ABCB11

Summary

Gene Symbol: ABCB11
Description: ATP binding cassette subfamily B member 11
Alias: ABC16, BRIC2, BSEP, PFIC-2, PFIC2, PGY4, SPGP, bile salt export pump, ABC member 16, MDR/TAP subfamily, ATP-binding cassette sub-family B member 11, ATP-binding cassette, sub-family B (MDR/TAP), member 11, progressive familial intrahepatic cholestasis 2, sister p-glycoprotein
Species: human
Products:     ABCB11

Top Publications

  1. Kubitz R, Brinkmeyer C, Sagir A, Herebian D, Haussinger D. Genetic variants of the bile salt export pump: inducers and modifiers of liver diseases. Dig Dis. 2011;29:89-92 pubmed publisher
    The bile salt export pump (BSEP, ABCB11) is the major transporter protein for the excretion of bile salts into bile...
  2. Liu L, Wang Z, Wang X, Zhu Q, Wang J. ABCB11 gene mutations in Chinese children with progressive intrahepatic cholestasis and low gamma glutamyltransferase. Liver Int. 2010;30:809-15 pubmed publisher
    ..b>ABCB11 gene mutations causing PFIC2 have been reported in some population groups, but not in mainland Chinese.
  3. Takeyama Y, Kanegae K, Inomata S, Takata K, Tanaka T, Ueda S, et al. Sustained upregulation of sodium taurocholate cotransporting polypeptide and bile salt export pump and downregulation of cholesterol 7?-hydroxylase in the liver of patients with end-stage primary biliary cirrhosis. Med Mol Morphol. 2010;43:134-8 pubmed publisher
    ..The mRNA expression levels of sodium taurocholate cotransporting polypeptide (NTCP), bile salt export pump (BSEP), and hepatic cholesterol 7?-hydroxylase (CYP7A1) were significantly higher in the PBC patients than ..
  4. Lang C, Meier Y, Stieger B, Beuers U, Lang T, Kerb R, et al. Mutations and polymorphisms in the bile salt export pump and the multidrug resistance protein 3 associated with drug-induced liver injury. Pharmacogenet Genomics. 2007;17:47-60 pubmed
    ..suggests that a genetically determined functional impairment of the hepatocellular efflux transporters bile salt export pump (BSEP, ABCB11) and multidrug resistance protein 3 (MDR3, ABCB4) play a pathophysiological role in the ..
  5. Thompson R, Strautnieks S. BSEP: function and role in progressive familial intrahepatic cholestasis. Semin Liver Dis. 2001;21:545-50 pubmed
    ..The recently described adenosine triphosphate (ATP)-dependent bile acid transporter, bile salt export pump (BSEP), formerly called sister of p-glycoprotein, is responsible for active transport of bile acids across ..
  6. Chen W, Erdos M, Jackson A, Saxena R, Sanna S, Silver K, et al. Variations in the G6PC2/ABCB11 genomic region are associated with fasting glucose levels. J Clin Invest. 2008;118:2620-8 pubmed publisher
    ..glucose-6-phosphatase catalytic subunit 2 (G6PC2) and ATP-binding cassette, subfamily B (MDR/TAP), member 11 (ABCB11)...
  7. Ho R, Leake B, Kilkenny D, Meyer zu Schwabedissen H, Glaeser H, Kroetz D, et al. Polymorphic variants in the human bile salt export pump (BSEP; ABCB11): functional characterization and interindividual variability. Pharmacogenet Genomics. 2010;20:45-57 pubmed publisher
    ..to identify and functionally characterize coding region nonsynonymous single nucleotide polymorphisms in the hepatic efflux transporter, bile salt export pump (BSEP; ABCB11), and to assess interindividual variability in BSEP expression.
  8. Pauli Magnus C, Lang T, Meier Y, Zodan Marin T, Jung D, Breymann C, et al. Sequence analysis of bile salt export pump (ABCB11) and multidrug resistance p-glycoprotein 3 (ABCB4, MDR3) in patients with intrahepatic cholestasis of pregnancy. Pharmacogenetics. 2004;14:91-102 pubmed
    ..There is increasing evidence that genetically determined dysfunction in the canalicular ABC transporters bile salt export pump (BSEP, ABCB11) and multidrug resistance protein 3 (MDR3, ABCB4) might be risk factors for ICP development...
  9. Anzivino C, Odoardi M, Meschiari E, Baldelli E, Facchinetti F, Neri I, et al. ABCB4 and ABCB11 mutations in intrahepatic cholestasis of pregnancy in an Italian population. Dig Liver Dis. 2013;45:226-32 pubmed publisher
    Genetic alterations in the ATP-binding cassette subfamily B member 4 (ABCB4) and ATP-binding cassette subfamily B member 11 (ABCB11) have been associated to the onset of intrahepatic cholestasis of pregnancy (ICP) in predisposed women.

More Information

Publications89

  1. Keitel V, Vogt C, Haussinger D, Kubitz R. Combined mutations of canalicular transporter proteins cause severe intrahepatic cholestasis of pregnancy. Gastroenterology. 2006;131:624-9 pubmed
    ..is evidence that mutations in the canalicular ABC transporter phospholipid flippase (MDR3) and in the bile salt export pump (BSEP) can predispose for the development of ICP...
  2. Lang T, Haberl M, Jung D, Drescher A, Schlagenhaufer R, Keil A, et al. Genetic variability, haplotype structures, and ethnic diversity of hepatic transporters MDR3 (ABCB4) and bile salt export pump (ABCB11). Drug Metab Dispos. 2006;34:1582-99 pubmed
    ..Mutations in ABCB4 and ABCB11 contribute to cholestatic diseases [e.g., progressive familial intrahepatic cholestasis 2 (PFIC2), PFIC3, and intrahepatic cholestasis of pregnancy], and our objective was to ..
  3. van Mil S, van der Woerd W, van der Brugge G, Sturm E, Jansen P, Bull L, et al. Benign recurrent intrahepatic cholestasis type 2 is caused by mutations in ABCB11. Gastroenterology. 2004;127:379-84 pubmed
    ..We hypothesized that a genetically distinct form of BRIC is associated with mutations in ABCB11. This gene encodes the bile salt export pump (BSEP) and is mutated in PFIC type 2.
  4. Pauli Magnus C, Kerb R, Fattinger K, Lang T, Anwald B, Kullak Ublick G, et al. BSEP and MDR3 haplotype structure in healthy Caucasians, primary biliary cirrhosis and primary sclerosing cholangitis. Hepatology. 2004;39:779-91 pubmed
    ..liver damage, also observed in hereditary or acquired dysfunction of the canalicular membrane transporters bile salt export pump (BSEP, ABCB11) and multidrug resistance protein type 3 (MDR3, ABCB4)...
  5. Saito S, Iida A, Sekine A, Miura Y, Ogawa C, Kawauchi S, et al. Three hundred twenty-six genetic variations in genes encoding nine members of ATP-binding cassette, subfamily B (ABCB/MDR/TAP), in the Japanese population. J Hum Genet. 2002;47:38-50 pubmed
    ..ABCB1 gene, 14 in TAP], 35 in TAP2, 48 in ABCB4, 13 in ABCB7, 21 in ABCB8, 21 in ABCB9, 13 in ABCB10, and 82 in ABCB11. Thirteen were located in 5' flanking regions, 237 in introns, 37 in exons, and 10 in 3' flanking regions...
  6. Kim S, Saito Y, Itoda M, Maekawa K, Kawamoto M, Kamatani N, et al. Genetic variations of the ABC transporter gene ABCB11 encoding the human bile salt export pump (BSEP) in a Japanese population. Drug Metab Pharmacokinet. 2009;24:277-81 pubmed
    The bile salt export pump (BSEP) encoded by ABCB11 is located in the canalicular membrane of hepatocytes and mediates the secretion of numerous conjugated bile salts into the bile canaliculus...
  7. Dixon P, van Mil S, Chambers J, Strautnieks S, Thompson R, Lammert F, et al. Contribution of variant alleles of ABCB11 to susceptibility to intrahepatic cholestasis of pregnancy. Gut. 2009;58:537-44 pubmed publisher
    ..ABCB11 encodes the bile salt export pump (BSEP); mutations cause a spectrum of cholestatic disease, and are implicated in the aetiology of ICP.
  8. Liu L, Wang X, Lu Y, Zhu Q, Wang J. Association of variants of ABCB11 with transient neonatal cholestasis. Pediatr Int. 2013;55:138-44 pubmed publisher
    The significance of ABCB11 variants have been studied in some cholestatic diseases, but this is not clear in transient neonatal cholestasis (TNC). The aim of the present study was to explore the association between ABCB11 variants and TNC.
  9. Iwata R, Baur K, Stieger B, Mertens J, Daly A, Frei P, et al. A common polymorphism in the ABCB11 gene is associated with advanced fibrosis in hepatitis C but not in non-alcoholic fatty liver disease. Clin Sci (Lond). 2011;120:287-96 pubmed publisher
    ..A total of 206 subjects with chronic HCV infection were included for ABCB11 (ATP-binding cassette, subfamily B, member II) 1331T>C and NR1H4 (nuclear receptor) -1G>T genotyping, 178 of ..
  10. Evason K, Bove K, Finegold M, Knisely A, Rhee S, Rosenthal P, et al. Morphologic findings in progressive familial intrahepatic cholestasis 2 (PFIC2): correlation with genetic and immunohistochemical studies. Am J Surg Pathol. 2011;35:687-96 pubmed publisher
    ..by cholestasis in infancy that may progress to cirrhosis, is caused by mutation in ABCB11, which encodes bile salt export pump (BSEP)...
  11. Byrne J, Strautnieks S, Ihrke G, Pagani F, Knisely A, Linton K, et al. Missense mutations and single nucleotide polymorphisms in ABCB11 impair bile salt export pump processing and function or disrupt pre-messenger RNA splicing. Hepatology. 2009;49:553-67 pubmed publisher
    The gene encoding the human bile salt export pump (BSEP), ABCB11, is mutated in several forms of intrahepatic cholestasis...
  12. Strautnieks S, Bull L, Knisely A, Kocoshis S, Dahl N, Arnell H, et al. A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis. Nat Genet. 1998;20:233-8 pubmed
    ..The product of the orthologous rat gene has been shown to be an effective bile acid transporter in vitro. These data provide evidence that SPGP is the human bile salt export pump (BSEP).
  13. Meier Y, Zodan T, Lang C, Zimmermann R, Kullak Ublick G, Meier P, et al. Increased susceptibility for intrahepatic cholestasis of pregnancy and contraceptive-induced cholestasis in carriers of the 1331T>C polymorphism in the bile salt export pump. World J Gastroenterol. 2008;14:38-45 pubmed
    To study the association of three common ABCB11 and ABCC2 polymorphisms (ABCB11: 1331T>C --> V444A; ABCC2: 3563T>A --> V1188E and 4544G>A --> C1515Y) with intrahepatic cholestasis of pregnancy (ICP) and contraceptive-..
  14. Chen H, Liu Y, Su Y, Wang N, Wu S, Ni Y, et al. Diagnosis of BSEP/ABCB11 mutations in Asian patients with cholestasis using denaturing high performance liquid chromatography. J Pediatr. 2008;153:825-32 pubmed publisher
    To determine if specific mutations were present in Asian patients with progressive familial intrahepatic cholestasis (PFIC) type 2 caused by defects in bile salt export pump (BSEP), encoded by ABCB11.
  15. Gerloff T, Stieger B, Hagenbuch B, Madon J, Landmann L, Roth J, et al. The sister of P-glycoprotein represents the canalicular bile salt export pump of mammalian liver. J Biol Chem. 1998;273:10046-50 pubmed
    ..These results indicate that the sister of P-glycoprotein is the major canalicular bile salt export pump of mammalian liver.
  16. Mullenbach R, Weber S, Krawczyk M, Zimmer V, Sarrazin C, Lammert F, et al. A frequent variant in the human bile salt export pump gene ABCB11 is associated with hepatitis C virus infection, but not liver stiffness in a German population. BMC Gastroenterol. 2012;12:63 pubmed
    The human ATP-binding cassette, subfamily B, member 11 (ABCB11) gene encodes the bile salt export pump, which is exclusively expressed at the canalicular membrane of hepatocytes. A frequent variant in the coding region, c...
  17. Iwata R, Stieger B, Mertens J, Muller T, Baur K, Frei P, et al. The role of bile acid retention and a common polymorphism in the ABCB11 gene as host factors affecting antiviral treatment response in chronic hepatitis C. J Viral Hepat. 2011;18:768-78 pubmed publisher
    ..We investigate the influence of plasma BA concentrations and an ABCB11 polymorphism associated with lower transporter expression on viral load and SVR...
  18. Eloranta M, Hakli T, Hiltunen M, Helisalmi S, Punnonen K, Heinonen S. Association of single nucleotide polymorphisms of the bile salt export pump gene with intrahepatic cholestasis of pregnancy. Scand J Gastroenterol. 2003;38:648-52 pubmed
    We determined whether genetic variability in the gene encoding the bile salt export pump (BSEP) contributes to individual differences in susceptibility to the development of intrahepatic cholestasis of pregnancy (ICP).
  19. Acalovschi M, Tirziu S, Chiorean E, Krawczyk M, Grünhage F, Lammert F. Common variants of ABCB4 and ABCB11 and plasma lipid levels: a study in sib pairs with gallstones, and controls. Lipids. 2009;44:521-6 pubmed publisher
    ..to analyze the relationship between plasma lipid levels and common polymorphisms of ABCB11 (encoding the bile salt export pump, BSEP) and ABCB4 (encoding the phospholipid transporter into bile, MDR3) genes...
  20. Jansen P, Strautnieks S, Jacquemin E, Hadchouel M, Sokal E, Hooiveld G, et al. Hepatocanalicular bile salt export pump deficiency in patients with progressive familial intrahepatic cholestasis. Gastroenterology. 1999;117:1370-9 pubmed
    ..We investigated expression of the bile salt export pump (BSEP) in liver samples from patients with a PFIC phenotype and correlated this with BSEP gene mutations.
  21. Chen H, Chang P, Hsu H, Ni Y, Hsu H, Lee J, et al. FIC1 and BSEP defects in Taiwanese patients with chronic intrahepatic cholestasis with low gamma-glutamyltranspeptidase levels. J Pediatr. 2002;140:119-24 pubmed
    To elucidate the frequency of FIC1 (ATP8B1) and BSEP (ABCB11) mutations in Taiwanese children with chronic intrahepatic cholestasis with low gamma-glutamyltranspeptidase (GGT) levels, we assessed 13 unrelated patients with infantile onset ..
  22. Strautnieks S, Byrne J, Pawlikowska L, Cebecauerova D, Rayner A, Dutton L, et al. Severe bile salt export pump deficiency: 82 different ABCB11 mutations in 109 families. Gastroenterology. 2008;134:1203-14 pubmed publisher
    Patients with severe bile salt export pump (BSEP) deficiency present as infants with progressive cholestatic liver disease...
  23. Matte U, Mourya R, Miethke A, Liu C, Kauffmann G, Moyer K, et al. Analysis of gene mutations in children with cholestasis of undefined etiology. J Pediatr Gastroenterol Nutr. 2010;51:488-493 pubmed publisher
    ..Here, we aimed to determine whether mutation screening of target genes could assign a molecular diagnosis in children with idiopathic cholestasis...
  24. Zinchuk V, Zinchuk O, Akimaru K, Moriya F, Okada T. Ethanol consumption alters expression and colocalization of bile salt export pump and multidrug resistance protein 2 in the rat. Histochem Cell Biol. 2007;127:503-12 pubmed
    ..regimen, we investigated the effects of chronic ethanol consumption on the expression and localization of bile salt export pump (Bsep), a major canalicular exporter of bile salts, and multidrug resistance protein 2 (Mrp2), a ..
  25. Kawase A, Handa A, Iwaki M. Effects of High-cholesterol Diet on Pravastatin Disposition in the Perfused Rat Liver. Eur J Drug Metab Pharmacokinet. 2017;42:519-526 pubmed publisher
    ..organic anion-transporting polypeptide (Oatp), multidrug resistance-associated protein (Mrp), and bile salt export pump (Bsep). In situ perfused rat liver system was performed...
  26. Zimmer V, Krawczyk M, Mahler M, Weber S, Mullenbach R, Lammert F. Severe hepatocellular dysfunction in obstetric cholestasis related to combined genetic variation in hepatobiliary transporters. Clin Exp Obstet Gynecol. 2012;39:32-5 pubmed
    ..background including variation in diverse hepatobiliary lipid transporters, such as ABCB4 (phospholipids) and ABCB11 (bile salts)...
  27. Kong Y, Gao X, Wang C, Ning C, Liu K, Liu Z, et al. Protective effects of yangonin from an edible botanical Kava against lithocholic acid-induced cholestasis and hepatotoxicity. Eur J Pharmacol. 2018;824:64-71 pubmed publisher
    ..indicated that yangonin promoted bile acid efflux and reduced hepatic uptake via an induction in FXR-target genes Bsep, Mrp2 expression and an inhibition in Ntcp, all of which are responsible for bile acid transport...
  28. Costa J, Reis Henriques M, Castro L, Ferreira M. Gene expression analysis of ABC efflux transporters, CYP1A and GST? in Nile tilapia after exposure to benzo(a)pyrene. Comp Biochem Physiol C Toxicol Pharmacol. 2012;155:469-82 pubmed publisher
    ..Partial mRNA sequences of ABC transporters (ABCB1b, ABCB11, ABCC1, ABCC2 and ABCG2) were identified, and their tissue distribution patterns evaluated in liver, gill and ..
  29. Jemnitz K, Veres Z, Vereczkey L. Contribution of high basolateral bile salt efflux to the lack of hepatotoxicity in rat in response to drugs inducing cholestasis in human. Toxicol Sci. 2010;115:80-8 pubmed publisher
    Intrahepatic bile acid accumulation due to inhibition of the bile salt export pump (BSEP) has been proposed as a mechanism for drug-induced cholestasis...
  30. Wendum D. [Liver disease associated with hereditary defects of hepatobiliary transporters]. Ann Pathol. 2010;30:426-31 pubmed publisher
    ..Then, some liver diseases related to mutations of biliary tranporters (FIC1/ATP8B1, BSEP/ABCB11, MDR3 /ABCB4 and MRP2/ABCC2) will be described with a focus on the pathological aspects...
  31. Fattinger K, Funk C, Pantze M, Weber C, Reichen J, Stieger B, et al. The endothelin antagonist bosentan inhibits the canalicular bile salt export pump: a potential mechanism for hepatic adverse reactions. Clin Pharmacol Ther. 2001;69:223-31 pubmed
    ..In this study we investigated whether inhibition of the hepatocanalicular bile salt export pump (rodents, Bsep; humans, BSEP ABCB11) could account for bosentan-induced liver injury.
  32. Miszczuk G, Barosso I, Larocca M, Marrone J, Marinelli R, Boaglio A, et al. Mechanisms of canalicular transporter endocytosis in the cholestatic rat liver. Biochim Biophys Acta Mol Basis Dis. 2018;1864:1072-1085 pubmed publisher
    ..canalicular secretion due to increased endocytosis and intracellular retention of canalicular transporters such as BSEP and MRP2 is a main, common pathomechanism of cholestasis...
  33. Toledo F, Basiglio C, Barosso I, Boaglio A, Zucchetti A, Sánchez Pozzi E, et al. Mitogen-activated protein kinases are involved in hepatocanalicular dysfunction and cholestasis induced by oxidative stress. Arch Toxicol. 2017;91:2391-2403 pubmed publisher
    ..disorganization followed by endocytic internalization of canalicular transporters relevant to bile formation (Mrp2, Bsep)...
  34. Matulewicz R, Odell D, Chung J, Ban K, Yang A, Bilimoria K. Association Between Resident Perceptions of Patient Safety and Duty Hour Violations. J Am Coll Surg. 2017;224:113-117.e4 pubmed publisher
    ..This negative perception increased with PGY level (PGY1: 18.5%, PGY2 to 3: 22.6%, PGY4 to 5: 32.0%; p < 0.001)...
  35. Shibata S, Hayakawa K, Egashira Y, Sanada H. Hypocholesterolemic mechanism of Chlorella: Chlorella and its indigestible fraction enhance hepatic cholesterol catabolism through up-regulation of cholesterol 7alpha-hydroxylase in rats. Biosci Biotechnol Biochem. 2007;71:916-25 pubmed
    ..low-density lipoprotein (LDL) receptor, scavenger receptor class B1, ATP-binding cassette (ABC) A1, ABCG5, or ABCB11. Furthermore, the effect of a 3...
  36. Donner M, Topp S, Cebula P, Krienen A, Gehrmann T, Sommerfeld A, et al. HbG200-mediated preinduction of heme oxygenase-1 improves bile flow and ameliorates pericentral downregulation of Bsep and Mrp2 following experimental liver ischemia and reperfusion. Biol Chem. 2013;394:97-112 pubmed publisher
    ..05) and biliary taurocholate excretion from 45.9±13.81 ?mol/3 h to 1.87±0.46 ?mol/3 h (p<0.05). Mrp2, Bsep and Ntcp peak immunofluorescence in pericentral hepatocytes decreased to 79.0±2.6% (p<0.001), 80.6±8...
  37. Knisely A. Progressive familial intrahepatic cholestasis: a personal perspective. Pediatr Dev Pathol. 2000;3:113-25 pubmed
    ..familial intrahepatic cholestasis, type 1), and PFIC-2, resulting from mutations in a gene called BSEP (bile salt export pump). Other forms of PFIC may yet be identified...
  38. Qu X, Tao L, Zhang S, Sun J, Niu J, Ding Y, et al. The role of Ntcp, Oatp2, Bsep and Mrp2 in liver injury induced by Dioscorea bulbifera L. and Diosbulbin B in mice. Environ Toxicol Pharmacol. 2017;51:16-22 pubmed publisher
  39. Andress E, Nicolaou M, McGeoghan F, Linton K. ABCB4 missense mutations D243A, K435T, G535D, I490T, R545C, and S978P significantly impair the lipid floppase and likely predispose to secondary pathologies in the human population. Cell Mol Life Sci. 2017;74:2513-2524 pubmed publisher
    ..They are synthesised in hepatocytes and secreted into the luminal space of the biliary tree by the bile salt export pump (BSEP), an ATP-binding cassette (ABC) transporter in the canalicular membrane...
  40. Xi L, Yao J, Wei Y, Wu X, Yao X, Liu H, et al. The in silico identification of human bile salt export pump (ABCB11) inhibitors associated with cholestatic drug-induced liver injury. Mol Biosyst. 2017;13:417-424 pubmed publisher
    ..Currently, there is increasing evidence that direct inhibition of the human bile salt export pump (BSEP/ABCB11) by drugs and/or metabolites is one of the most important mechanisms of cholestatic DILI...
  41. Nagasaka H, Yorifuji T, Kobayashi K, Takikawa H, Komatsu H, Inui A, et al. Favorable effect of 4-phenylacetate on liver functions attributable to enhanced bile salt export pump expression in ornithine transcarbamylase-deficient children. Mol Genet Metab. 2010;100:123-8 pubmed publisher
    ..experiments using animals and cultured cells strongly suggest that this agent enhances the function of bile salt export pump/ATP binding cassette B11 (BSEP/ABCB11) promoting bile acid excretion from hepatocytes to bile canaliculi, ..
  42. Tong Z, Yerramilli U, Surapaneni S, Kumar G. The interactions of lenalidomide with human uptake and efflux transporters and UDP-glucuronosyltransferase 1A1: lack of potential for drug-drug interactions. Cancer Chemother Pharmacol. 2014;73:869-74 pubmed publisher
    ..was also evaluated as an inhibitor of P-gp, BCRP, MRP2, OCT2, OAT1, OAT3, OATP1B1, OATP1B3 and bile salt export pump (BSEP)...
  43. Imagawa K, Hayashi H, Sabu Y, Tanikawa K, Fujishiro J, Kajikawa D, et al. Clinical phenotype and molecular analysis of a homozygous ABCB11 mutation responsible for progressive infantile cholestasis. J Hum Genet. 2018;63:569-577 pubmed publisher
    The bile salt export pump (BSEP) plays an important role in biliary secretion...
  44. El Guindi M, Sira M, Hussein M, Ehsan N, Elsheikh N.  Hepatic immunohistochemistry of bile transporters in progressive familial intrahepatic cholestasis. Ann Hepatol. 2016;15:222-9 pubmed publisher
    ..The aim of this study was to evaluate liver tissue immunohistochemistry of bile salt export pump (BSEP) and multidrug resistance 3 (MDR3) proteins in differentiating PFIC from other causes of neonatal ..
  45. Okushin K, Tsutsumi T, Enooku K, Fujinaga H, Kado A, Shibahara J, et al. The intrahepatic expression levels of bile acid transporters are inversely correlated with the histological progression of nonalcoholic fatty liver disease. J Gastroenterol. 2016;51:808-18 pubmed publisher
    ..Notably, the expression levels of a main export transporter, bile salt export pump (BSEP), significantly decreased as the NAS and the each NAS component increased in both genders...
  46. Wang L, Dong H, Soroka C, Wei N, Boyer J, Hochstrasser M. Degradation of the bile salt export pump at endoplasmic reticulum in progressive familial intrahepatic cholestasis type II. Hepatology. 2008;48:1558-69 pubmed publisher
    The bile salt export pump (Bsep) represents the major bile salt transport system at the canalicular membrane of hepatocytes...
  47. Knisely A, Strautnieks S, Meier Y, Stieger B, Byrne J, Portmann B, et al. Hepatocellular carcinoma in ten children under five years of age with bile salt export pump deficiency. Hepatology. 2006;44:478-86 pubmed
    ..and mutational-analysis studies could demonstrate that deficiency of the canalicular bile acid transporter bile salt export pump (BSEP) and mutation in ABCB11, encoding BSEP, underlay progressive familial intrahepatic cholestasis (PFIC)..
  48. Jouan E, Le Vee M, Denizot C, Parmentier Y, Fardel O. Drug Transporter Expression and Activity in Human Hepatoma HuH-7 Cells. Pharmaceutics. 2016;9: pubmed publisher
    ..hepatic drug transporters were not detected (NTCP, OATP1B1, organic anion transporter 2 (OAT2), OCT1 and bile salt export pump) or were found to be lower (OATP1B3, OATP2B1, multidrug and toxin extrusion protein 1, BCRP and MRP3) in ..
  49. Kawase A, Yamada A, Gamou Y, Tahara C, Takeshita F, Murata K, et al. Increased effects of ginsenosides on the expression of cholesterol 7?-hydroxylase but not the bile salt export pump are involved in cholesterol metabolism. J Nat Med. 2013;67:545-53 pubmed publisher
    ..the mRNA expression of cholesterol metabolism-related proteins such as cytochrome P450 (CYP)7A1 and bile salt export pump (BSEP) in the liver in hypercholesterolemic rats and rat primary hepatocytes...
  50. Wree A, Canbay A, Müller Beissenhirtz H, Dechene A, Gerken G, Duhrsen U, et al. Excessive bilirubin elevation in a patient with hereditary spherocytosis and intrahepatic cholestasis. Z Gastroenterol. 2011;49:977-80 pubmed publisher
    ..Mutations in the ABCB11 gene, encoding the bile salt export pump, can entail progressive familial intrahepatic cholestasis and benign recurred intrahepatic cholestasis...
  51. Poupon R, Barbu V, Chamouard P, Wendum D, Rosmorduc O, Housset C. Combined features of low phospholipid-associated cholelithiasis and progressive familial intrahepatic cholestasis 3. Liver Int. 2010;30:327-31 pubmed publisher
    ..Sequence analysis of ABCB11, potentially involved in similar symptoms, was also performed...
  52. Kagawa T, Watanabe N, Mochizuki K, Numari A, Ikeno Y, Itoh J, et al. Phenotypic differences in PFIC2 and BRIC2 correlate with protein stability of mutant Bsep and impaired taurocholate secretion in MDCK II cells. Am J Physiol Gastrointest Liver Physiol. 2008;294:G58-67 pubmed
    ..cholestasis (PFIC) 2 and benign recurrent intrahepatic cholestasis (BRIC) 2 are caused by mutations in the bile salt export pump (BSEP, ABCB11) gene; however, their prognosis differs...
  53. Albrecht C, Elliott J, Sardini A, Litman T, Stieger B, Meier P, et al. Functional analysis of candidate ABC transporter proteins for sitosterol transport. Biochim Biophys Acta. 2002;1567:133-42 pubmed
    ..resistance-associated protein (Mrp1; Abcc1), the breast cancer resistance protein (Bcrp; Abcg2) and the bile salt export pump (Bsep; Abcb11) was assessed using several assays...
  54. Shimizu H, Migita O, Kosaki R, Kasahara M, Fukuda A, Sakamoto S, et al. Living-related liver transplantation for siblings with progressive familial intrahepatic cholestasis 2, with novel genetic findings. Am J Transplant. 2011;11:394-8 pubmed publisher
    ..Genetic testing of each patient revealed two missense mutations of the bile salt export pump, S901R and C1083Y, which have not previously been associated with PFIC-2...
  55. van Beusekom C, van den Heuvel J, Koenderink J, Schrickx J, Russel F. The feline bile salt export pump: a structural and functional comparison with canine and human Bsep/BSEP. BMC Vet Res. 2013;9:259 pubmed publisher
    The bile salt export pump (BSEP/ABCB11) is the primary transporter for the excretion of bile acids from hepatocytes into bile...
  56. Aguilar Olivos N, Carrillo Córdova D, Oria Hernández J, Sánchez Valle V, Ponciano Rodríguez G, Ramírez Jaramillo M, et al. The nuclear receptor FXR, but not LXR, up-regulates bile acid transporter expression in non-alcoholic fatty liver disease. Ann Hepatol. 2015;14:487-93 pubmed
    ..alpha (LXRα) receptors- and BA transporters -sodium+/taurocholate cotransporting polypeptide (NTCP) and bile salt export pump (BSEP)- in liver biopsy samples of patients with simple steatosis (SS) and NASH...
  57. Stieger B. Recent insights into the function and regulation of the bile salt export pump (ABCB11). Curr Opin Lipidol. 2009;20:176-81 pubmed publisher
    ..Its impairment can be caused by inherited mutations or by acquired factors and leads to cholestasis. Bile salts are an important constituent of bile and are secreted by the bile salt export pump (BSEP) from hepatocytes.
  58. Testa A, Dall Angelo S, Mingarelli M, Augello A, Schweiger L, Welch A, et al. Design, synthesis, in vitro characterization and preliminary imaging studies on fluorinated bile acid derivatives as PET tracers to study hepatic transporters. Bioorg Med Chem. 2017;25:963-976 pubmed publisher
    ..LCATD (4c), was tested as a substrate of other liver uptake transporters OATP1B3, NTCP and efflux transporter BSEP to evaluate its specificity of liver transport...
  59. Hu G, He P, Liu Z, Chen Q, Zheng B, Zhang Q. Diagnosis of ABCB11 gene mutations in children with intrahepatic cholestasis using high resolution melting analysis and direct sequencing. Mol Med Rep. 2014;10:1264-74 pubmed publisher
    ..cholestasis, progressive familial intrahepatic cholestasis type 2 (PFIC2) is caused by pathogenic mutations in the ABCB11 gene, HRM was used to detect mutations in the ABCB11 gene in the present study, and the diagnosis for PFIC2 was ..
  60. Micheline D, Emmanuel J, Serge E. Effect of Ursodeoxycholic Acid on the Expression of the Hepatocellular Bile Acid Transporters (Ntcp and bsep) in Rats With Estrogen-Induced Cholestasis. J Pediatr Gastroenterol Nutr. 2002;35:185-91 pubmed
    ..The bile acid ursodeoxycholic acid improves bile flow in these animals. The purpose of this study was to examine the effect of ursodeoxycholic acid on the expression of hepatocellular bile acid carriers...
  61. Vitale G, Gitto S, Raimondi F, Mattiaccio A, Mantovani V, Vukotic R, et al. Cryptogenic cholestasis in young and adults: ATP8B1, ABCB11, ABCB4, and TJP2 gene variants analysis by high-throughput sequencing. J Gastroenterol. 2018;53:945-958 pubmed publisher
    Mutations in ATP-transporters ATPB81, ABCB11, and ABCB4 are responsible for progressive familial intrahepatic cholestasis (PFIC) 1, 2 and 3, and recently the gene for tight junction protein-2 (TJP2) has been linked to PFIC4...
  62. Lam P, Soroka C, Boyer J. The bile salt export pump: clinical and experimental aspects of genetic and acquired cholestatic liver disease. Semin Liver Dis. 2010;30:125-33 pubmed publisher
    The primary transporter responsible for bile salt secretion is the bile salt export pump (BSEP, ABCB11), a member of the ATP-binding cassette (ABC) superfamily, which is located at the bile canalicular apical domain of hepatocytes...
  63. Han Y, Busler D, Hong Y, Tian Y, Chen C, Rodrigues A. Transporter studies with the 3-O-sulfate conjugate of 17alpha-ethinylestradiol: assessment of human liver drug transporters. Drug Metab Dispos. 2010;38:1072-82 pubmed publisher
    ..EE2-Sul was also assessed as an efflux transporter substrate using membrane vesicles expressing bile salt export pump, breast cancer resistance protein (BCRP), and individual forms of multidrug resistance-associated protein (..
  64. Zhang Y, Lickteig A, Csanaky I, Klaassen C. Clofibrate decreases bile acids in livers of male mice by increasing biliary bile acid excretion in a PPAR?-dependent manner. Toxicol Sci. 2017;: pubmed publisher
    ..biliary excretion of BAs, which was associated with a significant induction of the canalicular efflux transporter (Bsep) in the liver...
  65. Lam P, Wang R, Ling V. Bile acid transport in sister of P-glycoprotein (ABCB11) knockout mice. Biochemistry. 2005;44:12598-605 pubmed
    ..of the ATP binding cassette transporter (ABC) superfamily, known as sister of P-glycoprotein (Spgp) or bile salt export pump (Bsep, ABCB11)...
  66. Stieger B, Meier Y, Meier P. The bile salt export pump. Pflugers Arch. 2007;453:611-20 pubmed
    Canalicular secretion of bile salts mediated by the bile salt export pump Bsep constitutes the major driving force for the generation of bile flow...
  67. Alrefai W, Gill R. Bile acid transporters: structure, function, regulation and pathophysiological implications. Pharm Res. 2007;24:1803-23 pubmed
    ..bile acid binding protein, bile acids are secreted into the canaliculus via ATP-dependent bile salt excretory pump (BSEP) and multi drug resistant proteins (MRPs)...
  68. Crocenzi F, Basiglio C, Pérez L, Portesio M, Pozzi E, Roma M. Silibinin prevents cholestasis-associated retrieval of the bile salt export pump, Bsep, in isolated rat hepatocyte couplets: possible involvement of cAMP. Biochem Pharmacol. 2005;69:1113-20 pubmed
    ..E(2)17G) and taurolithocholate (TLC) induce acute cholestasis-associated with retrieval of the bile salt export pump (Bsep), which parallels with alteration in transport activity...
  69. Jain A, Sharma A, Arora S, Blomenkamp K, Jun I, Luong R, et al. Preserved Gut Microbial Diversity Accompanies Upregulation of TGR5 and Hepatobiliary Transporters in Bile Acid-Treated Animals Receiving Parenteral Nutrition. JPEN J Parenter Enteral Nutr. 2017;41:198-207 pubmed publisher
    ..BAs also regulate the bile salt export pump (BSEP) involved in cholestasis...
  70. Lam P, Pearson C, Soroka C, Xu S, Mennone A, Boyer J. Levels of plasma membrane expression in progressive and benign mutations of the bile salt export pump (Bsep/Abcb11) correlate with severity of cholestatic diseases. Am J Physiol Cell Physiol. 2007;293:C1709-16 pubmed
    Human BSEP (ABCB11) mutations are the molecular basis for at least three clinical forms of liver disease, progressive familial intrahepatic cholestasis type 2 (PFIC2), benign recurrent intrahepatic cholestasis type 2 (BRIC2), and ..
  71. Zaja R, Munić V, Smital T. Cloning and mRNA expression analysis of an ABCG2 (BCRP) efflux transporter in rainbow trout (Oncorhynchus mykiss) liver and primary hepatocytes. Mar Environ Res. 2008;66:77-9 pubmed publisher
    ..expression levels to the expression of other ABC transporters expressed in apical membrane of hepatocytes (ABCB1, ABCB11 and ABCC2)...
  72. Li T, Matozel M, Boehme S, Kong B, Nilsson L, Guo G, et al. Overexpression of cholesterol 7?-hydroxylase promotes hepatic bile acid synthesis and secretion and maintains cholesterol homeostasis. Hepatology. 2011;53:996-1006 pubmed publisher
    ..adenosine triphosphate-binding cassette G5/G8 [ABCG5/G8], scavenger receptor class B, member 1) and bile acid (ABCB11) transporters were significantly induced in Cyp7a1-tg mice...
  73. Tian J, Zhu J, Yi Y, Li C, Zhang Y, Zhao Y, et al. Dose-related liver injury of Geniposide associated with the alteration in bile acid synthesis and transportation. Sci Rep. 2017;7:8938 pubmed publisher
    ..also down-regulated expression of Farnesoid X receptor (FXR), small heterodimer partner (SHP) and bile salt export pump (BSEP)...
  74. Kosters A, Karpen S. Bile acid transporters in health and disease. Xenobiotica. 2008;38:1043-71 pubmed
    ..polypeptide (NTCP), while secretion across the canalicular membrane into the bile is carried out by the bile salt export pump (BSEP)...
  75. Borlak J, Klutcka T. Expression of basolateral and canalicular transporters in rat liver and cultures of primary hepatocytes. Xenobiotica. 2004;34:935-47 pubmed
    ..transporters in cultures of primary rat sandwiched hepatocytes were investigated, and the expression of cMOAT, spgp, mdrla and mdr2 were shown to be comparable with transcript levels observed in vivo...
  76. Elferink M, Olinga P, Draaisma A, Merema M, Faber K, Slooff M, et al. LPS-induced downregulation of MRP2 and BSEP in human liver is due to a posttranscriptional process. Am J Physiol Gastrointest Liver Physiol. 2004;287:G1008-16 pubmed
    ..transporters, including the basolateral Na+-dependent taurocholate transporter (ntcp) and the canalicular bile salt export pump (bsep) and multidrug resistance-associated protein 2 (mrp2)...
  77. Schneider G, Paus T, Kullak Ublick G, Meier P, Wienker T, Lang T, et al. Linkage between a new splicing site mutation in the MDR3 alias ABCB4 gene and intrahepatic cholestasis of pregnancy. Hepatology. 2007;45:150-8 pubmed
    ..The familial cholestasis type 1 (FIC1, ATP8B1) gene, bile salt export pump (BSEP, ABCB11) and MDR3 gene were analyzed in 55 relatives...
  78. Dawson S, Stahl S, Paul N, Barber J, Kenna J. In vitro inhibition of the bile salt export pump correlates with risk of cholestatic drug-induced liver injury in humans. Drug Metab Dispos. 2012;40:130-8 pubmed publisher
    Inhibition of the activity of the human bile salt export pump (BSEP: ABCB11) has been proposed to play a role in drug-induced liver injury (DILI)...
  79. Meng Q, Chen X, Wang C, Liu Q, Sun H, Sun P, et al. Protective effects of alisol B 23-acetate from edible botanical Rhizoma alismatis against carbon tetrachloride-induced hepatotoxicity in mice. Food Funct. 2015;6:1241-50 pubmed publisher
    ..hepatic uptake transporter Ntcp, bile acid synthetic enzymes Cyp7a1, Cyp8b1, and an increase in efflux transporter Bsep, Mrp2 expression...
  80. Wang R, Sheps J, Ling V. ABC transporters, bile acids, and inflammatory stress in liver cancer. Curr Pharm Biotechnol. 2011;12:636-46 pubmed
    ..b>BSEP provides for efficient secretion of bile acids across the canalicular membrane against a steep concentration ..