ABC-family proteins mediated transport (Homo sapiens)

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1, 12, 5257, 18, 22, 68, 748, 35, 45, 5014, 30, 62, 75, 782, 10, 16, 63, 7820, 29, 37, 39, 42...4841, 733, 11, 1925, 467, 18, 22, 68, 746, 15, 40, 6027, 5649, 7254, 585634, 3624, 66, 765, 6431, 43, 57, 59, 70...5551, 69384, 5, 9, 21, 27...716, 15, 40, 6026, 28, 5317, 23, 4432, 7933peroxisomal matrixcytosolendoplasmic reticulum lumenlysosomal lumenmitochondrial matrixtransport vesiclelamellar bodyUBC(381-456) ADPH2OPiUBC(305-380) Pimisfolded CFTR:ERADcomplexUBC(305-380) OS9 H2OPSMD6 ABCB7(23-752) PC ADPPEX3H2OPSMD12 PiUBC(229-304) RNF185 UBC(381-456) ABCCsADP23mer peptideADPPSMD13 ABCB9 dimerABCD3 KCNJ11:ABCC9SPHM PiABCC4 ABCA6 ERLIN2 RNF185 ABCC11 ABCB9 PEX19 PSMC5 ABCA7 phytosterols APOA1(25-266) a xenobioticADPUBA52(1-76) UBC(305-380) CFTR G551DUbhemeH2OABCC1 misfolded CFTR VCP DERL3 PiATPPSMA8 ADPUbRNF185 UBC(609-684) CFTR UBC(77-152) ABCA7-dependentphospholipidsABCG4 dimerEIF2S1 26S proteasomeK+PSMB4 ERLEC1 H2OABCC6 a xenobioticPG MTABC3 dimerPSMD5 PSMB11 ABCA4PC PiIvacaftor ATPRPS27A(1-76) PSMB9 lipidsABCA8 Ub-misfolded nascentCFTR:ERAD complexUBB(1-76) CFTRUBC(153-228) SPHM PiUBC(609-684) PSMA6 ABCB4sterolsABCG5 RNF5 UBB(1-76) UBC(457-532) PiDERL2 PCUBC(1-76) CHOLUBC(229-304) Ivacaftor:CFTR G551DABCD2 misfolded CFTREIF2S2 Cl-UBC(381-456) PSMA3 EIF2S3 PSMC1 PSMA4 H2OH2OUBC(533-608) ABCG1 ATPDERL3 CHOLABCF1ABCF1 ADPABCB10 ATPOS9 ABCF1:EIF2S1:EIF2S2:EIF2S3ADPATPH2OERLIN1 organic anionUBA52(1-76) PSMB1 PSMD4 ATPH2OLCFACFTRUBC(1-76) PiPEX19:ABCD1/2/3PSME3 ADPABCC2BMG,BDGUBB(77-152) PG ATPDERL1 ADPATPOS9 PiABCB8 H2OPSMF1 KCNJ11 PSMB6 ABCA2 ABCD2 ABCD1 ABCC5 PSMD14 PSMD2 DERL2 atRALABCA7-dependentphospholipidsPiCHOLSEL1L RPS27A(1-76) PCsterolsERLEC1 EIF2S3 SEL1L UBB(153-228) Cl-ADPERLEC1 porphyrinPiABCC10 atRALUBC(381-456) UBA52(1-76) UBC(609-684) UBB(1-76) PiPSMD1 H2OH2OH2OABCA9 PiPSMD11 Heme transportersRNF5 CHOLUBC(77-152) CHOL PC UBC(533-608) BMG ABCC2 EIF2S2 UBA52(1-76) PSME4 DERL1 CHOL UBC(305-380) ADPATPABCD3 UBB(77-152) UBC(609-684) UBC(1-76) PSME1 ATPPSMB5 UBC(1-76) ABCC9 VCP misfolded CFTR H2ORPS27A(1-76) PSMA1 DERL2 ERLIN2 UBC(153-228) ABCD1 PC ATPATPUb-misfolded nascentCFTRABCC9 ABCB5 PSMD10 ADPK+PSMA5 ABCB6 UBC(153-228) BDG UBC(457-532) ADPPSMB7 PSMC3 ATPIvacaftorUBC(77-152) hemePC, PGPSMD7 lipidsporphyrinATPPSMB2 ADPCl-ADPUBC(457-532) DERL1 UBB(77-152) UBB(1-76) UBB(77-152) H2OUBC(229-304) PEX19ADPABCA cholesteroltransportersPSMD8 BDG SEL1L ABCA10 HCO3-ERLIN1 CHOLRPS27A(1-76) ABCA5RNF5 CHOLBMG,BDGATPPSMA7 PiERAD complexPSMD9 PSMD3 ADPPiPiHCO3-PSMC4 PSMC2 ATPLCFACHOLUBC(153-228) ABCA3PSME2 ERLIN1 organic anionH2OABCG4 23mer peptidemisfolded CFTR PSMB8 ABCG5:ABCG8ABCB1 UBB(153-228) H2OUBB(153-228) PSMB3 EIF2S1:EIF2S2:EIF2S3UBC(533-608) BMG CFTR G551D ABCC3 ABCA12SHFM1 ABCG1 dimerphytosterols EIF2S1 ATPPiPSMB10 ATPUBC(533-608) PC, PGERLIN2 PSMA2 UBC(229-304) UBC(457-532) UBC(77-152) H2OVCP PSMC6 DERL3 PiABCA7-1:ApoA1complexABCA8/B1/B5ADPABCG8 UBB(153-228) ABCD1-3 dimers13, 4713


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). View original pathway at:Reactome.

Comments

Reactome-Converter 
Pathway is converted from Reactome ID: 382556
Reactome-version 
Reactome version: 65
Reactome Author 
Reactome Author: Gopinathrao, G

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Bibliography

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History

View all...
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

View all...
NameTypeDatabase referenceComment
23mer peptideR-ALL-5223341 (Reactome)
23mer peptideR-ALL-5223344 (Reactome)
26S proteasomeComplexR-HSA-68819 (Reactome)
ABCA cholesterol transportersComplexR-HSA-383207 (Reactome)
ABCA10 ProteinQ8WWZ4 (Uniprot-TrEMBL)
ABCA12ProteinQ86UK0 (Uniprot-TrEMBL)
ABCA2 ProteinQ9BZC7 (Uniprot-TrEMBL)
ABCA3ProteinQ99758 (Uniprot-TrEMBL)
ABCA4ProteinP78363 (Uniprot-TrEMBL)
ABCA5ProteinQ8WWZ7 (Uniprot-TrEMBL)
ABCA6 ProteinQ8N139 (Uniprot-TrEMBL)
ABCA7 ProteinQ8IZY2 (Uniprot-TrEMBL)
ABCA7-1:ApoA1 complexComplexR-HSA-382558 (Reactome)
ABCA7-dependent phospholipidsComplexR-ALL-382526 (Reactome)
ABCA7-dependent phospholipidsComplexR-ALL-382530 (Reactome)
ABCA8 ProteinO94911 (Uniprot-TrEMBL)
ABCA8/B1/B5ComplexR-HSA-1467470 (Reactome)
ABCA9 ProteinQ8IUA7 (Uniprot-TrEMBL)
ABCB1 ProteinP08183 (Uniprot-TrEMBL)
ABCB10 ProteinQ9NRK6 (Uniprot-TrEMBL)
ABCB4ProteinP21439 (Uniprot-TrEMBL)
ABCB5 ProteinQ2M3G0 (Uniprot-TrEMBL)
ABCB6 ProteinQ9NP58 (Uniprot-TrEMBL)
ABCB7(23-752) ProteinO75027 (Uniprot-TrEMBL)
ABCB8 ProteinQ9NUT2 (Uniprot-TrEMBL)
ABCB9 ProteinQ9NP78 (Uniprot-TrEMBL)
ABCB9 dimerComplexR-HSA-5223347 (Reactome)
ABCC1 ProteinP33527 (Uniprot-TrEMBL)
ABCC10 ProteinQ5T3U5 (Uniprot-TrEMBL)
ABCC11 ProteinQ96J66 (Uniprot-TrEMBL)
ABCC2 ProteinQ92887 (Uniprot-TrEMBL)
ABCC2ProteinQ92887 (Uniprot-TrEMBL)
ABCC3 ProteinO15438 (Uniprot-TrEMBL)
ABCC4 ProteinO15439 (Uniprot-TrEMBL)
ABCC5 ProteinO15440 (Uniprot-TrEMBL)
ABCC6 ProteinO95255 (Uniprot-TrEMBL)
ABCC9 ProteinO60706 (Uniprot-TrEMBL)
ABCCsComplexR-HSA-1454912 (Reactome)
ABCD1 ProteinP33897 (Uniprot-TrEMBL)
ABCD1-3 dimersComplexR-HSA-1456465 (Reactome)
ABCD2 ProteinQ9UBJ2 (Uniprot-TrEMBL)
ABCD3 ProteinP28288 (Uniprot-TrEMBL)
ABCF1 ProteinQ8NE71 (Uniprot-TrEMBL)
ABCF1:EIF2S1:EIF2S2:EIF2S3ComplexR-HSA-5226905 (Reactome)
ABCF1ProteinQ8NE71 (Uniprot-TrEMBL)
ABCG1 ProteinP45844 (Uniprot-TrEMBL)
ABCG1 dimerComplexR-HSA-194222 (Reactome)
ABCG4 ProteinQ9H172 (Uniprot-TrEMBL)
ABCG4 dimerComplexR-HSA-1454940 (Reactome)
ABCG5 ProteinQ9H222 (Uniprot-TrEMBL)
ABCG5:ABCG8ComplexR-HSA-265452 (Reactome)
ABCG8 ProteinQ9H221 (Uniprot-TrEMBL)
ADPMetaboliteCHEBI:16761 (ChEBI)
APOA1(25-266) ProteinP02647 (Uniprot-TrEMBL)
ATPMetaboliteCHEBI:15422 (ChEBI)
BDG MetaboliteCHEBI:18392 (ChEBI)
BMG MetaboliteCHEBI:16427 (ChEBI)
BMG,BDGComplexR-ALL-5679034 (Reactome)
BMG,BDGComplexR-ALL-5679042 (Reactome)
CFTR G551D ProteinP13569 (Uniprot-TrEMBL)
CFTR G551DProteinP13569 (Uniprot-TrEMBL)
CFTR ProteinP13569 (Uniprot-TrEMBL)
CFTRProteinP13569 (Uniprot-TrEMBL)
CHOL MetaboliteCHEBI:16113 (ChEBI)
CHOLMetaboliteCHEBI:16113 (ChEBI)
Cl-MetaboliteCHEBI:17996 (ChEBI)
DERL1 ProteinQ9BUN8 (Uniprot-TrEMBL)
DERL2 ProteinQ9GZP9 (Uniprot-TrEMBL)
DERL3 ProteinQ96Q80 (Uniprot-TrEMBL)
EIF2S1 ProteinP05198 (Uniprot-TrEMBL)
EIF2S1:EIF2S2:EIF2S3ComplexR-HSA-72515 (Reactome)
EIF2S2 ProteinP20042 (Uniprot-TrEMBL)
EIF2S3 ProteinP41091 (Uniprot-TrEMBL)
ERAD complexComplexR-HSA-8866531 (Reactome)
ERLEC1 ProteinQ96DZ1 (Uniprot-TrEMBL)
ERLIN1 ProteinO75477 (Uniprot-TrEMBL)
ERLIN2 ProteinO94905 (Uniprot-TrEMBL)
H2OMetaboliteCHEBI:15377 (ChEBI)
HCO3-MetaboliteCHEBI:17544 (ChEBI)
Heme transportersComplexR-HSA-1369030 (Reactome)
Ivacaftor MetaboliteCHEBI:66901 (ChEBI)
Ivacaftor:CFTR G551DComplexR-HSA-5678990 (Reactome)
IvacaftorMetaboliteCHEBI:66901 (ChEBI)
K+MetaboliteCHEBI:29103 (ChEBI)
KCNJ11 ProteinQ14654 (Uniprot-TrEMBL)
KCNJ11:ABCC9ComplexR-HSA-5678267 (Reactome)
LCFAMetaboliteCHEBI:15904 (ChEBI)
MTABC3 dimerComplexR-HSA-1368982 (Reactome)
OS9 ProteinQ13438 (Uniprot-TrEMBL)
PC MetaboliteCHEBI:16110 (ChEBI)
PC, PGComplexR-ALL-5684863 (Reactome)
PC, PGComplexR-ALL-5684869 (Reactome)
PCMetaboliteCHEBI:16110 (ChEBI)
PEX19 ProteinP40855 (Uniprot-TrEMBL)
PEX19:ABCD1/2/3ComplexR-HSA-382586 (Reactome)
PEX19ProteinP40855 (Uniprot-TrEMBL)
PEX3ProteinP56589 (Uniprot-TrEMBL)
PG MetaboliteCHEBI:17517 (ChEBI)
PSMA1 ProteinP25786 (Uniprot-TrEMBL)
PSMA2 ProteinP25787 (Uniprot-TrEMBL)
PSMA3 ProteinP25788 (Uniprot-TrEMBL)
PSMA4 ProteinP25789 (Uniprot-TrEMBL)
PSMA5 ProteinP28066 (Uniprot-TrEMBL)
PSMA6 ProteinP60900 (Uniprot-TrEMBL)
PSMA7 ProteinO14818 (Uniprot-TrEMBL)
PSMA8 ProteinQ8TAA3 (Uniprot-TrEMBL)
PSMB1 ProteinP20618 (Uniprot-TrEMBL)
PSMB10 ProteinP40306 (Uniprot-TrEMBL)
PSMB11 ProteinA5LHX3 (Uniprot-TrEMBL)
PSMB2 ProteinP49721 (Uniprot-TrEMBL)
PSMB3 ProteinP49720 (Uniprot-TrEMBL)
PSMB4 ProteinP28070 (Uniprot-TrEMBL)
PSMB5 ProteinP28074 (Uniprot-TrEMBL)
PSMB6 ProteinP28072 (Uniprot-TrEMBL)
PSMB7 ProteinQ99436 (Uniprot-TrEMBL)
PSMB8 ProteinP28062 (Uniprot-TrEMBL)
PSMB9 ProteinP28065 (Uniprot-TrEMBL)
PSMC1 ProteinP62191 (Uniprot-TrEMBL)
PSMC2 ProteinP35998 (Uniprot-TrEMBL)
PSMC3 ProteinP17980 (Uniprot-TrEMBL)
PSMC4 ProteinP43686 (Uniprot-TrEMBL)
PSMC5 ProteinP62195 (Uniprot-TrEMBL)
PSMC6 ProteinP62333 (Uniprot-TrEMBL)
PSMD1 ProteinQ99460 (Uniprot-TrEMBL)
PSMD10 ProteinO75832 (Uniprot-TrEMBL)
PSMD11 ProteinO00231 (Uniprot-TrEMBL)
PSMD12 ProteinO00232 (Uniprot-TrEMBL)
PSMD13 ProteinQ9UNM6 (Uniprot-TrEMBL)
PSMD14 ProteinO00487 (Uniprot-TrEMBL)
PSMD2 ProteinQ13200 (Uniprot-TrEMBL)
PSMD3 ProteinO43242 (Uniprot-TrEMBL)
PSMD4 ProteinP55036 (Uniprot-TrEMBL)
PSMD5 ProteinQ16401 (Uniprot-TrEMBL)
PSMD6 ProteinQ15008 (Uniprot-TrEMBL)
PSMD7 ProteinP51665 (Uniprot-TrEMBL)
PSMD8 ProteinP48556 (Uniprot-TrEMBL)
PSMD9 ProteinO00233 (Uniprot-TrEMBL)
PSME1 ProteinQ06323 (Uniprot-TrEMBL)
PSME2 ProteinQ9UL46 (Uniprot-TrEMBL)
PSME3 ProteinP61289 (Uniprot-TrEMBL)
PSME4 ProteinQ14997 (Uniprot-TrEMBL)
PSMF1 ProteinQ92530 (Uniprot-TrEMBL)
PiMetaboliteCHEBI:18367 (ChEBI)
RNF185 ProteinQ96GF1 (Uniprot-TrEMBL)
RNF5 ProteinQ99942 (Uniprot-TrEMBL)
RPS27A(1-76) ProteinP62979 (Uniprot-TrEMBL)
SEL1L ProteinQ9UBV2 (Uniprot-TrEMBL)
SHFM1 ProteinP60896 (Uniprot-TrEMBL)
SPHM MetaboliteCHEBI:17636 (ChEBI)
UBA52(1-76) ProteinP62987 (Uniprot-TrEMBL)
UBB(1-76) ProteinP0CG47 (Uniprot-TrEMBL)
UBB(153-228) ProteinP0CG47 (Uniprot-TrEMBL)
UBB(77-152) ProteinP0CG47 (Uniprot-TrEMBL)
UBC(1-76) ProteinP0CG48 (Uniprot-TrEMBL)
UBC(153-228) ProteinP0CG48 (Uniprot-TrEMBL)
UBC(229-304) ProteinP0CG48 (Uniprot-TrEMBL)
UBC(305-380) ProteinP0CG48 (Uniprot-TrEMBL)
UBC(381-456) ProteinP0CG48 (Uniprot-TrEMBL)
UBC(457-532) ProteinP0CG48 (Uniprot-TrEMBL)
UBC(533-608) ProteinP0CG48 (Uniprot-TrEMBL)
UBC(609-684) ProteinP0CG48 (Uniprot-TrEMBL)
UBC(77-152) ProteinP0CG48 (Uniprot-TrEMBL)
Ub-misfolded nascent CFTR:ERAD complexComplexR-HSA-8866535 (Reactome)
Ub-misfolded nascent CFTRComplexR-HSA-8866534 (Reactome)
UbComplexR-HSA-6793517 (Reactome)
VCP ProteinP55072 (Uniprot-TrEMBL)
a xenobioticMetaboliteCHEBI:35703 (ChEBI)
atRALMetaboliteCHEBI:17898 (ChEBI)
hemeMetaboliteCHEBI:17627 (ChEBI)
lipidsMetaboliteCHEBI:18059 (ChEBI)
misfolded CFTR ProteinP13569 (Uniprot-TrEMBL)
misfolded CFTR:ERAD complexComplexR-HSA-8866538 (Reactome)
misfolded CFTRProteinP13569 (Uniprot-TrEMBL)
organic anionMetaboliteCHEBI:25696 (ChEBI)
phytosterols MetaboliteCHEBI:26125 (ChEBI)
porphyrinMetaboliteCHEBI:8337 (ChEBI)
sterolsComplexR-ALL-265781 (Reactome)
sterolsComplexR-ALL-265784 (Reactome)

Annotated Interactions

View all...
SourceTargetTypeDatabase referenceComment
23mer peptideArrowR-HSA-5223317 (Reactome)
23mer peptideR-HSA-5223317 (Reactome)
26S proteasomemim-catalysisR-HSA-8866553 (Reactome)
ABCA cholesterol transportersmim-catalysisR-HSA-1369028 (Reactome)
ABCA cholesterol transportersmim-catalysisR-HSA-1369052 (Reactome)
ABCA12mim-catalysisR-HSA-5682285 (Reactome)
ABCA3mim-catalysisR-HSA-5683714 (Reactome)
ABCA4mim-catalysisR-HSA-1467466 (Reactome)
ABCA5mim-catalysisR-HSA-8848053 (Reactome)
ABCA7-1:ApoA1 complexmim-catalysisR-HSA-382553 (Reactome)
ABCA7-dependent phospholipidsArrowR-HSA-382553 (Reactome)
ABCA7-dependent phospholipidsR-HSA-382553 (Reactome)
ABCA8/B1/B5mim-catalysisR-HSA-1467457 (Reactome)
ABCB4mim-catalysisR-HSA-5678706 (Reactome)
ABCB9 dimermim-catalysisR-HSA-5223317 (Reactome)
ABCC2mim-catalysisR-HSA-5679041 (Reactome)
ABCCsmim-catalysisR-HSA-1454916 (Reactome)
ABCD1-3 dimersArrowR-HSA-382613 (Reactome)
ABCD1-3 dimersmim-catalysisR-HSA-382575 (Reactome)
ABCF1:EIF2S1:EIF2S2:EIF2S3ArrowR-HSA-5227009 (Reactome)
ABCF1R-HSA-5227009 (Reactome)
ABCG1 dimermim-catalysisR-HSA-266082 (Reactome)
ABCG4 dimermim-catalysisR-HSA-1454928 (Reactome)
ABCG5:ABCG8mim-catalysisR-HSA-265783 (Reactome)
ADPArrowR-HSA-1369028 (Reactome)
ADPArrowR-HSA-1369052 (Reactome)
ADPArrowR-HSA-1369065 (Reactome)
ADPArrowR-HSA-1454916 (Reactome)
ADPArrowR-HSA-1454928 (Reactome)
ADPArrowR-HSA-1467457 (Reactome)
ADPArrowR-HSA-1467466 (Reactome)
ADPArrowR-HSA-265783 (Reactome)
ADPArrowR-HSA-266082 (Reactome)
ADPArrowR-HSA-382553 (Reactome)
ADPArrowR-HSA-382560 (Reactome)
ADPArrowR-HSA-382575 (Reactome)
ADPArrowR-HSA-383190 (Reactome)
ADPArrowR-HSA-5223317 (Reactome)
ADPArrowR-HSA-5678706 (Reactome)
ADPArrowR-HSA-5678863 (Reactome)
ADPArrowR-HSA-5678992 (Reactome)
ADPArrowR-HSA-5679041 (Reactome)
ADPArrowR-HSA-5682285 (Reactome)
ADPArrowR-HSA-5683714 (Reactome)
ADPArrowR-HSA-8848053 (Reactome)
ADPArrowR-HSA-8866542 (Reactome)
ATPR-HSA-1369028 (Reactome)
ATPR-HSA-1369052 (Reactome)
ATPR-HSA-1369065 (Reactome)
ATPR-HSA-1454916 (Reactome)
ATPR-HSA-1454928 (Reactome)
ATPR-HSA-1467457 (Reactome)
ATPR-HSA-1467466 (Reactome)
ATPR-HSA-265783 (Reactome)
ATPR-HSA-266082 (Reactome)
ATPR-HSA-382553 (Reactome)
ATPR-HSA-382560 (Reactome)
ATPR-HSA-382575 (Reactome)
ATPR-HSA-383190 (Reactome)
ATPR-HSA-5223317 (Reactome)
ATPR-HSA-5678706 (Reactome)
ATPR-HSA-5678863 (Reactome)
ATPR-HSA-5678992 (Reactome)
ATPR-HSA-5679041 (Reactome)
ATPR-HSA-5682285 (Reactome)
ATPR-HSA-5683714 (Reactome)
ATPR-HSA-8848053 (Reactome)
ATPR-HSA-8866542 (Reactome)
BMG,BDGArrowR-HSA-5679041 (Reactome)
BMG,BDGR-HSA-5679041 (Reactome)
CFTR G551DR-HSA-5679000 (Reactome)
CFTRArrowR-HSA-8866851 (Reactome)
CFTRR-HSA-8866851 (Reactome)
CFTRmim-catalysisR-HSA-383190 (Reactome)
CFTRmim-catalysisR-HSA-5678863 (Reactome)
CHOLArrowR-HSA-1369028 (Reactome)
CHOLArrowR-HSA-1369052 (Reactome)
CHOLArrowR-HSA-1454928 (Reactome)
CHOLArrowR-HSA-266082 (Reactome)
CHOLArrowR-HSA-8848053 (Reactome)
CHOLR-HSA-1369028 (Reactome)
CHOLR-HSA-1369052 (Reactome)
CHOLR-HSA-1454928 (Reactome)
CHOLR-HSA-266082 (Reactome)
CHOLR-HSA-8848053 (Reactome)
Cl-ArrowR-HSA-5678863 (Reactome)
Cl-ArrowR-HSA-5678992 (Reactome)
Cl-R-HSA-5678863 (Reactome)
Cl-R-HSA-5678992 (Reactome)
EIF2S1:EIF2S2:EIF2S3R-HSA-5227009 (Reactome)
ERAD complexArrowR-HSA-8866542 (Reactome)
ERAD complexR-HSA-8866551 (Reactome)
H2OR-HSA-1369028 (Reactome)
H2OR-HSA-1369052 (Reactome)
H2OR-HSA-1369065 (Reactome)
H2OR-HSA-1454916 (Reactome)
H2OR-HSA-1454928 (Reactome)
H2OR-HSA-1467457 (Reactome)
H2OR-HSA-1467466 (Reactome)
H2OR-HSA-265783 (Reactome)
H2OR-HSA-266082 (Reactome)
H2OR-HSA-382553 (Reactome)
H2OR-HSA-382560 (Reactome)
H2OR-HSA-382575 (Reactome)
H2OR-HSA-383190 (Reactome)
H2OR-HSA-5223317 (Reactome)
H2OR-HSA-5678706 (Reactome)
H2OR-HSA-5678863 (Reactome)
H2OR-HSA-5678992 (Reactome)
H2OR-HSA-5679041 (Reactome)
H2OR-HSA-5682285 (Reactome)
H2OR-HSA-5683714 (Reactome)
H2OR-HSA-8848053 (Reactome)
H2OR-HSA-8866542 (Reactome)
HCO3-ArrowR-HSA-383190 (Reactome)
HCO3-R-HSA-383190 (Reactome)
Heme transportersmim-catalysisR-HSA-382560 (Reactome)
Ivacaftor:CFTR G551DArrowR-HSA-5679000 (Reactome)
Ivacaftor:CFTR G551Dmim-catalysisR-HSA-5678992 (Reactome)
IvacaftorR-HSA-5679000 (Reactome)
K+ArrowR-HSA-5678261 (Reactome)
K+R-HSA-5678261 (Reactome)
KCNJ11:ABCC9mim-catalysisR-HSA-5678261 (Reactome)
LCFAArrowR-HSA-382575 (Reactome)
LCFAR-HSA-382575 (Reactome)
MTABC3 dimermim-catalysisR-HSA-1369065 (Reactome)
PC, PGArrowR-HSA-5683714 (Reactome)
PC, PGR-HSA-5683714 (Reactome)
PCArrowR-HSA-5678706 (Reactome)
PCR-HSA-5678706 (Reactome)
PEX19:ABCD1/2/3R-HSA-382613 (Reactome)
PEX19ArrowR-HSA-382613 (Reactome)
PEX3ArrowR-HSA-382613 (Reactome)
PEX3R-HSA-382613 (Reactome)
PiArrowR-HSA-1369028 (Reactome)
PiArrowR-HSA-1369052 (Reactome)
PiArrowR-HSA-1369065 (Reactome)
PiArrowR-HSA-1454916 (Reactome)
PiArrowR-HSA-1454928 (Reactome)
PiArrowR-HSA-1467457 (Reactome)
PiArrowR-HSA-1467466 (Reactome)
PiArrowR-HSA-265783 (Reactome)
PiArrowR-HSA-266082 (Reactome)
PiArrowR-HSA-382553 (Reactome)
PiArrowR-HSA-382560 (Reactome)
PiArrowR-HSA-382575 (Reactome)
PiArrowR-HSA-383190 (Reactome)
PiArrowR-HSA-5223317 (Reactome)
PiArrowR-HSA-5678706 (Reactome)
PiArrowR-HSA-5678863 (Reactome)
PiArrowR-HSA-5678992 (Reactome)
PiArrowR-HSA-5679041 (Reactome)
PiArrowR-HSA-5682285 (Reactome)
PiArrowR-HSA-5683714 (Reactome)
PiArrowR-HSA-8848053 (Reactome)
PiArrowR-HSA-8866542 (Reactome)
R-HSA-1369028 (Reactome) The exact roles of ABCA2 (Vulevic et al. 2001, Kaminski et al. 2001), ABCA6 (Kaminski & Wenzel et al. 2001), ABCA9 (Piehler et al. 2002) and ABCA10 (Wenzel et al. 2003), 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.
R-HSA-1369052 (Reactome) The exact roles of ABCA2 (Vulevic et al. 2001, Kaminski et al. 2001), ABCA6 (Kaminski & Wenzel et al. 2001), ABCA9 (Piehler et al. 2002) and ABCA10 (Wenzel et al. 2003), 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.
R-HSA-1369065 (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).
R-HSA-1454916 (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.
R-HSA-1454928 (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).
R-HSA-1467457 (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.
R-HSA-1467466 (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).
R-HSA-265783 (Reactome) The complex of ATP-binding cassette sub-family G members 5 and 8 (ABCG5:ABCG8) 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).
R-HSA-266082 (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.
R-HSA-382553 (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.
R-HSA-382560 (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.
R-HSA-382575 (Reactome) The 70-kDa peroxisomal membrane protein (PMP70) and the adrenoleukodystrophy protein (ALDP aka ABCD1) are half ATP binding cassette (ABC) transporters in the peroxisome membrane. They are involved in metabolic transport of long and very long chain fatty acids into peroxisomes. Mutations in the ALD gene result in the X-linked neurodegenerative disorder adrenoleukodystrophy (ALD; MIM:300100). ABCD1 deficiency impairs the peroxisomal beta-oxidation of very long-chain fatty acids (VLCFA) and facilitates their further chain elongation by ELOVL1 resulting in accumulation of VLCFA in plasma and tissues. While all patients with ALD have mutations in the ABCD1 gene, there is no general genotype-phenotype correlation. In addition to ABCD1, other genes and environmental factors determine clinical features of ALD (Kemp et al. 2012, Berger et al. 2014).
R-HSA-382613 (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.
R-HSA-383190 (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.
R-HSA-5223317 (Reactome) ATP-binding cassette sub-family B member 9 (ABCB9, aka lysosomal ABC transporter associated with antigen processing-like, TAPL) is a homodimeric ATP-dependent low affinity peptide transporter (Wolters et al. 2005), localised on the lysosomal membrane (Zhang et al. 2000). It is able to transport a broad spectrum of peptides (from 6mer up to at least 59mer peptides, optimum of 23mers) from the cytosol to the lysosomal lumen. ABCB9 favours positively charged, aromatic or hydrophobic residues in the N- and C-terminal positions whereas negatively charged residues and asparagine and methionine residues are not favoured (Wolters et al. 2005, Demirel et al. 2007, Zhao et al. 2008). The reaction described here shows the transport of the optimum 23mer peptide.
R-HSA-5227009 (Reactome) ATP-binding cassette sub-family F member 1 (ABCF1 aka ABC50) is unlike most ABC proteins in that it does not possess membrane-spanning domains. ABCF1 interacts with eukaryotic initiation factor 2 complex (EIF2S1:EIF2S2:EIF2S3), a key player in translation initiation and control and in ribosome regulation. ABCF1 is predominantly located in the cytosol, whereas a smaller amount is also found in the nucleoplasm but not in the nucleolus. Knockout of ABCF1 impaired translation of both cap-dependent and cap-independent reporters, consistent with a positive role for ABCF1 in the function of the EIF2 complex (Paytubi et al. 2008, Paytubi et al. 2009).
R-HSA-5678261 (Reactome) ATP-sensitive inward rectifier potassium channel 11 (KCNJ11) is an inward rectifier potassium channel, favouring potassium flow into the cell rather than out of it. KCNJ11 can complex with ATP-binding cassette sub-family member 9 (ABCC9) to form cardiac and smooth muscle-type K+(ATP) channels. KCNJ11 forms the channel pore while ABCC9 is required for activation and regulation (Babenko et al. 1998, Tammaro & Ashcroft 2007).
R-HSA-5678706 (Reactome) Multidrug resistance protein 3 (ATP-binding cassette sub-family B member 4, ABCB4 aka MDR3) mediates the ATP-dependent export of organic anions and drugs from hepatocytes into the canalicular lumen in the presence of bile salts. ABCB4 is especially important for the export of phospholipids such as phosphatidylcholine (PC) from the plasma membrane of hepatocytes (Morita et al. 2007). Biliary phospholipids associate with bile salts and cholesterol in mixed micelles, thereby reducing the detergent activity and cytotoxicity of bile salts and preventing cholesterol crystallisation. Thus, ABCB4 plays a crucial role in bile formation and lipid homeostasis (Morita & Terada 2014).
R-HSA-5678863 (Reactome) Cystic fibrosis transmembrane conductance regulator (CFTR) is a low conductance chloride-selective channel that mediates the transport of chloride ions in human airway epithelial cells which plays a key role in maintaining homoeostasis of epithelial secretions in the lungs. Defects in CFTR can cause cystic fibrosis (CF; MIM:602421), a common generalised disorder in Caucasians affecting the exocrine glands. CF results in an ionic imbalance that impairs clearance of secretions, not only in the lung, but also in the pancreas, gastrointestinal tract and liver (Riordan et al. 1999, Ousingsawat et al. 2011).
R-HSA-5678992 (Reactome) Cystic fibrosis transmembrane conductance regulator (CFTR) is a low conductance chloride-selective channel that mediates the transport of chloride ions in human airway epithelial cells which plays a key role in maintaining homoeostasis of epithelial secretions in the lungs. Defects in CFTR can cause cystic fibrosis (CF; MIM:602421), a common generalised disorder in Caucasians affecting the exocrine glands. CF results in an ionic imbalance that impairs clearance of secretions, not only in the lung, but also in the pancreas, gastrointestinal tract and liver. Wide-ranging manifestations of the disease include chronic lung disease, exocrine pancreatic insufficiency, blockage of the terminal ileum, male infertility and salty sweat.

The class 3 mutations of CFTR such as G551D strongly decrease the time spent by CFTR in the open state (a gating defect). Results from 2-phase clinical trials using VX-770 (aka Ivacaftor), a CFTR potentiator, showed an increased CFTR channel open probability in G551D patients. Ivacaftor use showed improvements in CFTR and lung function of patients with at least one G551D allele (Accurso et al. 2010, Ramsey et al. 2011, Kapoor et al. 2014). In 2012, the FDA approved Ivacaftor (under the trade name Kalydeco) for use in cystic fibrosis patients with the G551D mutation (Ledford 2012).
R-HSA-5679000 (Reactome) Defects in cystic fibrosis transmembrane conductance regulator (CFTR) can cause cystic fibrosis (CF; MIM:602421), a common generalised disorder in Caucasians affecting the exocrine glands. CF results in an ionic imbalance that impairs clearance of secretions, not only in the lung, but also in the pancreas, gastrointestinal tract and liver. Wide-ranging manifestations of the disease include chronic lung disease, exocrine pancreatic insufficiency, blockage of the terminal ileum, male infertility and salty sweat. The class 3 mutations of CFTR such as G551D strongly decrease the time spent by CFTR in the open state (a gating defect). Results from 2-phase clinical trials using VX-770 (aka Ivacaftor), a CFTR potentiator, showed an increased CFTR channel open probability in G551D patients. Ivacaftor use showed improvements in CFTR and lung function of patients with at least one G551D allele (Accurso et al. 2010, Ramsey et al. 2011, Kapoor et al. 2014). In 2012, the FDA approved Ivacaftor (under the trade name Kalydeco) for use in cystic fibrosis patients with the G551D mutation (Ledford 2012).
R-HSA-5679041 (Reactome) Canalicular multispecific organic anion transporter 1 (ABCC2 aka multidrug resistance-associated protein 2, MRP2), in addition to transporting many organic anions, mediates the ATP-dependent transport of glutathione and glucuronate conjugates from hepatocytes into bile. In the reaction annotated here, ABCC2 specifically transports, with high affinity and efficiency, mono- and di-glucuronated bilirubin into bile (Kamisako et al. 1999). Bilirubin, the end product of heme breakdown, is an important constituent of bile and is responsible for its characteristic colour.
R-HSA-5682285 (Reactome) ATP-binding cassette sub-family A member 12 (ABCA12) is thought to function as an epidermal keratinocyte lipid transporter. Lipids such as glucosylceramides and gangliosides form extracellular lipid layers in the stratum corneum of the epidermis, essential for skin barrier function (Akiyama et al. 2005). Mutations in ABCA12 can cause autosomal recessive congenital ichthyoses (ARCI).
R-HSA-5683714 (Reactome) ATP-binding cassette sub-family A member 3 (ABCA3) plays an important role in the formation of pulmonary surfactant, probably by transporting phospholipids such as phosphatidylcholine (PC) and phosphatidylglycerol (PG) from the ER membrane to lamellar bodies (LBs). PC and PG are the major phospholipid constituents of pulmonary surfactant. LBs are the surfactant storage organelles of type II epithelial cells from where phospholipids can be secreted together with surfactant proteins (SFTPs) into the alveolar airspace (Klugbauer & 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).
R-HSA-8848053 (Reactome) The ABC transporter subfamily A member ABCA5 is thought to mediate cholesterol (CHOL) efflux from lysosomes (Petry et al. 2003). ABCA5 defects are associated with autosomal recessive congenital generalised hypertrichosis terminalis (CGHT; MIM:135400). Lysosomal function is reduced with an accumulation of autophagosomes and their cargo as well as increased endolysosomal cholesterol in CGHT keratinocytes, resulting in hair overgrowth (DeStefano et al. 2014).
R-HSA-8866542 (Reactome) Retrotranslocation of the misfolded CFTR likely depends on the ERAD ATPase VCP (reviewed in Vembar and Brodsky, 2008; Pranke and Sermet-Gaudelus, 2014).
R-HSA-8866546 (Reactome) RNF5 and RNF185 ubiquitinate misfolded CFTR as part of the retrotranslocon that targets the receptor for degradation through the ERAD pathway. Both depletion of the E3 ligases by siRNA and expression of a catalytically inactive form of the enzyme strongly inhibits CFTR degradation (El Khouri et al, 2013; Younger et al, 2006).
R-HSA-8866551 (Reactome) Up to two thirds of wild-type CFTR is targeted for co-translational degradation by the ERAD pathway due to inefficient folding (Jensen et al, 1995; Ward et al, 1994; Ward et al, 1995; Gelman et al, 2002; Lukacs et al, 1994). Misfolded CFTR is ubiquitinated in the ER by E3 ligases RNF5 and RNF185, likely as part of a mulitprotein retrotranslocation complex containing the hexameric ATPase VCP and various scaffolding and structural proteins (reviewed in Vembar and Brodsky, 2008). Consistent with this, RNF185 interacts directly both with CFTR and with other components of the ERAD machinery, including E2 proteins, ERLIN and DERLIN proteins (Younger et al, 2006; El Khouri et al, 2013).
R-HSA-8866553 (Reactome) After VCP-mediated translocation to the cytosol, misfolded CFTR is degraded by the 26S proteasome (El Khouri et al, 2013).
R-HSA-8866851 (Reactome) The cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-regulated chloride ion channel that undergoes multiple folding processes and post-translational modifications during its biosynthesis. 60-80% of CFTR protein encoded by the wild-type (WT) gene is successfully modified and transits the secretory system to the plasma membrane. The remaining misfolded protein is targeted for degradation by the ER, lysosomes or autophagy (reviewed in Pranke and Sermet-Gaudelus, 2014)
Ub-misfolded nascent CFTR:ERAD complexArrowR-HSA-8866546 (Reactome)
Ub-misfolded nascent CFTR:ERAD complexR-HSA-8866542 (Reactome)
Ub-misfolded nascent CFTRArrowR-HSA-8866542 (Reactome)
Ub-misfolded nascent CFTRR-HSA-8866553 (Reactome)
UbArrowR-HSA-8866553 (Reactome)
UbR-HSA-8866546 (Reactome)
a xenobioticArrowR-HSA-1467457 (Reactome)
a xenobioticR-HSA-1467457 (Reactome)
atRALArrowR-HSA-1467466 (Reactome)
atRALR-HSA-1467466 (Reactome)
hemeArrowR-HSA-382560 (Reactome)
hemeR-HSA-382560 (Reactome)
lipidsArrowR-HSA-5682285 (Reactome)
lipidsR-HSA-5682285 (Reactome)
misfolded CFTR:ERAD complexArrowR-HSA-8866551 (Reactome)
misfolded CFTR:ERAD complexR-HSA-8866546 (Reactome)
misfolded CFTR:ERAD complexmim-catalysisR-HSA-8866546 (Reactome)
misfolded CFTRR-HSA-8866551 (Reactome)
organic anionArrowR-HSA-1454916 (Reactome)
organic anionR-HSA-1454916 (Reactome)
porphyrinArrowR-HSA-1369065 (Reactome)
porphyrinR-HSA-1369065 (Reactome)
sterolsArrowR-HSA-265783 (Reactome)
sterolsR-HSA-265783 (Reactome)
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