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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 Antibodies (60) and ABCB11 Kits (12) and many more products for this protein.
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Data show the gene expression profiling of ABC (show ABCB6 Proteins) transporters in seven tissues.
cloned one partial and two full gene sequences, which show high degree of identity with mammalian Pgp1 (ABCB1 (show ABCB1 Proteins)), BSEP (ABCB11) and MRP2 (ABCC2 (show ABCC2 Proteins)) 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 Proteins) gene mutation (n = 7) had a minimally detectable THBA proportion (0.23-2.99% of total BAs). Three patients with an ATP8B1 (show ATP8B1 Proteins) 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 Proteins), 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 Proteins), 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 Proteins)) group. Nonetheless, the negative immunoreaction was demonstrated in a considerable number of the non-PFIC (show ATP8B1 Proteins) group.BSEP immunoreaction was negative in the majority (82.4%) of PFIC2 but in none of the two patients with PFIC1 (show ATP8B1 Proteins).
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: p.Thr777Met, p.Tyr2260Cys; ABCB11: p.Val1112Phe, c.611+1G > A, p.Gly628Trpfs*3 and NPC1 (show NPC1 Proteins): p.Glu391Lys) for the diagnostic of inherited infantile cholestatic disorders.
Report highly specific expression of BSEP and MDR3 (show ABCB4 Proteins) 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 Proteins) 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 Proteins)/AMPK (show PRKAA1 Proteins) and PKA control ABCB11 trafficking and polarization in hepatocytes.
CA feeding of Bsep (-/-) mice increased hepatic Cyp3a11 protein levels.
FXR (show NR1H4 Proteins) 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 Proteins), are functional targets of miR (show MLXIP Proteins)-33, a micro-RNA that is expressed from within an intron of SREBP-2 (show SREBF2 Proteins).
Abcb11 deficiency induces cholestasis coupled to impaired beta-fatty acid oxidation in mice.
hyperosmotic cholestasis is triggered by a NADPH oxidase (show NOX1 Proteins)-driven reactive oxygen species formation that mediates Fyn (show FYN Proteins)-dependent retrieval of the Mrp2 (show ABCC2 Proteins) and Bsep from the canalicular membrane, which may involve an increased cortactin (show CTTN Proteins) 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