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ABCB4 Antibody

  • 货号：
    CSB-PA001050LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunohistochemistry of paraffin-embedded human melanoma using CSB-PA001050LA01HU at dilution of 1:100
    • Immunofluorescence staining of A549 cells with CSB-PA001050LA01HU at 1:200, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) ABCB4 Polyclonal antibody
  • Uniprot No.：
    P21439
  • 基因名：
    ABCB4
  • 别名：
    ABC 21 antibody; ABC B4 antibody; ABC21 antibody; ABCB 4 antibody; Abcb4 antibody; ABCB4 protein antibody; ATP binding cassette sub family B MDR/TAP member 4 antibody; ATP binding cassette sub family B member 4 antibody; ATP-binding cassette sub-family B member 4 antibody; GBD1 antibody; ICP3 antibody; MDR 3 antibody; MDR2 antibody; MDR2/3 antibody; MDR3 antibody; MDR3 P glycoprotein antibody; MDR3 P gp antibody; MDR3_HUMAN antibody; Multidrug resistance protein 3 antibody; Multiple drug resistance 3 antibody; P glycoprotein 3 antibody; P-glycoprotein 3 antibody; PFIC 3 antibody; PFIC3 antibody; PGY 3 antibody; PGY3 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Phosphatidylcholine translocator ABCB4 protein (530-693AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，ABCB4 Antibody (CSB-PA001050LA01HU)，的标记方式是Non-conjugated。对于ABCB4 Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA001050LB01HU ABCB4 Antibody, HRP conjugated ELISA
    FITC CSB-PA001050LC01HU ABCB4 Antibody, FITC conjugated
    Biotin CSB-PA001050LD01HU ABCB4 Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC, IF
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:20-1:200
    IF 1:200-1:500
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 功能：
    Energy-dependent phospholipid efflux translocator that acts as a positive regulator of biliary lipid secretion. Functions as a floppase that translocates specifically phosphatidylcholine (PC) from the inner to the outer leaflet of the canalicular membrane bilayer into the canaliculi of hepatocytes. Translocation of PC makes the biliary phospholipids available for extraction into the canaliculi lumen by bile salt mixed micelles and therefore protects the biliary tree from the detergent activity of bile salts. Plays a role in the recruitment of phosphatidylcholine (PC), phosphatidylethanolamine (PE) and sphingomyelin (SM) molecules to nonraft membranes and to further enrichment of SM and cholesterol in raft membranes in hepatocytes. Required for proper phospholipid bile formation. Indirectly involved in cholesterol efflux activity from hepatocytes into the canalicular lumen in the presence of bile salts in an ATP-dependent manner. Promotes biliary phospholipid secretion as canaliculi-containing vesicles from the canalicular plasma membrane. In cooperation with ATP8B1, functions to protect hepatocytes from the deleterious detergent activity of bile salts. Does not confer multidrug resistance.
  • 基因功能参考文献：
    1. ABCB4 is down-regulated in the 5-Fu resistant cells and knockdown of ABCB4 alleviated the cell apoptosis and predicts a shorter recurrence-free survival and overall survival in colorectal cancer. PMID: 29371412
    2. 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 PMID: 30091450
    3. reduced ABCB4 expression predisposes to extrahepatic biliary atresia PMID: 28355206
    4. The functional impact of ABCB4 mutations found in progressive familial intrahepatic cholestasis type 3. PMID: 27256251
    5. Several members of a family exhibited cholestasis and were found to have a substitution of glycine 68 by arginine in ABCB4 due to a missense mutation at base 202. The 18-year-old propositus was heterozygous for this mutation and also suffered acute pancreatitis. PMID: 26242827
    6. ABCB4 variants identified in patients with biliary diseases PMID: 28587926
    7. results are suggestive of a potential downstream molecular effect for the described polymorphisms on the expression pattern of the ABCB4 underlining the importance of synonymous variants PMID: 27788395
    8. Ivacaftor is a potential therapy for selected patients with cystic fibrosis with mutations of ABCB4. PMID: 28012258
    9. Data show that patients with low multidrug resistance protein 3 (MDR3) expression were significantly associated with a better outcome than patients with high MDR3 expression. PMID: 28061436
    10. In patients with intrahepatic cholestasis of pregnancy, ABCB4 gene mutation was not associated with response to ursodeoxycholic acid treatment. PMID: 27825922
    11. Data suggest that MDR3 isoforms, both human and mouse isoforms, exhibit different affinities for fluorescent dyes and drugs; thus, ligands likely occupy partially overlapping but distinct binding sites. PMID: 28441502
    12. Single-nucleotide polymorphism in ABCB4 gene is associated with gallbladder cancer. PMID: 28274756
    13. the cholestatic potential of certain drugs may be aggravated by simultaneous inhibition of BSEP and MDR3. PMID: 27112167
    14. Negative immunoreaction of MDR3 was found in the majority of the progressive familial intrahepatic cholestasis (PFIC) group. Nonetheless, the negative immunoreaction was demonstrated in a considerable number of the non-PFIC group. Negative MDR3 immunoreaction was more frequently associated with PFIC3 compared to non-PFIC group. PMID: 26845599
    15. Exons 6, 8 and 9 mutations of ABCB4 gene are not common among Egyptian children with Progressive Familial Intrahepatic Cholestasis type 3. PMID: 27075526
    16. Case Report: novel ABCB4 mutation in mother and daughter with progressive familial intrahepatic cholestasis 3. PMID: 26796082
    17. ABCB4 mutation causing progressive familial intrahepatic cholestasis 3 in two brothers, and intrahepatic cholestasis of pregnancy in their mother is described. PMID: 26699824
    18. A mutant ABCB4 genotype is related to the clinical expression of cholestatic liver disease. PMID: 26324191
    19. There are varying effects of ABCB4 missense mutations but even a modest reduction in MDR3 activity may contribute or predispose to the onset of Cholestatic liver disease in the pediatric age. PMID: 26153658
    20. A mutation of ABCB4 protein as a cause of liver disease PMID: 26410236
    21. most severe phenotypes appreciated by the duration of transplant-free survival were caused by ABCB4 variants that were markedly retained in the endoplasmic reticulum and expressed in a homozygous status PMID: 26474921
    22. Report highly specific expression of BSEP and MDR3 in hepatocellular carcinoma. PMID: 26735860
    23. Interaction of ABCB4 with EBP50 through its PDZ-like motif plays a critical role in the regulation of ABCB4 expression and stability at the canalicular plasma membrane. PMID: 26789121
    24. Case Report: liver fibrosis associated with MDR3 deficiency can be reversed by long-term treatment with UDCA, at least when there is residual expression of the protein. PMID: 26256905
    25. These results suggest that a SM-rich membrane environment is required for ABCB4 to function. PMID: 25601960
    26. The exome-sequencing of 3 family members identified 7 potential mutations: of these, rs58238559, a rare missense genetic variant in the ABCB4 gene was carried by all affected members; rs58238559 in ABCB4 is a rare missense variant with a significant effect on the development of atrial flutter or atrial fibrillation. PMID: 25888430
    27. variants in the genes ABCB4, TP53AIP1, ARHGAP32 and TMEM88B were identified linked to the alexithymia phenotype. PMID: 25882097
    28. ABCB4 is a frequently silenced gene in different cancers and it may act tumor suppressivly in lung cancer. PMID: 25367630
    29. Case Report: novel ABCB4 mutation in a Chinese patient with progressive familial intrahepatic cholestasis type 3. PMID: 25593501
    30. several mutations in ABCB4 increase risk of liver diseases. PMID: 25807286
    31. data on the Q1174E mutant demonstrated basal ATPase activity, but PC lipids were incapable of stimulating ATPase activity highlighting the role of the extended X loop in the cross-talk of the nucleotide-binding domain and the transmembrane domain. PMID: 25533467
    32. ABCB4 mutations have roles in hormonal cholestasis but not pediatric idiopathic gallstones PMID: 24914347
    33. intratumoral expression affected the prognosis of patients with hepatocellular carcinoma PMID: 25173835
    34. These data provide experimental evidence of the correlation between the degree of MDR3 floppase activity and the clinical outcomes of PFIC3 PMID: 24594635
    35. Two ABCB4 point mutations of strategic NBD-motifs do not prevent protein targeting to the plasma membrane but promote MDR3 dysfunction. PMID: 24045840
    36. We identified disease-associated variants of ABCB4 involved in the phosphorylation of its N-terminal domain and leading to decreased phosphatidylcholine secretion. PMID: 24723470
    37. The level of ABCB4 functionality correlates with, and is the primary determinant of, cholestatic disease severity in these patients. PMID: 24806754
    38. Fenofibrate transactivates MDR3 gene transcription by way of the binding of PPARalpha to three novel and functionally critical response elements in the MDR3 promoter. PMID: 24122873
    39. Six single nucleotide polymorphisms in ABCB4 that showed significant evidence of association were identified. PMID: 24366234
    40. Case Report: novel ABCB4 mutation in progressive familial intrahepatic cholestasis III. PMID: 23574360
    41. ABCB4 located in nonrafts, but not in rafts, is predominantly involved in the efflux of phospholipids and other substrates. PMID: 23468132
    42. Genotyping of Italian women revealed 2 novel variants of ABCB4 (p.I587DfsX603, p.I738LfsX744) associated with intrahepatic cholestasis of pregnancy. PMID: 23022423
    43. this study suggests that two common haplotypes of MDR3 can regulate the transcriptional rate of MDR3 and that NF-Y may be one of the transcriptional factors involved in this regulation. PMID: 23261441
    44. Data indicate that serum glucose levels are associated with a common ABCB4 variant. PMID: 22982378
    45. ABCB4/MDR3 gene mutations in cholangiocarcinomas. PMID: 22387667
    46. In mainland Chinese children, some cases of chronic intrahepatic cholestasis with high gamma-glutamyl transpeptidase could be attributed to ABCB4 mutations. PMID: 22343912
    47. A significant subset of patients with symptomatic cholestasis/cholelithiasis hae underlying ABCB4 deletions. PMID: 21989363
    48. Liver lesions previously unreported in association with MDR3/ABCB4 gene mutations PMID: 22331132
    49. Patients undergoing partial hepatectomy with low post-operative bilirubin had lower levels of NTCP, MDR3 and BSEP mRNA compared to those with high bilirubin after Pringle manoeuvre. PMID: 22098322
    50. LPAC syndrome is based on a mutation in the ABCB4 gene which codes for MDR3 (multidrug resistance protein 3). PMID: 21161147

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  • 相关疾病：
    Cholestasis, progressive familial intrahepatic, 3 (PFIC3); Cholestasis of pregnancy, intrahepatic 3 (ICP3); Gallbladder disease 1 (GBD1)
  • 亚细胞定位：
    Cell membrane; Multi-pass membrane protein. Apical cell membrane; Multi-pass membrane protein. Membrane raft. Cytoplasm. Cytoplasmic vesicle, clathrin-coated vesicle.
  • 蛋白家族：
    ABC transporter superfamily, ABCB family, Multidrug resistance exporter (TC 3.A.1.201) subfamily
  • 数据库链接：

    HGNC: 45

    OMIM: 171060

    KEGG: hsa:5244

    STRING: 9606.ENSP00000265723

    UniGene: Hs.654403