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  4. LBR Antibody
  • 货号：
    CSB-PA070468
  • 规格：
    ￥1100
  • 图片：
    • The image on the left is immunohistochemistry of paraffin-embedded Human ovarian cancer tissue using CSB-PA070468(LBR Antibody) at dilution 1/50, on the right is treated with fusion protein. (Original magnification: ×200)
    • Gel: 6%SDS-PAGE, Lysate: 40 μg, Lane: Human fetal brain tissue, Primary antibody: CSB-PA070468(LBR Antibody) at dilution 1/300, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 3 minutes
  • 其他：

产品详情

  • Uniprot No.：
    Q14739
  • 基因名：
    LBR
  • 别名：
    DHCR 14B antibody; DHCR14B antibody; Integral nuclear envelope inner membrane protein antibody; Lamin-B receptor antibody; LBR antibody; LBR_HUMAN antibody; LMN 2R antibody; LMN2R antibody; MGC9041 antibody; PHA antibody; PRO0650 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Fusion protein of Human LBR
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen affinity purification
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB,IHC
  • 推荐稀释比：
    Application Recommended Dilution
    ELISA 1:2000-1:5000
    WB 1:500-1:2000
    IHC 1:50-1:200
  • 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：
    Catalyzes the reduction of the C14-unsaturated bond of lanosterol, as part of the metabolic pathway leading to cholesterol biosynthesis. Plays a critical role in myeloid cell cholesterol biosynthesis which is essential to both myeloid cell growth and functional maturation. Mediates the activation of NADPH oxidases, perhaps by maintaining critical levels of cholesterol required for membrane lipid raft formation during neutrophil differentiation. Anchors the lamina and the heterochromatin to the inner nuclear membrane.
  • 基因功能参考文献：
    1. The s demonstrate that human LBR is essential for cholesterol synthesis. PMID: 27336722
    2. These observations point to a new mechanism regulating the localization of LBR, which is governed by an ELYS-mediated phosphorylation network. PMID: 27802161
    3. The primary response of cells to various stresses leading to senescence consists of the down-regulation of LBR and LB1 to attain reversal of the chromatin architecture. PMID: 27760841
    4. We have shown here that lamin B receptor (LBR) showed a change in localization in both BrdU-induced and replicative senescent cells. PMID: 27059139
    5. Data indicate that proto-oncogene protein c-akt (Akt) phosphorylates distinct sites than SRPK1 protein within the arginine-serine (RS) domain of Lamin B Receptor (LBR). PMID: 27105349
    6. Lamin B receptor mRNA expression was directly associated with tumor grade in breast cancer patients(grade 1 vs. grade 3 - 0.00 vs. 0.00; p = 0.0479) and Nottingham Prognostic Index (NPI1 vs. NPI3 - 0.00 vs. 0.00; p = 0.0551). PMID: 24293108
    7. Mutation in LBR gene is associated with pelger-huet anomaly and a mild skeletal phenotype. PMID: 23824842
    8. Lymphohematopoietic licence: sterol C-14 reductase activity of lamin B receptor (Lbr) is essential for neutrophil differentiation. PMID: 22581323
    9. LBR missense mutations can abolish sterol reductase activity, causing lethal Greenberg dysplasia but not Pelger anomaly PMID: 21327084
    10. The LBR knockdown cells retain an ovoid shaped nucleus with reduced levels of lamin A/C during in vitro granulopoiesis induced with retinoic acid. PMID: 21327094
    11. Our studies reveal for the first time a mechanism that controls the timing of nuclear envelope reassembly through modification of the integral nuclear membrane protein lamin B receptor PMID: 21795390
    12. a highly significant gene-dosage effect between the gene copy number and the nuclear segmentation index of neutrophils PMID: 21326950
    13. LBR is heavily expressed in papillary thyroid carcinoma cells, but an abnormal folding of the protein might explain its lack of immunohistochemical reactivity and be associated with the anomalous folding of the nuclear membrane. PMID: 21103616
    14. LBR targets the membrane precursor vesicles to chromatin by interacting with importin beta in a LBR phosphorylation-dependent manner during the NE assembly at the end of mitosis PMID: 20576617
    15. LBR mutant variants and sterol reductases can severely interfere with the regular organization of the nuclear envelope and the endoplasmic reticulum. PMID: 19940018
    16. results suggest that LMNA, ZMPSTE24, and LBR sequence variations are not major genetic determinants involved in scleroderma pathogenesis PMID: 19645629
    17. The binding of lamin B receptor to chromatin is regulated by phosphorylation in the arginine-serine repeat-containing REGION PMID: 11846796
    18. Mutations in the gene encoding the lamin B receptor produce an altered nuclear morphology in granulocytes (Pelger-Huet anomaly). PMID: 12118250
    19. Autosomal recessive HEM/Greenberg skeletal dysplasia is caused by 3 beta-hydroxysterol delta 14-reductase deficiency due to mutations in this gene. PMID: 12618959
    20. examination of temporal association with protamine 1 during spermiogenesis PMID: 14701833
    21. SR protein kinase and cdc2 kinase regulate lamin B receptor binding to chromatin PMID: 14718546
    22. MeCP2 interacts in vitro and in vivo with the inner nuclear membrane protein LBR, and the unstructured aminoacidic sequence linking the MBD and TRD domains of MeCP2 is responsible for this association. PMID: 19331822

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  • 相关疾病：
    Pelger-Huet anomaly (PHA); Greenberg dysplasia (GRBGD); Reynolds syndrome (REYNS)
  • 亚细胞定位：
    Nucleus inner membrane; Multi-pass membrane protein. Endoplasmic reticulum membrane. Cytoplasm. Nucleus.
  • 蛋白家族：
    ERG4/ERG24 family
  • 组织特异性：
    Expressed in the bone marrow, liver, heart, adrenal gland, lung, placenta and uterus. Expressed in osteoclasts and osteoblast-like cells.
  • 数据库链接：

    HGNC: 6518

    OMIM: 169400

    KEGG: hsa:3930

    STRING: 9606.ENSP00000272163

    UniGene: Hs.435166