ABC-family proteins mediated transport (Homo sapiens)

From WikiPathways

Revision as of 21:49, 4 March 2011 by MaintBot (Talk | contribs)
Jump to: navigation, search
1, 213-179-1214, 25, 2618-243-827-29mitochondrial matrixcytosolperoxisomal matrixPEX19PEX19:ABCD1/ABCD3HCO3-ABCA7-dependent phospholipidsCFTRhemeATPlong-chain fatty acidsABCD1/ABCD3 homodimerABCB7ABCA7-dependent phospholipidsABCA6, 10, 12 transportershemecholesterolHCO3-PEX3OrthophosphateABCA7-1:ApoA1 complexH2OADPlong-chain fatty acidscholesterol


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 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.

Try the New WikiPathways

View approved pathways at the new wikipathways.org.

Quality Tags

Ontology Terms

 

Bibliography

View all...
  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

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
ABCA6, 10, 12

transporters

UnknownREACT_17258 (Reactome)
ABCA7-1:ApoA1

complex

ComplexREACT_17496 (Reactome)
ABCA7-dependent

phospholipids

MetaboliteREACT_15647 (Reactome)
ABCA7-dependent

phospholipids

MetaboliteREACT_16108 (Reactome)
ABCB7 UnknownREACT_23221 (Reactome)
ABCD1/ABCD3

homodimer

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)
PEX19:

ABCD1/ABCD3

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

acids

Metabolite15904 (ChEBI)

Annotated Interactions

No annotated interactions

Retrieved from "https://classic.wikipathways.org/index.php/Pathway:WP1780"
Personal tools