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The membrane-associated protein encoded by ABCB11 is a member of the superfamily of ATP-binding cassette (ABC) transporters. Additionally we are shipping ABCB11 Kits (12) and ABCB11 Proteins (8) and many more products for this protein.
Showing 10 out of 60 products:
Human Polyclonal ABCB11 Primary Antibody for IHC (p), WB - ABIN390059
Strautnieks, Bull, Knisely, Kocoshis, Dahl, Arnell, Sokal, Dahan, Childs, Ling, Tanner, Kagalwalla, Németh, Pawlowska, Baker, Mieli-Vergani, Freimer, Gardiner, Thompson: A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis. in Nature genetics 1998
Show all 3 Pubmed References
Human Polyclonal ABCB11 Primary Antibody for IHC, IHC (p) - ABIN4277166
Sambrotta, Strautnieks, Papouli, Rushton, Clark, Parry, Logan, Newbury, Kamath, Ling, Grammatikopoulos, Wagner, Magee, Sokol, Mieli-Vergani, Smith, Johnson, McClean, Simpson, Knisely, Bull, Thompson: Mutations in TJP2 cause progressive cholestatic liver disease. in Nature genetics 2014
Show all 2 Pubmed References
Data show the gene expression profiling of ABC (show ABCB6 Antibodies) transporters in seven tissues.
cloned one partial and two full gene sequences, which show high degree of identity with mammalian Pgp1 (ABCB1 (show ABCB1 Antibodies)), BSEP (ABCB11) and MRP2 (ABCC2 (show ABCC2 Antibodies)) efflux transporters and found identical relative expression patterns for both liver and primary hepatocytes
Case Report: compound heterozygotes for two missense mutations of the ABCB11 with a mild form of progressive familial intrahepatic cholestasis type 2.
Patients with a confirmed ABCB11 or tight junction protein 2 (show TJP2 Antibodies) gene mutation (n = 7) had a minimally detectable THBA proportion (0.23-2.99% of total BAs). Three patients with an ATP8B1 (show ATP8B1 Antibodies) mutation had an elevated THBA proportion (7.51-37.26%).
By these complementary approaches, a set of ten novel BSEP interaction partners was identified. With the exception of radixin (show RDX Antibodies), all other interaction partners were integral or membrane-associated proteins including proteins of the early secretory pathway and the bile acyl-CoA synthetase (show SLC27A5 Antibodies), the second to last, ER-associated enzyme of bile salt synthesis
Among the Han individuals aged over 40 years in Hunan, China, genotype CC or CT of BSEP gene SNP rs2287622 may correlate with higher risk of chronic hepatitis C in comparison with genotype TT.
Case Reports: late onset progressive familial intrahepatic cholestasis 2 secondary to novel ABCB11 mutations.
Negative immunoreaction of BSEP was found in the majority of the progressive familial intrahepatic cholestasis (PFIC (show ATP8B1 Antibodies)) group. Nonetheless, the negative immunoreaction was demonstrated in a considerable number of the non-PFIC (show ATP8B1 Antibodies) group.BSEP immunoreaction was negative in the majority (82.4%) of PFIC2 but in none of the two patients with PFIC1 (show ATP8B1 Antibodies).
The results revealed that functional impairment of BSEP predisposes the cells to altered BA disposition and is a susceptive factor to drug-induced cholestatic injury.
Results identified six novel mutations (PKHD1 (show PKHD1 Antibodies): p.Thr777Met, p.Tyr2260Cys; ABCB11: p.Val1112Phe, c.611+1G > A, p.Gly628Trpfs*3 and NPC1 (show NPC1 Antibodies): p.Glu391Lys) for the diagnostic of inherited infantile cholestatic disorders.
Report highly specific expression of BSEP and MDR3 (show ABCB4 Antibodies) in hepatocellular carcinoma.
GGT levels in patients with ATP8B1 or ABCB11 deficiency varied with age. The peak GGT value was <70U/L in the 2nd~6th month of life, <60U/L in the 7th~12th month and <50U/L beyond one year
Isoniazid/rifampicin administration significantly down-regulated the expression of hepatic bile acids transporters Ntcp (show SLC10A1 Antibodies) and Bsep in liver.
mechanism of increased biliary lipid secretion in Bsep-/- mice is based on increased expression of the responsible canalicular transporter proteins.
LKB1 (show STK11 Antibodies)/AMPK (show PRKAA1 Antibodies) and PKA control ABCB11 trafficking and polarization in hepatocytes.
CA feeding of Bsep (-/-) mice increased hepatic Cyp3a11 protein levels.
FXR (show NR1H4 Antibodies) regulates BSEP in an isoform-dependent and species-specific manner
Ursodeoxycholic acid fed to abcb11-/- mice caused liver damage and the appearance of biliary tetra- and penta-hydroxy bile acids.
Hepatic Abcb11 overexpression in mice increases the conservation of bile acids within the enterohepatic circulation.
The authors show that two of these transporters, ABCB11 and ATP8B1 (show ATP8B1 Antibodies), are functional targets of miR (show MLXIP Antibodies)-33, a micro-RNA that is expressed from within an intron of SREBP-2 (show SREBF2 Antibodies).
Abcb11 deficiency induces cholestasis coupled to impaired beta-fatty acid oxidation in mice.
hyperosmotic cholestasis is triggered by a NADPH oxidase (show NOX1 Antibodies)-driven reactive oxygen species formation that mediates Fyn (show FYN Antibodies)-dependent retrieval of the Mrp2 (show ABCC2 Antibodies) and Bsep from the canalicular membrane, which may involve an increased cortactin (show CTTN Antibodies) phosphorylation.
The membrane-associated protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the MDR/TAP subfamily. Members of the MDR/TAP subfamily are involved in multidrug resistance. The protein encoded by this gene is the major canalicular bile salt export pump in man. Mutations in this gene cause a form of progressive familial intrahepatic cholestases which are a group of inherited disorders with severe cholestatic liver disease from early infancy.
ATP-binding cassette, sub-family B (MDR/TAP), member 11
, bile salt export pump
, bile salt export pump-like
, ABC member 16, MDR/TAP subfamily
, ATP-binding cassette sub-family B member 11
, progressive familial intrahepatic cholestasis 2
, sister p-glycoprotein
, ATP-binding cassette, sub-family B, member 11
, sister of P-glycoprotein
, liver bile salt export pump