), and of ABCC3 in bile salt efflux.

REACT_111164 (Reactome) Some members of the ABC transporter superfamily are able to mediate the efflux of a broad range of cytotoxic drugs from cells, leading to the name multidrug resistance (MDR) proteins (Seeger and van Veen 2009). The ABCB1 (P-glycoprotein 1[PGP], multidrug resistance protein 1 [MRP1]) is the most characterised MDR (Shen et al. 1986, Gottesman & Pastan 1988). ABCB5 (Frank et al. 2005) and ABCA8 (Tsuruoka et al. 2002) are newer members of MDRs.
REACT_111169 (Reactome) ABCA3 plays an important role in the formation of pulmonary surfactant, probably by transporting lipids such as cholesterol (Klugbauer and Hofmann 1996, Yamano et al. 2001). Defects in ABCA3 are the cause of pulmonary surfactant metabolism dysfunction type 3 (SMDP3) [MIM:610921] (Shulenin et al. 2004).
The exact roles of ABCA2 (Vulevic et al. 2001, Kaminski et al. 2001), ABCA6 (Kaminski & Wenzel et al. 2001), ABCA9 (Piehler et al. 2002), ABCA10 (Wenzel et al. 2003) and ABCA12 (Annilo et al. 2002), candidates for ABC lipid transporter-related activities, need to be elucidated. Even thought cholesterol-responsiveness has been noted in experimental systems, contribution of these proteins in regulation or in active transport is not yet clear.
REACT_111189 (Reactome) Rhodopsin (RHO) is localised to both the disc membrane and the plasma membrane of rod outer segments (ROS). All-trans-retinal (atRAL) released from rhodopsin during the bleaching process, needs to translocate to the cytosol for reduction to all-trans-retinol (atROL) via all-trans-retinol dehydrogenases. Although atRAL can diffuse through membranes unaided, there exists an ABC transporter on disc membranes which may facilitate the transport of excess atRAL. Retinal-specific ATP-binding cassette transporter (ABCA4, ABCR) is the only ABC transporter which mediates the transport of retinoids (Biswas & Biswas 2000). Studies using bovine ABCA4 demonstrates atRAL transport (Sun et al. 1999). Human ABCR was found to be identical to the ABC transporter linked to Stargardt's disease type 1 (STGD1, MIM:248200), a cause of macular degeneration in childhood (Nasonkin et al. 1998).
REACT_13440 (Reactome) ABCG5/8 in the plasma membrane mediates the ATP-dependent export of cytosolic sterols (cholesterol and phytosterols). Mutations affecting the ABCG5/8 proteins are associated with the accumulation of high levels of cholesterol and phytosterols in the body, demonstrating the specificity and physiological importance of this process (Berge et al. 2000). Human ABCG5/8 has not been studied in detail, but the homologous mouse protein complex mediate ATP-dependent sterol export (Wang et al. 2006). The mouse proteins localize to the apical plasma membranes of enterocytes and hepatocytes, consistent with the hypothesis that in vivo ABCG5/8 mediates sterol export into the gut lumen and from hepatocytes into the bile (Graf et al. 2003).
REACT_13681 (Reactome) In an ATP-dependent reaction, ABCG1 mediates the movement of intracellular cholesterol to the extracellular face of the plasma membrane. In a tissue culture model system, the active form of ABCG1 is predominantly a tetramer (Vuaghan and Oram 2005). The number of lipid molecules transported per ATP consumed is not known.
REACT_15300 (Reactome) Regulation of epithelial chloride flux, which is defective in patients with cystic fibrosis, may be mediated by phosphorylation of the cystic fibrosis transmembrane conductance regulator (CFTR) by cyclic AMP-dependent protein kinase (PKA) or protein kinase C (PKC). CFTR regulates both HCO(3)(-) secretion and HCO(3)(-) salvage in secretory epithelia.
REACT_15322 (Reactome) The 70-kDa peroxisomal membrane protein (PMP70) and the adrenoleukodystrophy protein (ALDP) are half ATP binding cassette (ABC) transporters in the peroxisome membrane. Mutations in the ALD gene encoding ALDP result in the X-linked neurodegenerative disorder adrenoleukodystrophy (Roerig et al. 2001). They are involved in metabolic transport of long and very long chain fatty acids into peroxisomes. ATP binding/hydrolysis by and phosphorylation of PMP70 and ALDP are involved in the regulation of fatty acid transport into peroxisomes (Tanaka et al. 2002).
REACT_15367 (Reactome) ABCA7 has the ability to bind apolipoproteins and promote efflux of cellular phospholipids and may have a possible role in cellular phospholipid metabolism in peripheral tissues. Like many other ABC-transporters, the exact role of ABCA7 is waiting to be elucidated.
REACT_15396 (Reactome) PEX19 is a chaperone protein that binds a broad spectrum of peroxisomal membrane proteins (PMPs), and interacts with regions of PMPs required for their targeting to peroxisomes. PEX3 is required for PEX19 to dock at peroxisomes, interacts specifically with the docking domain of PEX19, and is required for recruitment of the PEX19 docking domain to peroxisomes. The ABC transporters D1, D2 and D3 must first form dimers to become fully functional (Liu et al.1999) which then can bind with PEX19.
REACT_22342 (Reactome) Mitochondrial ABC transporters are thought to play a key role in iron metabolism and heme biosynthesis. All mitochondrial ABC transporters described to date are of the half-transporter type and would probably function as dimers (Ramjeesingh et al. 2003) but their dimerization partners have not yet been identified. ABC7 is the functional human orthologue of yeast Atm1p (Csere et al. 1998), is predicted to dimerize in the same way as Atm1p (Chloupková et al. 2004) and is probably involved in iron homeostasis. Defects in ABCB7 are the cause of X-linked sideroblastic anemia with ataxia (ASAT) [MIM:301310] (Allikmets et al. 1999). Human genes ABCB8 and ABCB10 encode mABC1 and mABC2 respectively (Hogue et al. 1999, Zhang et al 2000 respectively). They would be expected to dimerize, as demonstrated for mABC2 (Graf et al. 2004). Both are believed to have similar functionality to ABC7 although this has not been demonstrated yet.
a xenobioticArrowREACT_111164 (Reactome)
a xenobioticREACT_111164 (Reactome)
atRALArrowREACT_111189 (Reactome)
atRALREACT_111189 (Reactome)
cholesterolArrowREACT_13681 (Reactome)
cholesterolREACT_13681 (Reactome)
hemeArrowREACT_22342 (Reactome)
hemeREACT_22342 (Reactome)
organic anionArrowREACT_111162 (Reactome)
organic anionREACT_111162 (Reactome)
porphyrinArrowREACT_111156 (Reactome)
porphyrinREACT_111156 (Reactome)
sterolsArrowREACT_13440 (Reactome)
sterolsREACT_13440 (Reactome)
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