ABC-family proteins mediated transport (Homo sapiens)

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13, 34, 53116917, 21, 49, 75, 8026, 586411, 4215, 672, 4, 15, 37, 42...435, 357, 7017, 21, 49, 75, 8024, 30, 38, 73, 816, 23, 50, 7829, 46, 65, 6632, 51, 773, 20, 33, 47, 55...6, 23, 50, 7827, 28, 4140, 5612, 741, 8, 1614, 22, 25, 31, 59...1519, 48769, 18, 546124, 36, 39, 45, 6344, 57transport vesiclelysosomal lumenperoxisomal matrixlamellar bodymitochondrial matrixcytosolendoplasmic reticulum lumenH2ODERL3 ATPADPABCG5 APOA1(25-266) OS9 ADPEIF2S3 UBC(381-456) atRALATPPiH2OABCA7-dependentphospholipidsADPADPADPmisfolded CFTR ABCD1 PSMD14 PC PC, PGABCD2 PiLCFAPG PSMC5 ABCD2 DERL3 ABCB9 dimerERLIN1 UBB(1-76) ERLEC1 UBA52(1-76) ATPATPUBB(77-152) EIF2S2 PSMA6 PSMD7 H2OABCA4H2OATPERLIN2 PSMC4 ATPPSMC3 misfolded CFTR:ERADcomplexABCA12PSMD5 lipidslipidsCHOLUBA52(1-76) PSMD6 ABCC9 UBC(609-684) ADPCl-PiPiABCF1:EIF2S1:EIF2S2:EIF2S3PiatRALUBC(609-684) H2ORNF5 UBC(77-152) PiRPS27A(1-76) H2OUBC(153-228) UBC(457-532) PSMD9 ADPATPPSME4 OS9 CFTR G551DPEX19 ABCB9 PSMB3 PiADPUBB(1-76) PiABCA7-1:ApoA1complexBMG,BDGATPERAD complexRNF5 ATPUBC(1-76) PSMD2 ABCG1 dimerhemeUBC(1-76) PiH2ORPS27A(1-76) Ub-misfolded nascentCFTRABCC11 a xenobioticPia xenobioticUBC(153-228) ABCA10 UBB(1-76) UBC(533-608) IvacaftorABCB1 PEX3ERLIN1 PSMD13 PSMC2 Cl-PSMD3 H2OCHOLCHOL PSMA2 ABCC9 CFTR G551D UBC(381-456) ATPABCA2 BMG OS9 UbH2OsterolsBMG,BDGABCC3 UBC(77-152) PSMD11 UBA52(1-76) ATPPiPSMB9 UBC(609-684) KCNJ11:ABCC9PSMC6 PSMD4 ATPABCB6 UBC(77-152) PiUBA52(1-76) ATPIvacaftor:CFTR G551DPiSPHM HCO3-UBC(305-380) RNF185 UBC(533-608) ABCC2 ABCCsPSMB7 sterolsDERL1 H2OEIF2S2 PSME1 H2OPSMA4 PSMB8 CHOLPSME3 SEL1L UBC(1-76) ABCA cholesteroltransportersUBB(153-228) PEX19:ABCD1/2/3ERLIN1 PCporphyrinUBC(229-304) PSMD10 ERLIN2 UBB(77-152) UBC(1-76) PSMA3 PiCl-ATPCHOLABCC6 UBC(305-380) CHOL ABCG1 misfolded CFTR PSMB6 ADPPSMF1 PSMA8 VCP SPHM ABCC10 CHOLABCG4 dimerABCA8/B1/B5PSMD1 UBB(77-152) BMG K+porphyrinphytosterols CFTRPiATPUBB(153-228) PSMD8 CHOLDERL2 K+PC PSMB10 PSMB5 UBC(381-456) PSMA7 ADPVCP RNF185 UBC(457-532) ATPUBB(153-228) UBB(1-76) PSMB1 PG ABCA9 ERLEC1 H2OCFTRADPUBC(457-532) PSME2 Ivacaftor H2OUBB(77-152) PiDERL2 ABCA7-dependentphospholipidsRNF185 EIF2S1 23mer peptideABCC4 RNF5 H2Ophytosterols ABCD3 misfolded CFTRPSMD12 misfolded CFTR PCVCP UbCHOLADPABCG5:ABCG8PSMB11 H2OPiPiDERL2 PiADPDERL1 ABCF1 UBC(457-532) Heme transportersUBC(381-456) ABCC1 PC UBC(77-152) ABCB5 ABCB10 UBC(229-304) UBC(305-380) DERL3 23mer peptidePC ABCD3 DERL1 PSMC1 26S proteasomeUb-misfolded nascentCFTR:ERAD complexUBB(153-228) H2ORPS27A(1-76) organic anionABCB8 ABCA5SEL1L EIF2S1:EIF2S2:EIF2S3UBC(229-304) UBC(305-380) RPS27A(1-76) ADPMTABC3 dimerABCA3SHFM1 hemeATPUBC(229-304) ATPERLEC1 ERLIN2 ADPUBC(533-608) PEX19PSMB2 ABCD1 ADPKCNJ11 LCFAPSMA5 organic anionABCD1-3 dimersEIF2S3 UBC(609-684) ABCG8 CFTR ADPADPUBC(153-228) ABCB4ABCC2ABCB7(23-752) HCO3-UBC(153-228) UBC(533-608) ADPABCG4 ABCA8 ABCF1SEL1L H2OPSMB4 EIF2S1 ABCC5 PSMA1 ABCA6 ABCA7 PC, PGBDG H2OBDG 10, 5210


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.


Pathway is converted from Reactome ID: 382556
Reactome version: 66
Reactome Author 
Reactome Author: Gopinathrao, G

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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
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)