ATP-Binding Cassette, Sub-Family B (MDR/TAP), Member 11 (ABCB11) Kits ELISA

Rainbow Trout (Oncorhynchus mykiss) ATP-Binding Cassette, Sub-Family B (MDR/TAP), Member 11 (ABCB11) interaction partners

1. Data show the gene expression profiling of ABC transporters in seven tissues.

2. cloned one partial and two full gene sequences, which show high degree of identity with mammalian Pgp1 (ABCB1), BSEP (ABCB11) and MRP2 (ABCC2) efflux transporters and found identical relative expression patterns for both liver and primary hepatocytes

Human ATP-Binding Cassette, Sub-Family B (MDR/TAP), Member 11 (ABCB11) interaction partners

1. Disease-causing mutations, including ATP8B1, ABCB11, ABCB4, ABCC2, TJP2, NR1H4 (FXR), JAG1, AKR1D1, CYP7B1, PKHD1, ATP7B, and SLC25A13, were identified. Nine patients had unpredicted genetic diagnosis with atypical phenotype or novel mutations in the investigational genes.

2. The c.3223C>T is a novel nonsense mutation in ABCB11 which may cause premature termination of translation in patients with progressive familial intrahepatic cholestasis

3. The current meta-analysis indicated that BSEP rs2287622 polymorphism could increase the susceptibility of intrahepatic cholestasis of pregnancy (ICP) in Asians and in general populations, while rs473351, D482G, and rs853782 polymorphisms were not obviously associated with ICP risk. [Meta-Analysis]

4. Report biallelic heterozygosity for several ABCB11 mutations in severe intrahepatic cholestasis of pregnancy.

5. ABCB11 mutations alter the bile acid metabolome. The overall hydrophobicity indices of total bile acids in both the ABCB11-mutated group and the undiagnosed cholestasis group were lower than those of healthy controls.

6. Mutations in the genes responsible for PFIC may be involved in both young and adults with cryptogenic cholestasis in a considerable number of cases, including in heterozygous status.

7. The mutated loci at rs118109635 and rs497692 of the ABCB11 gene were correlated with PISs.

8. We concluded that Egyptian patients having chronic hepatitis C genotype 4 with CC genotype of ABCB11 SNP 1331T>C and high plasma bile acid levels at cutoff value of 75.5 mumol/L were associated with advanced hepatic fibrosis.

9. The overrepresentation of mutated variants of the 1331T > C ABCB11 polymorphism in the ICP group suggests its contribution to the etiology of the intrahepatic cholestasis of pregnancy. Analysis of genotypes' co-existence pointed to the possibility of the mutated variants of polymorphism 1954A > G ABCB4 and 1331T > C ABCB11 having a summation effect on the development ofintrahepatic cholestasis of pregnancy

10. These data demonstrated that c.386G>A (p.C129Y) in ABCB11 was a causative mutation that correlated with the phenotype of patients with PFIC2, the impairment of biliary excretion into bile canaliculi, and the absence of canalicular BSEP expression in liver histological assessments.

11. FIC1, BSEP, and MDR3 represent hepatobiliary transport proteins essential for bile formation.

12. This is the first report on a diagnostic test for simultaneous genotyping of IFNL3, ABCB11, and RNF7 in Chronic hepatitis C (CHC) patients. Reliable and inexpensive, the assay should provide useful information for the clinical management of CHC, like identification of patients at risk of rapid disease progression or with high chances of response to classic therapy.

13. Residual function of BSEP as well as substrate specificity influence the therapeutic effectiveness of partial external biliary diversion in progressive familial intrahepatic cholestasis type 2.

14. Study suggested that the CC genotype of rs2287616 variant of ABCB11 gene might contribute to anti-tuberculosis drug-induced cholestatic liver injury in Chinese patients.

15. Case Report: compound heterozygotes for two missense mutations of the ABCB11 with a mild form of progressive familial intrahepatic cholestasis type 2.

16. Patients with a confirmed ABCB11 or tight junction protein 2 gene mutation (n = 7) had a minimally detectable THBA proportion (0.23-2.99% of total BAs). Three patients with an ATP8B1 mutation had an elevated THBA proportion (7.51-37.26%).

17. By these complementary approaches, a set of ten novel BSEP interaction partners was identified. With the exception of radixin, all other interaction partners were integral or membrane-associated proteins including proteins of the early secretory pathway and the bile acyl-CoA synthetase, the second to last, ER-associated enzyme of bile salt synthesis

18. Report ABAB11 missense mutations in Korean infants with progressive familial intrahepatic cholestasis.

19. 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.

20. Case Reports: late onset progressive familial intrahepatic cholestasis 2 secondary to novel ABCB11 mutations.

Mouse (Murine) ATP-Binding Cassette, Sub-Family B (MDR/TAP), Member 11 (ABCB11) interaction partners

1. Studied effect of endotoxin exposure on liver following partial hepatectomy; found endotoxin exposure induced autoantibody production against Bsep (bile salt export pump) leading to dysfunction of hepatobiliary transporter systems.

2. Isoniazid/rifampicin administration significantly down-regulated the expression of hepatic bile acids transporters Ntcp and Bsep in liver.

3. mechanism of increased biliary lipid secretion in Bsep-/- mice is based on increased expression of the responsible canalicular transporter proteins.

4. expression repressed by estradiol in the late stages of pregnancy

5. LKB1/AMPK and PKA control ABCB11 trafficking and polarization in hepatocytes.

6. CA feeding of Bsep (-/-) mice increased hepatic Cyp3a11 protein levels.

7. FXR regulates BSEP in an isoform-dependent and species-specific manner

8. Ursodeoxycholic acid fed to abcb11-/- mice caused liver damage and the appearance of biliary tetra- and penta-hydroxy bile acids.

9. Hepatic Abcb11 overexpression in mice increases the conservation of bile acids within the enterohepatic circulation.

10. The authors 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.

11. Abcb11 deficiency induces cholestasis coupled to impaired beta-fatty acid oxidation in mice.

12. hyperosmotic cholestasis is triggered by a NADPH oxidase-driven reactive oxygen species formation that mediates Fyn-dependent retrieval of the Mrp2 and Bsep from the canalicular membrane, which may involve an increased cortactin phosphorylation.

13. Hepatic overexpression of Abcb11 in mice promotes diet-induced obesity and hypercholesterolemia; increased intestinal cholesterol absorption by hydrophobic bile acids might cause these features.

14. bile salt export pump (BSEP)

15. Atypical 3 alpha,6 beta,7 beta,12 alpha-tetrahydroxy-5 beta-cholan-24-oic acid appears in spgp knockout mice

16. regulation of Abcb11 may be important for the pathogenesis and treatment of steatohepatitis

17. the effect of the D482G progressive familial intrahepatic cholestasis type 2 related mutation on BSEP function

18. We conclude that gallstone-susceptible C57L/J mice demonstrate increased gene and canalicular membrane expression of Abcb11, however, taurocholate transport is functionally diminished.

19. Elevated level of Mdr1 in Abcb11 knockout mice functions as an alternative pathway to transport bile acids

20. Hepatic overexpression of Abcb11 increases the rate of cholesterol gallstone formation,likely due to increases in bile salt hydrophobicity but not because of alterations of other biliary lipid transporters. This strongly support Abcb11 as a Lith 1 gene.