ABCB subfamily | Transporters | IUPHAR/BPS Guide to PHARMACOLOGY

ABCB subfamily C

Unless otherwise stated all data on this page refer to the human proteins. Gene information is provided for human (Hs), mouse (Mm) and rat (Rn).

Overview

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The ABCB subfamily is composed of four full transporters and two half transporters. This is the only human subfamily to have both half and full types of transporters. ABCB1 was discovered as a protein overexpressed in certain drug resistant tumor cells. It is expressed primarily in the blood brain barrier and liver and is thought to be involved in protecting cells from toxins. Cells that overexpress this protein exhibit multi-drug resistance [1,5].

Transporters

777

MDR1, PGP1 (ABCB1) C Show summary »« Hide summary More detailed page go icon to follow link

Target Id	768
Systematic nomenclature	ABCB1
Common abbreviation	MDR1, PGP1
Previous and unofficial names	ABC20 \| Abcb1 \| Abcb1a \| ATP-binding cassette \| ATP-binding cassette, sub-family B (MDR/TAP), member 1 \| ATP-binding cassette, subfamily B (MDR/TAP), member 1A \| ATP binding cassette subfamily B member 1 \| CD243 \| CLCS \| colchicin sensitivity \| Evi32 \| GP170 \| MDR1 \| Mdr1a \| MDR3 \| mdr-3 \| multidrug resistance protein 1 \| multiple drug resistant 1a \| P-glycoprotein 1 \| Pgp \| P-gp \| PGY1 \| Pgy3 \| Pgy-3
Genes	ABCB1 (Hs), Abcb1a (Mm), Abcb1a (Rn)
Ensembl ID	ENSG00000085563 (Hs), ENSMUSG00000040584 (Mm), ENSRNOG00000008012 (Rn)
UniProtKB AC	P08183 (Hs), P21447 (Mm)
Comment	Responsible for the cellular export of many therapeutic drugs. The mouse and rat have two Abcb1 genes (gene names; Abcb1a and Abcb1b) while the human has only the one gene, ABCB1.

TAP1 (ABCB2) C Show summary »« Hide summary

Target Id	769
Systematic nomenclature	ABCB2
Common abbreviation	TAP1
Previous and unofficial names	Antigen peptide transporter 1 \| APT1 \| peptide supply factor 1 (PSF-1) \| peptide transporter TAP1 \| RING4 \| TAP \| ATP-binding cassette sub-family B member 2 \| Cim \| Transporter 1 ABC (ATP binding cassette) \| Ham1 \| MTP1 \| peptide transporter involved in antigen processing 1 \| transporter 1, ATP-binding cassette, sub-family B (MDR/TAP) \| transporter 1 \| transporter 1, ATP binding cassette subfamily B member
Genes	TAP1 (Hs), Tap1 (Mm), Tap1 (Rn)
Ensembl ID	ENSG00000168394 (Hs), ENSMUSG00000037321 (Mm), ENSRNOG00000000457 (Rn)
UniProtKB AC	Q03518 (Hs), P21958 (Mm), P36370 (Rn)
Comment	Endoplasmic reticulum peptide transporter is a hetero-dimer composed of the two half-transporters, TAP1 (ABCB2) and TAP2 (ABCB3). The transporter shuttles peptides into the endoplasmic reticulum where they are loaded onto major histocompatibility complex class I (MHCI) molecules via the macromoldecular peptide-loading complex and are eventually presented at the cell surface, attributing to TAP an important role in the adaptive immune response [18].

TAP2 (ABCB3) C Show summary »« Hide summary More detailed page go icon to follow link

Target Id	770
Systematic nomenclature	ABCB3
Common abbreviation	TAP2
Previous and unofficial names	Antigen peptide transporter 2 (APT2) \| peptide supply factor 2 (PSF-2) \| Peptide transporter TAP2 \| ATP-binding cassette sub-family B member 3 \| Cim \| Transporter 2 ABC (ATP binding cassette) \| Ham2 \| MTP2 \| peptide transporter involved in antigen processing 2 \| RING11 \| transporter 2, ATP-binding cassette, sub-family B (MDR/TAP) \| transporter 2 \| transporter 2, ATP binding cassette subfamily B member
Genes	TAP2 (Hs), Tap2 (Mm), Tap2 (Rn)
Ensembl ID	ENSG00000204267 (Hs), ENSMUSG00000024339 (Mm), ENSRNOG00000000455 (Rn)
UniProtKB AC	Q03519 (Hs), P36371 (Mm), P36372 (Rn)
Comment	Endoplasmic reticulum peptide transporter is a hetero-dimer composed of the two half-transporters, TAP1 (ABCB2) and TAP2 (ABCB3). The transporter shuttles peptides into the endoplasmic reticulum where they are loaded onto major histocompatibility complex class I (MHCI) molecules via the macromolecular peptide-loading complex and are eventually presented at the cell surface, attributing to TAP an important role in the adaptive immune response [18].

PGY3 (ABCB4) C Show summary »« Hide summary

Target Id	771
Systematic nomenclature	ABCB4
Common abbreviation	PGY3
Previous and unofficial names	P-glycoprotein 3 \| GBD1 \| MDR2 \| MDR3 \| PFIC-3 \| PGY3 \| ATP-binding cassette sub-family B (MDR/TAP) member 4 (P-glycoprotein 3/ multidrug resistance 2 \| multidrug resistance protein 2 \| multidrug resistance protein 3 \| P-glycoprotein 2 \| P-glycoprotein 3/ multidrug resistance 2 \| Pgy2 \| ATP-binding cassette, subfamily B (MDR/TAP), member 4 \| ATP-binding cassette
Genes	ABCB4 (Hs), Abcb4 (Mm), Abcb4 (Rn)
Ensembl ID	ENSG00000005471 (Hs), ENSMUSG00000042476 (Mm), ENSRNOG00000008012 (Rn), ENSRNOG00000068001 (Rn)
UniProtKB AC	P21439 (Hs), P21440 (Mm), Q08201 (Rn)
Comment	Transports phosphatidylcholine from intracellular to extracellular face of the hepatocyte canalicular membrane [15]. Heterozygous ABCB4 variants contribute to mild cholestatic phenotypes, while homozygous deficiency leads to Progressive Intrahepatic Familial Cholestasis (PFIC) Type 3, and increased risk of cholesterol gallstones [10].

ABCB5 C Show summary »« Hide summary

Target Id	772
Systematic nomenclature	ABCB5
Previous and unofficial names	ABCB5alpha \| ABCB5beta \| ATP-binding cassette, sub-family B (MDR/TAP), member 5 \| ATP-binding cassette
Genes	ABCB5 (Hs), Abcb5 (Mm), Abcb5 (Rn)
Ensembl ID	ENSG00000004846 (Hs), ENSMUSG00000072791 (Mm), ENSRNOG00000006398 (Rn)
UniProtKB AC	Q2M3G0 (Hs)
Comment	A drug efflux transporter that has been shown to identify cancer stem-like cells in diverse human malignancies, and is also identified as a limbal stem cell that is required for corneal development and repair [13,22].

MTABC3 (ABCB6) C Show summary »« Hide summary

Target Id	773
Systematic nomenclature	ABCB6
Common abbreviation	MTABC3
Previous and unofficial names	Mitochondrial ABC transporter 3 \| P-glycoprotein-related protein \| ubiquitously expressed mammalian ABC half transporter \| MTABC3 \| umat \| ATP-binding cassette half-transporter \| ATP-binding cassette sub-family B member 6, mitochondrial \| ATP-binding cassette, sub-family B (MDR/TAP), member 6 \| ATP-binding cassette, sub-family B (MDR/TAP), member 6 (Langereis blood group) \| ATP-binding cassette, subfamily B (MDR/TAP), member 6 \| ATP-binding cassette \| ATP binding cassette subfamily B member 6 (Langereis blood group)
Genes	ABCB6 (Hs), Abcb6 (Mm), Abcb6 (Rn)
Ensembl ID	ENSG00000115657 (Hs), ENSMUSG00000026198 (Mm), ENSRNOG00000018697 (Rn)
UniProtKB AC	Q9NP58 (Hs), Q9DC29 (Mm), O70595 (Rn)
Comment	Putative mitochondrial porphyrin transporter [12]; other subcellular localizations are possible, such as the plasma membrane, as a specific determinant of the Langereis blood group system [9]. Loss of Abcb6 expression in mice leads to decreased expression and activity of CYP450 [4].

ABC7 (ABCB7) C Show summary »« Hide summary

Target Id	774
Systematic nomenclature	ABCB7
Common abbreviation	ABC7
Previous and unofficial names	ABC7 \| ASAT \| ABC transporter 7 protein \| ATP-binding cassette sub-family B member 7, mitochondrial \| ATP-binding cassette transporter 7 \| ATP-binding cassette, sub-family B (MDR/TAP), member 7 \| Atm1p \| ATP-binding cassette, subfamily B (MDR/TAP), member 7 \| ATP-binding cassette
Genes	ABCB7 (Hs), Abcb7 (Mm), Abcb7 (Rn)
Ensembl ID	ENSG00000131269 (Hs), ENSMUSG00000031333 (Mm), ENSRNOG00000002790 (Rn)
UniProtKB AC	O75027 (Hs), Q61102 (Mm), Q704E8 (Rn)
Comment	Mitochondrial; reportedly essential for haematopoiesis [16]. Deletion studies in mice demonstrate that Abcb7 is essential in mammals and substantiate a role for mitochondria in cytosolic Fe-S cluster assembly [17].

MABC1 (ABCB8) C Show summary »« Hide summary

Target Id	775
Systematic nomenclature	ABCB8
Common abbreviation	MABC1
Previous and unofficial names	MABC1 \| mitochondrial ABC protein \| ABC transporter 8 \| ATP-binding cassette sub-family B member 8, mitochondrial \| ATP-binding cassette, sub-family B (MDR/TAP), member 8 \| ATP-binding cassette, subfamily B (MDR/TAP), member 8 \| ATP-binding cassette
Genes	ABCB8 (Hs), Abcb8 (Mm), Abcb8 (Rn)
Ensembl ID	ENSG00000197150 (Hs), ENSMUSG00000028973 (Mm), ENSRNOG00000008557 (Rn)
UniProtKB AC	Q9NUT2 (Hs), Q9CXJ4 (Mm), Q5RKI8 (Rn)
Comment	Mitochondrial; suggested to play a role in chemoresistance of melanoma [7]. Cardiac specific deletion of Abcb8 leads to cardiomyopathy and accumulation of mitochondrial iron, and is thus thought to modulate mitochondrial iron export [11].

TAPL (ABCB9) C Show summary »« Hide summary

Target Id	776
Systematic nomenclature	ABCB9
Common abbreviation	TAPL
Previous and unofficial names	ABC transporter 9 protein \| ATP-binding cassette, sub-family B (MDR/TAP), member 9 \| Tapl \| TAP-like ABC transporter \| ATP-binding cassette, subfamily B (MDR/TAP), member 9 \| ATP-binding cassette
Genes	ABCB9 (Hs), Abcb9 (Mm), Abcb9 (Rn)
Ensembl ID	ENSG00000150967 (Hs), ENSMUSG00000029408 (Mm), ENSRNOG00000001082 (Rn)
UniProtKB AC	Q9NP78 (Hs), Q9JJ59 (Mm), Q9QYJ4 (Rn)
Comment	A homodimeric transport complex that translocates cytosolic peptides into the lumne of lysosome for degradation [6].

MTABC2 (ABCB10) C Show summary »« Hide summary

Target Id	777
Systematic nomenclature	ABCB10
Common abbreviation	MTABC2
Previous and unofficial names	Mitochondrial ABC transporter 2 \| M-ABC2 \| MTABC2 \| ABC transporter 10 protein \| ATP-binding cassette, sub-family B (MDR/TAP), member 10 \| ABC-me \| ATP-binding cassette sub-family B member 10, mitochondrial \| ATP-binding cassette, subfamily B (MDR/TAP), member 10 \| ATP-binding cassette
Genes	ABCB10 (Hs), Abcb10 (Mm), Abcb10 (Rn)
Ensembl ID	ENSG00000135776 (Hs), ENSMUSG00000031974 (Mm), ENSRNOG00000017993 (Rn)
UniProtKB AC	Q9NRK6 (Hs), Q9JI39 (Mm)
Comment	Mitochondrial location; the first human ABC transporter to have a crystal structure reported [19]. ABCB10 is important in early steps of heme synthesis in the heart and is required for normal red blood cell development [2,21].

ABC16 (ABCB11) C Show summary »« Hide summary

Target Id

778

Systematic nomenclature

ABCB11

Common abbreviation

ABC16

Previous and unofficial names

Genes

ABCB11 (Hs), Abcb11 (Mm), Abcb11 (Rn)

Ensembl ID

ENSG00000073734 (Hs), ENSMUSG00000027048 (Mm), ENSRNOG00000050860 (Rn)

UniProtKB AC

O95342 (Hs), Q9QY30 (Mm), O70127 (Rn)

Ligands

glycochenodeoxycholic acid (Binding) pK_i 5.2 [3]

Comment

Loss-of-function mutations are associated with progressive familial intrahepatic cholestasis type 2 [20]. ATP-dependent transport of bile acids into the confines of the canalicular space by ABCB11 (BSEP) generates an osmotic gradient and thereby, bile flow. Mutations in BSEP that decrease its function or expression cause Progressive Familial Cholestasis Type 2 (PFIC2), which in severe cases, can be fatal in the absence of a liver transplant. Drugs that inhibit BSEP function with IC50 values less than 25 μM [14] or decrease its expression [8] can cause Drug-Induced Liver Injury (DILI) in the form of cholestatic liver injury.

References

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1. Alexander SPH, Kelly E, Mathie A, Peters JA, Veale EL, Armstrong JF, Faccenda E, Harding SD, Pawson AJ, Sharman JL et al.. (2019) THE CONCISE GUIDE TO PHARMACOLOGY 2019/20: Transporters. Br J Pharmacol, 176 Suppl 1: S397-S493. [PMID:31710713]

2. Bayeva M, Khechaduri A, Wu R, Burke MA, Wasserstrom JA, Singh N, Liesa M, Shirihai OS, Langer NB, Paw BH et al.. (2013) ATP-binding cassette B10 regulates early steps of heme synthesis. Circ Res, 113 (3): 279-87. [PMID:23720443]

3. Byrne JA, Strautnieks SS, Mieli-Vergani G, Higgins CF, Linton KJ, Thompson RJ. (2002) The human bile salt export pump: characterization of substrate specificity and identification of inhibitors. Gastroenterology, 123 (5): 1649-58. [PMID:12404239]

4. Chavan H, Li F, Tessman R, Mickey K, Dorko K, Schmitt T, Kumer S, Gunewardena S, Gaikwad N, Krishnamurthy P. (2015) Functional coupling of ATP-binding cassette transporter Abcb6 to cytochrome P450 expression and activity in liver. J Biol Chem, 290 (12): 7871-86. [PMID:25623066]

5. Dean M, Hamon Y, Chimini G. (2001) The human ATP-binding cassette (ABC) transporter superfamily. J Lipid Res, 42 (7): 1007-17. [PMID:11441126]

6. Demirel Ö, Jan I, Wolters D, Blanz J, Saftig P, Tampé R, Abele R. (2012) The lysosomal polypeptide transporter TAPL is stabilized by interaction with LAMP-1 and LAMP-2. J Cell Sci, 125 (Pt 18): 4230-40. [PMID:22641697]

7. Elliott AM, Al-Hajj MA. (2009) ABCB8 mediates doxorubicin resistance in melanoma cells by protecting the mitochondrial genome. Mol Cancer Res, 7 (1): 79-87. [PMID:19147539]

8. Garzel B, Yang H, Zhang L, Huang SM, Polli JE, Wang H. (2014) The role of bile salt export pump gene repression in drug-induced cholestatic liver toxicity. Drug Metab Dispos, 42 (3): 318-22. [PMID:24335466]

9. Helias V, Saison C, Ballif BA, Peyrard T, Takahashi J, Takahashi H, Tanaka M, Deybach JC, Puy H, Le Gall M et al.. (2012) ABCB6 is dispensable for erythropoiesis and specifies the new blood group system Langereis. Nat Genet, 44 (2): 170-3. [PMID:22246506]

10. Hirschfield GM, Chapman RW, Karlsen TH, Lammert F, Lazaridis KN, Mason AL. (2013) The genetics of complex cholestatic disorders. Gastroenterology, 144 (7): 1357-74. [PMID:23583734]

11. Ichikawa Y, Bayeva M, Ghanefar M, Potini V, Sun L, Mutharasan RK, Wu R, Khechaduri A, Jairaj Naik T, Ardehali H. (2012) Disruption of ATP-binding cassette B8 in mice leads to cardiomyopathy through a decrease in mitochondrial iron export. Proc Natl Acad Sci USA, 109 (11): 4152-7. [PMID:22375032]

12. Krishnamurthy PC, Du G, Fukuda Y, Sun D, Sampath J, Mercer KE, Wang J, Sosa-Pineda B, Murti KG, Schuetz JD. (2006) Identification of a mammalian mitochondrial porphyrin transporter. Nature, 443 (7111): 586-9. [PMID:17006453]

13. Ksander BR, Kolovou PE, Wilson BJ, Saab KR, Guo Q, Ma J, McGuire SP, Gregory MS, Vincent WJ, Perez VL et al.. (2014) ABCB5 is a limbal stem cell gene required for corneal development and repair. Nature, 511 (7509): 353-7. [PMID:25030174]

14. Morgan RE, Trauner M, van Staden CJ, Lee PH, Ramachandran B, Eschenberg M, Afshari CA, Qualls Jr CW, Lightfoot-Dunn R, Hamadeh HK. (2010) Interference with bile salt export pump function is a susceptibility factor for human liver injury in drug development. Toxicol Sci, 118 (2): 485-500. [PMID:20829430]

15. Oude Elferink RP, Paulusma CC. (2007) Function and pathophysiological importance of ABCB4 (MDR3 P-glycoprotein). Pflugers Arch, 453 (5): 601-10. [PMID:16622704]

16. Pondarre C, Campagna DR, Antiochos B, Sikorski L, Mulhern H, Fleming MD. (2007) Abcb7, the gene responsible for X-linked sideroblastic anemia with ataxia, is essential for hematopoiesis. Blood, 109 (8): 3567-9. [PMID:17192398]

17. Pondarré C, Antiochos BB, Campagna DR, Clarke SL, Greer EL, Deck KM, McDonald A, Han AP, Medlock A, Kutok JL et al.. (2006) The mitochondrial ATP-binding cassette transporter Abcb7 is essential in mice and participates in cytosolic iron-sulfur cluster biogenesis. Hum Mol Genet, 15 (6): 953-64. [PMID:16467350]

18. Seyffer F, Tampé R. (2015) ABC transporters in adaptive immunity. Biochim Biophys Acta, 1850 (3): 449-60. [PMID:24923865]

19. Shintre CA, Pike AC, Li Q, Kim JI, Barr AJ, Goubin S, Shrestha L, Yang J, Berridge G, Ross J et al.. (2013) Structures of ABCB10, a human ATP-binding cassette transporter in apo- and nucleotide-bound states. Proc Natl Acad Sci USA, 110 (24): 9710-5. [PMID:23716676]

20. Stieger B. (2009) Recent insights into the function and regulation of the bile salt export pump (ABCB11). Curr Opin Lipidol, 20 (3): 176-81. [PMID:19684528]

21. Tang L, Bergevoet SM, Bakker-Verweij G, Harteveld CL, Giordano PC, Nijtmans L, de Witte T, Jansen JH, Raymakers RA, van der Reijden BA. (2012) Human mitochondrial ATP-binding cassette transporter ABCB10 is required for efficient red blood cell development. Br J Haematol, 157 (1): 151-4. [PMID:22085049]

22. Wilson BJ, Saab KR, Ma J, Schatton T, Pütz P, Zhan Q, Murphy GF, Gasser M, Waaga-Gasser AM, Frank NY et al.. (2014) ABCB5 maintains melanoma-initiating cells through a proinflammatory cytokine signaling circuit. Cancer Res, 74 (15): 4196-207. [PMID:24934811]

NC-IUPHAR subcommittee and family contributors

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Concise Guide to PHARMACOLOGY citation:

Alexander SPH, Fabbro D, Kelly E, Mathie AA, Peters JA, Veale EL, Armstrong JF, Faccenda E, Harding SD, Davies JA et al. (2023) The Concise Guide to PHARMACOLOGY 2023/24: Transporters. Br J Pharmacol. 180 Suppl 2:S374-469.

ABCB subfamily C

Overview

Transporters

References

NC-IUPHAR subcommittee and family contributors

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