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The membrane-associated protein encoded by ABCB11 is a member of the superfamily of ATP-binding cassette (ABC) transporters. De plus, nous expédions ABCB11 Anticorps (60) et ABCB11 Protéines (8) et beaucoup plus de produits pour cette protéine.
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Data show the gene expression profiling of ABC (Montrer ABCB6 Kits ELISA) transporters in seven tissues.
cloned one partial and two full gene sequences, which show high degree of identity with mammalian Pgp1 (ABCB1 (Montrer ABCB1 Kits ELISA)), BSEP (ABCB11) and MRP2 (ABCC2 (Montrer ABCC2 Kits ELISA)) 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 (Montrer TJP2 Kits ELISA) gene mutation (n = 7) had a minimally detectable THBA proportion (0.23-2.99% of total BAs). Three patients with an ATP8B1 (Montrer ATP8B1 Kits ELISA) 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 (Montrer RDX Kits ELISA), all other interaction partners were integral or membrane-associated proteins including proteins of the early secretory pathway and the bile acyl-CoA synthetase (Montrer SLC27A5 Kits ELISA), 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 (Montrer ATP8B1 Kits ELISA)) group. Nonetheless, the negative immunoreaction was demonstrated in a considerable number of the non-PFIC (Montrer ATP8B1 Kits ELISA) group.BSEP immunoreaction was negative in the majority (82.4%) of PFIC2 but in none of the two patients with PFIC1 (Montrer ATP8B1 Kits ELISA).
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 (Montrer NPC1 Kits ELISA): p.Glu391Lys) for the diagnostic of inherited infantile cholestatic disorders.
Report highly specific expression of BSEP and MDR3 (Montrer ABCB4 Kits ELISA) 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 (Montrer SLC10A1 Kits ELISA) 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 (Montrer STK11 Kits ELISA)/AMPK (Montrer PRKAA1 Kits ELISA) and PKA control ABCB11 trafficking and polarization in hepatocytes.
CA feeding of Bsep (-/-) mice increased hepatic Cyp3a11 protein levels.
FXR (Montrer NR1H4 Kits ELISA) 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 (Montrer ATP8B1 Kits ELISA), are functional targets of miR (Montrer MLXIP Kits ELISA)-33, a micro-RNA that is expressed from within an intron of SREBP-2 (Montrer SREBF2 Kits ELISA).
Abcb11 deficiency induces cholestasis coupled to impaired beta-fatty acid oxidation in mice.
hyperosmotic cholestasis is triggered by a NADPH oxidase (Montrer NOX1 Kits ELISA)-driven reactive oxygen species formation that mediates Fyn (Montrer FYN Kits ELISA)-dependent retrieval of the Mrp2 (Montrer ABCC2 Kits ELISA) and Bsep from the canalicular membrane, which may involve an increased cortactin (Montrer CTTN Kits ELISA) 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