ABC-family proteins mediated transport (Homo sapiens)

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15, 22, 418, 25, 261, 9, 17, 32, 4510, 165, 212, 11, 17, 34, 36243, 13, 19, 29-31, 35...6, 18, 23, 27, 40...3, 13, 19, 29-31, 35...14, 20, 3728, 42, 49484, 7, 12, 33, 38...ABCG1 dimer mitochondrial matrixABCD1/2/3 dimers ABCD1ABCD2 peroxisomal matrixABCD2ABCD3 ABCD1ABCD3 MTABC3 dimer ABCA7-1ApoA1 complex cytosolABCG5ABCG8 heterodimer ABCG4 dimer PEX19ABCD1/2/3 transport vesicleABCD1ABCD1 ADPHCO3-PiATPH2OABCA cholesterol transportersporphyrinCHOLABCD1/2/3 dimersCHOLH2OPiATPPiABCG4 dimerABCD2 H2OPiatRALABCA7-dependent phospholipidsABCG5 ADPLFCAADPAPOA1PEX19ABCD1/2/3ATPADPH2OH2OHCO3-organic anionPEX19 ABCA7-dependent phospholipidsADPABCG1 H2OsterolsABCD1 ABCG4 ADPhemea xenobioticATPADPATPABCA8/B1/B5organic anionABCCscholesterola xenobioticADPADPABCG5ABCG8 heterodimerCFTRCHOLcholesterolPEX3ABCD3ATPATPhemeatRALABCG8 H2OABCG1 dimerH2OABCB6 PiCHOLABCA7-1ApoA1 complexPiPiPiPEX19PiADPporphyrinPiATPABCA4Heme transporterssterolsH2OH2OLCFAABCA7 ATPMTABC3 dimerATP


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Wikipathways-description 
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: http://www.reactome.org/PathwayBrowser/#DB=gk_current&FOCUS_SPECIES_ID=48887&FOCUS_PATHWAY_ID=382556

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Bibliography

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History

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CompareRevisionActionTimeUserComment
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

DataNodes

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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

View all...
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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