ABC-family proteins mediated transport (Homo sapiens)

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1, 218-2413-1727-299-123-814, 25, 26peroxisomal matrixcytosolmitochondrial matrixHCO3-HCO3-H2OhemeATPABCA7-dependent phospholipidslong-chain fatty acidscholesterolABCA6, 10, 12 transportersABCD1/ABCD3 homodimerABCA7-1:ApoA1 complexPEX19long-chain fatty acidsPEX19:ABCD1/ABCD3OrthophosphateCFTRABCB7ADPABCA7-dependent phospholipidscholesterolPEX3heme


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 also 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.

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  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
  33. Csere P, Lill R, Kispal G.; ''Identification of a human mitochondrial ABC transporter, the functional orthologue of yeast Atm1p.''; PubMed Europe PMC
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  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
  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
  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
  41. Biswas EE, Biswas SB.; ''The C-terminal nucleotide binding domain of the human retinal ABCR protein is an adenosine triphosphatase.''; PubMed Europe PMC
  42. Vembar SS, Brodsky JL.; ''One step at a time: endoplasmic reticulum-associated degradation.''; PubMed Europe PMC
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  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
  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
  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
  47. Chloupková M, Reaves SK, LeBard LM, Koeller DM.; ''The mitochondrial ABC transporter Atm1p functions as a homodimer.''; PubMed Europe PMC
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  80. Kaminski WE, Wenzel JJ, Piehler A, Langmann T, Schmitz G.; ''ABCA6, a novel a subclass ABC transporter.''; PubMed Europe PMC
  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


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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


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NameTypeDatabase referenceComment
ABCA6, 10, 12


UnknownREACT_17258 (Reactome)


ComplexREACT_17496 (Reactome)


MetaboliteREACT_15647 (Reactome)


MetaboliteREACT_16108 (Reactome)
ABCB7 UnknownREACT_23221 (Reactome)


UnknownREACT_15658 (Reactome)
ADP Metabolite16761 (ChEBI)
ATP Metabolite15422 (ChEBI)
CFTR ProteinP13569 (UniProt)
H2O Metabolite15377 (ChEBI)
HCO3- Metabolite17544 (ChEBI)
Orthophosphate Metabolite18367 (ChEBI)
PEX19 UnknownREACT_15871 (Reactome)


ComplexREACT_15838 (Reactome)
PEX3 ProteinP56589 (UniProt)
cholesterol Metabolite16113 (ChEBI)
heme Metabolite17627 (ChEBI)
long-chain fatty


Metabolite15904 (ChEBI)

Annotated Interactions

No annotated interactions

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