Abcb11

Summary

Gene Symbol: Abcb11
Description: ATP-binding cassette, sub-family B (MDR/TAP), member 11
Alias: ABC16, Bsep, Lith1, PFIC2, PGY4, SPGP, bile salt export pump, ATP-binding cassette, sub-family B, member 11, sister of P-glycoprotein
Species: mouse
Products:     Abcb11

Top Publications

  1. Wang R, Chen H, Liu L, Sheps J, Phillips M, Ling V. Compensatory role of P-glycoproteins in knockout mice lacking the bile salt export pump. Hepatology. 2009;50:948-56 pubmed publisher
    b>Bile salt export pump (BSEP; ATP-binding cassette, subfamily B, member 11) mutations in humans result in progressive familial intrahepatic cholestasis type 2, a fatal liver disease with greatly reduced bile flow...
  2. Wang R, Lam P, Liu L, Forrest D, Yousef I, Mignault D, et al. Severe cholestasis induced by cholic acid feeding in knockout mice of sister of P-glycoprotein. Hepatology. 2003;38:1489-99 pubmed
    ..bile acid secretion, a process mediated by the sister of P-glycoprotein (Spgp or Abcb11) also known as the bile salt export pump (Bsep)...
  3. Hrycay E, Forrest D, Liu L, Wang R, Tai J, Deo A, et al. Hepatic bile acid metabolism and expression of cytochrome P450 and related enzymes are altered in Bsep (-/-) mice. Mol Cell Biochem. 2014;389:119-32 pubmed publisher
    The bile salt export pump (BSEP/Bsep; gene symbol ABCB11/Abcb11) translocates bile salts across the hepatocyte canalicular membrane into bile in humans and mice...
  4. Yska M, Buis C, Monbaliu D, Schuurs T, Gouw A, Kahmann O, et al. The role of bile salt toxicity in the pathogenesis of bile duct injury after non-heart-beating porcine liver transplantation. Transplantation. 2008;85:1625-31 pubmed publisher
    ..The role of bile salt toxicity in the development of biliary strictures after NHB liver transplantation is unclear...
  5. Song X, Chen Y, Valanejad L, Kaimal R, Yan B, Stoner M, et al. Mechanistic insights into isoform-dependent and species-specific regulation of bile salt export pump by farnesoid X receptor. J Lipid Res. 2013;54:3030-44 pubmed publisher
    Expression of bile salt export pump (BSEP) is regulated by the bile acid/farnesoid X receptor (FXR) signaling pathway. Two FXR isoforms, FXR?1 and FXR?2, are predominantly expressed in human liver...
  6. Zhang Y, Li F, Patterson A, Wang Y, Krausz K, Neale G, et al. Abcb11 deficiency induces cholestasis coupled to impaired ?-fatty acid oxidation in mice. J Biol Chem. 2012;287:24784-94 pubmed publisher
    The bile salt export pump (BSEP) is an ATP-binding cassette transporter that serves as the primary system for removing bile salts from the liver...
  7. Bouchard G, Nelson H, Lammert F, Rowe L, Carey M, Paigen B. High-resolution maps of the murine Chromosome 2 region containing the cholesterol gallstone locus, Lith1. Mamm Genome. 1999;10:1070-4 pubmed
    ..markers in the D2Mit182 to D2Mit14 region and two candidate genes for Lith1, the canalicular bile salt export pump (Bsep) also known as sister of P-glycoprotein (Spgp) and the low-density-lipoprotein-receptor-related gene ..
  8. Phan J, Pesaran T, Davis R, Reue K. The Diet1 locus confers protection against hypercholesterolemia through enhanced bile acid metabolism. J Biol Chem. 2002;277:469-77 pubmed
    ..Genetic analysis of serum bile acid levels revealed co-segregation with Diet1, indicating that this locus is likely responsible for both increased bile acid excretion and resistance to hypercholesterolemia in B6By mice...
  9. Cheng X, Buckley D, Klaassen C. Regulation of hepatic bile acid transporters Ntcp and Bsep expression. Biochem Pharmacol. 2007;74:1665-76 pubmed
    Sodium-taurocholate cotransporting polypeptide (Ntcp) and bile salt export pump (Bsep) are two key transporters for hepatic bile acid uptake and excretion...

More Information

Publications41

  1. Klett E, Lu K, Kosters A, Vink E, Lee M, Altenburg M, et al. A mouse model of sitosterolemia: absence of Abcg8/sterolin-2 results in failure to secrete biliary cholesterol. BMC Med. 2004;2:5 pubmed
    ..No phenotypic difference has yet been described in humans with complete defects in either ABCG5 or ABCG8. These proteins, based upon the defects in humans, are responsible for regulating dietary sterol entry and biliary sterol secretion...
  2. Green R, Hoda F, Ward K. Molecular cloning and characterization of the murine bile salt export pump. Gene. 2000;241:117-23 pubmed
    ..Recently, the rat sister-of-p-glycoprotein/bile salt export pump (spgp/BSEP) was demonstrated to encode for the rat ATP-dependent canalicular bile salt export protein, and ..
  3. Ito M, Yamanashi Y, Toyoda Y, Izumi Nakaseko H, Oda S, Sugiyama A, et al. Disruption of Stard10 gene alters the PPAR?-mediated bile acid homeostasis. Biochim Biophys Acta. 2013;1831:459-68 pubmed publisher
    ..These results indicate that STARD10 is involved in regulating bile acid metabolism through the modulation of PPAR?-mediated mechanism. ..
  4. 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)...
  5. Wang R, Salem M, Yousef I, Tuchweber B, Lam P, Childs S, et al. Targeted inactivation of sister of P-glycoprotein gene (spgp) in mice results in nonprogressive but persistent intrahepatic cholestasis. Proc Natl Acad Sci U S A. 2001;98:2011-6 pubmed
    Mutations in the sister of P-glycoprotein (Spgp) or bile salt export pump (BSEP) are associated with Progressive Familial Intrahepatic Cholestasis (PFIC2). Spgp is predominantly expressed in the canalicular membranes of liver...
  6. Allen R, Marquart T, Albert C, Suchy F, Wang D, Ananthanarayanan M, et al. miR-33 controls the expression of biliary transporters, and mediates statin- and diet-induced hepatotoxicity. EMBO Mol Med. 2012;4:882-95 pubmed publisher
    ..We show that two of these transporters, ABCB11 and ATP8B1, are functional targets of miR-33, a micro-RNA that is expressed from within an intron of SREBP-2...
  7. Song X, Vasilenko A, Chen Y, Valanejad L, Verma R, Yan B, et al. Transcriptional dynamics of bile salt export pump during pregnancy: mechanisms and implications in intrahepatic cholestasis of pregnancy. Hepatology. 2014;60:1993-2007 pubmed publisher
    b>Bile salt export pump (BSEP) is responsible for biliary secretion of bile acids, a rate-limiting step in the enterohepatic circulation of bile acids and transactivated by nuclear receptor farnesoid X receptor (FXR)...
  8. Paulusma C, de Waart D, Kunne C, Mok K, Elferink R. Activity of the bile salt export pump (ABCB11) is critically dependent on canalicular membrane cholesterol content. J Biol Chem. 2009;284:9947-54 pubmed publisher
    ..cholesterol extraction from the canalicular membrane impairs the activity of the bile salt transporter, ABCB11, and as a consequence causes cholestasis...
  9. Kikuchi S, Hata M, Fukumoto K, Yamane Y, Matsui T, Tamura A, et al. Radixin deficiency causes conjugated hyperbilirubinemia with loss of Mrp2 from bile canalicular membranes. Nat Genet. 2002;31:320-5 pubmed
    ..These findings indicate that radixin is required for secretion of conjugated bilirubin through its support of Mrp2 localization at BCMs...
  10. Homolya L, Fu D, Sengupta P, Jarnik M, Gillet J, Vitale Cross L, et al. LKB1/AMPK and PKA control ABCB11 trafficking and polarization in hepatocytes. PLoS ONE. 2014;9:e91921 pubmed publisher
    ..cultures from control and liver-specific LKB1 knockout mice to examine the role of LKB1 in trafficking of ABCB11, the canalicular bile acid transporter...
  11. Kuo S, Merhige P, Hagey L. The effect of dietary prebiotics and probiotics on body weight, large intestine indices, and fecal bile acid profile in wild type and IL10-/- mice. PLoS ONE. 2013;8:e60270 pubmed publisher
    ..In addition, fecal bile acid profiling could potentially be a non-invasive tool in monitoring the intestinal environment. ..
  12. Shih D, Bussen M, Sehayek E, Ananthanarayanan M, Shneider B, Suchy F, et al. Hepatocyte nuclear factor-1alpha is an essential regulator of bile acid and plasma cholesterol metabolism. Nat Genet. 2001;27:375-82 pubmed
    ..Our studies demonstrate that Tcf1, in addition to being an important regulator of insulin secretion, is an essential transcriptional regulator of bile acid and HDL-cholesterol metabolism. ..
  13. Cantore M, Reinehr R, Sommerfeld A, Becker M, Haussinger D. The Src family kinase Fyn mediates hyperosmolarity-induced Mrp2 and Bsep retrieval from canalicular membrane. J Biol Chem. 2011;286:45014-29 pubmed publisher
    ..D'urso, D., Keppler, D., and Häussinger, D. (1997) Gastroenterology 113, 1438-1442) and Bsep retrieval (Schmitt, M., Kubitz, R., Lizun, S., Wettstein, M., and Häussinger, D...
  14. Wolters H, Elzinga B, Baller J, Boverhof R, Schwarz M, Stieger B, et al. Effects of bile salt flux variations on the expression of hepatic bile salt transporters in vivo in mice. J Hepatol. 2002;37:556-63 pubmed
    ..We investigated the physiological relevance of this regulation by evaluating transporter expression in mice experiencing different transhepatic bile salt fluxes...
  15. Huls M, van den Heuvel J, Dijkman H, Russel F, Masereeuw R. ABC transporter expression profiling after ischemic reperfusion injury in mouse kidney. Kidney Int. 2006;69:2186-93 pubmed
    ..Abcb11 is the major liver canalicular bile salt export pump. Here we show for the first time expression in the kidney and localization of the transporter to the ..
  16. Henkel A, Wei Z, Cohen D, Green R. Mice overexpressing hepatic Abcb11 rapidly develop cholesterol gallstones. Mamm Genome. 2005;16:903-8 pubmed
    ..QTL analysis has identified the Lith 1 loci on mouse Chromosome 2, and the hepatic bile salt transporter Abcb11 maps to the Lith 1 locus...
  17. Chopra A, Kommagani R, Saha P, Louet J, Salazar C, Song J, et al. Cellular energy depletion resets whole-body energy by promoting coactivator-mediated dietary fuel absorption. Cell Metab. 2011;13:35-43 pubmed publisher
    ..can be completely rescued by replenishing intestinal BA or by genetically restoring the levels of hepatic bile salt export pump (BSEP)...
  18. Plass J, Mol O, Heegsma J, Geuken M, de Bruin J, Elling G, et al. A progressive familial intrahepatic cholestasis type 2 mutation causes an unstable, temperature-sensitive bile salt export pump. J Hepatol. 2004;40:24-30 pubmed
    ..The disease is caused by mutations in the bile salt export pump (BSEP). Ten different missense mutations have been described...
  19. Perwaiz S, Forrest D, Mignault D, Tuchweber B, Phillip M, Wang R, et al. Appearance of atypical 3 alpha,6 beta,7 beta,12 alpha-tetrahydroxy-5 beta-cholan-24-oic acid in spgp knockout mice. J Lipid Res. 2003;44:494-502 pubmed
    ..The sister-of-p-glycoprotein (spgp) gene was shown to encode the canalicular bile salt export protein, and mutations in spgp gene were identified as ..
  20. Gooijert K, Havinga R, Wolters H, Wang R, Ling V, Tazuma S, et al. The mechanism of increased biliary lipid secretion in mice with genetic inactivation of bile salt export pump. Am J Physiol Gastrointest Liver Physiol. 2015;308:G450-7 pubmed publisher
    Human bile salt export pump (BSEP) mutations underlie progressive familial intrahepatic cholestasis type 2 (PFIC2)...
  21. Wang R, Liu L, Sheps J, Forrest D, Hofmann A, Hagey L, et al. Defective canalicular transport and toxicity of dietary ursodeoxycholic acid in the abcb11-/- mouse: transport and gene expression studies. Am J Physiol Gastrointest Liver Physiol. 2013;305:G286-94 pubmed publisher
    The bile salt export pump (BSEP), encoded by the abcb11 gene, is the major canalicular transporter of bile acids from the hepatocyte...
  22. Paigen B, Schork N, Svenson K, Cheah Y, Mu J, Lammert F, et al. Quantitative trait loci mapping for cholesterol gallstones in AKR/J and C57L/J strains of mice. Physiol Genomics. 2000;4:59-65 pubmed
    ..The major locus, Lith1, mapped near D2Mit56 and was confirmed by constructing a congenic strain, AK. L-Lith1(s)...
  23. Guo Y, Xu X, Zhang Q, Li C, Deng Y, Jiang P, et al. The inhibition of hepatic bile acids transporters Ntcp and Bsep is involved in the pathogenesis of isoniazid/rifampicin-induced hepatotoxicity. Toxicol Mech Methods. 2015;25:382-7 pubmed publisher
    ..present study investigates whether sodium taurocholate cotransporting polypeptide (NTCP/Ntcp; SLC10A1) and bile salt export pump (BSEP/Bsep; ABCB11) are involved in the anti-tuberculosis medicines induced liver injury...
  24. Henkel A, Kavesh M, Kriss M, Dewey A, Rinella M, Green R. Hepatic overexpression of abcb11 promotes hypercholesterolemia and obesity in mice. Gastroenterology. 2011;141:1404-11, 1411.e1-2 pubmed publisher
    b>ABCB11 is a canalicular transport protein that controls the rate-limiting step in hepatic bile acid secretion. Its expression levels vary in humans, and it is not clear how these variations affect lipid metabolism...
  25. Imai S, Kikuchi R, Kusuhara H, Yagi S, Shiota K, Sugiyama Y. Analysis of DNA methylation and histone modification profiles of liver-specific transporters. Mol Pharmacol. 2009;75:568-76 pubmed publisher
    ..underlies the tissue-specific expression of mouse liver-specific transporters (Oatp1b2/Slco1b2, Ntcp/Slc10a1, Bsep/Abcb11, and Abcg5/g8)...
  26. Figge A, Lammert F, Paigen B, Henkel A, Matern S, Korstanje R, et al. Hepatic overexpression of murine Abcb11 increases hepatobiliary lipid secretion and reduces hepatic steatosis. J Biol Chem. 2004;279:2790-9 pubmed
    Abcb11 encodes for the liver bile salt export pump, which is rate-limiting for hepatobiliary bile salt secretion...
  27. Hoda F, Green R. Hepatic canalicular membrane transport of bile salt in C57L/J and AKR/J mice: implications for cholesterol gallstone formation. J Membr Biol. 2003;196:9-14 pubmed
    ..Abcb11 encodes for the liver canalicular membrane bile salt export pump (BSEP), which maps to this QTL and is a candidate gene for Lith 1...
  28. Vergnes L, Lee J, Chin R, Auwerx J, Reue K. Diet1 functions in the FGF15/19 enterohepatic signaling axis to modulate bile acid and lipid levels. Cell Metab. 2013;17:916-28 pubmed publisher
    ..Diet1 and FGF19 proteins exhibited overlapping subcellular localization in cultured intestinal cells. These results establish Diet1 as a control point in enterohepatic bile acid signaling and lipid homeostasis. ..
  29. Csanaky I, Aleksunes L, Tanaka Y, Klaassen C. Role of hepatic transporters in prevention of bile acid toxicity after partial hepatectomy in mice. Am J Physiol Gastrointest Liver Physiol. 2009;297:G419-33 pubmed publisher
    ..The mRNA and protein levels of the BA uptake transporter Ntcp were unchanged after PHx, whereas the canalicular Bsep protein increased twofold at 48 h. The basolateral efflux transporter Mrp3 was induced at the mRNA (2...
  30. Schriml L, Dean M. Identification of 18 mouse ABC genes and characterization of the ABC superfamily in Mus musculus. Genomics. 2000;64:24-31 pubmed
    ..The mouse ABC genes could be grouped into the seven previously described human ABC subfamilies. Three mouse ABC genes mapped to regions implicated in cholesterol gallstone susceptibility...
  31. Kamiya A, Gonzalez F. TNF-alpha regulates mouse fetal hepatic maturation induced by oncostatin M and extracellular matrices. Hepatology. 2004;40:527-36 pubmed
    ..In conclusion, TNFalpha is a suppressive factor of hepatic maturation. The balance between hepatic maturation factor (OSM and extracellular matrices) and TNFalpha is important for liver development. ..
  32. Henkel A, Gooijert K, Havinga R, Boverhof R, Green R, Verkade H. Hepatic overexpression of Abcb11 in mice promotes the conservation of bile acids within the enterohepatic circulation. Am J Physiol Gastrointest Liver Physiol. 2013;304:G221-6 pubmed publisher
    The bile salt export pump, encoded by ABCB11, is the predominant canalicular transport protein for biliary bile acid secretion...