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CRB2 Antibody

  • 中文名称：
    CRB2兔多克隆抗体
  • 货号：
    CSB-PA692212LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • IHC image of CSB-PA692212LA01HU diluted at 1:1600 and staining in paraffin-embedded human glioma performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) CRB2 Polyclonal antibody
  • Uniprot No.：
    Q5IJ48
  • 基因名：
  • 别名：
    Crb2 antibody; CRUM2_HUMAN antibody; Crumbs homolog 2 (Drosophila) antibody; Crumbs homolog 2 antibody; Crumbs-like protein 2 antibody; RP11-230L22.2 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Protein crumbs homolog 2 protein (253-483AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，CRB2 Antibody (CSB-PA692212LA01HU)，的标记方式是Non-conjugated。对于CRB2 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-PA692212LB01HU CRB2 Antibody, HRP conjugated ELISA
    FITC CSB-PA692212LC01HU CRB2 Antibody, FITC conjugated
    Biotin CSB-PA692212LD01HU CRB2 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
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:1000-1:2000
  • 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：
    Apical polarity protein that plays a central role during the epithelial-to-mesenchymal transition (EMT) at gastrulation, when newly specified mesodermal cells move inside the embryo. Acts by promoting cell ingression, the process by which cells leave the epithelial epiblast and move inside the embryo to form a new tissue layer. The anisotropic distribution of CRB2 and MYH10/myosin-IIB at cell edges define which cells will ingress: cells with high apical CRB2 are probably extruded from the epiblast by neighboring cells with high levels of apical MYH10/myosin-IIB. Plays a role in the maintenance of retinal neuroepithelium organization, structural integrity, adhesion, photoreceptor polarity and retinal photoreceptor layer thickness. May play a role in determining the length of cone photoreceptor outer segments and proliferation of late-born progenitor cells. Also required for maintenance of the apical polarity complex during development of the cortex. Inhibits gamma-secretase-dependent cleavage of APP and secretion of amyloid-beta peptide 40 and amyloid-beta peptide 42, and thereby inhibits gamma-secretase-dependent Notch transcription.
  • 基因功能参考文献：
    1. Case Report: steroid-resistant nephrotic syndrome caused by a novel Crumbs homolog 2 mutation. PMID: 29473663
    2. These findings demonstrate that Crb2 abnormalities caused by these mutations are the mechanism of steroid-resistant nephrotic syndrome PMID: 27942854
    3. Clinically, CRB2 should be assessed when ciliopathy is suspected, especially in Ashkenazi Jews, where we found that p.N800K carrier frequency is 1 of 64. Patients harboring CRB2 mutations should be tested for the complete range of ciliopathy manifestations. PMID: 26925547
    4. Additional sequence variants in genes involved in kidney development were found in patients with CRB2-related syndrome, suggesting that these variants may modify the phenotype. PMID: 27004616
    5. We demonstrate that CRB2 mutations result in loss of function and therefore constitute causative mutations leading to Nephrotic syndrome in humans. PMID: 25557779
    6. The three families with six affected individuals present compelling evidence for the role of CRB2 in human disease, with a phenotype comprising severe, congenital neurological and renal involvement. PMID: 25557780
    7. Study showed that CRB1 and CRB2 in human retinas have an opposite pattern of expression in Muller glia and photoreceptor cells compared with mouse retinas, and that Crb2 influences the severity of the murine Crb1-linked retinal dystrophies. PMID: 24565864
    8. Observational study of gene-disease association. (HuGE Navigator) PMID: 20583170
    9. results suggest that CRB2 functions as an inhibitory binding protein that is involved in the formation of a mature but inactive pool of the gamma-secretase complex PMID: 20299451
    10. Crumbs homolog 2 gene maps to human chromosome 9q33.3 PMID: 14767562
    11. This study shows that CRB2 sequence variants are not a common cause of autosomal recessive RP and LCA. PMID: 15851977
    12. Using X-ray crystallography and NMR spectroscopy, we show that, despite low amino acid sequence conservation, both 53BP1 and Crb2 contain tandem tudor domains that interact with histone H4 specifically dimethylated at Lys20 (H4-K20me2). PMID: 17190600
    13. overexpression of human CRB1 and related isoforms, CRB2 and CRB3, had no effect on the levels of presenilin complex components, on NCT maturation or on PS endoproteolysis PMID: 17988153
    14. The CRB2 gene encodes a transmembrane protein (1285 aa) and a secreted protein (1176 aa). The transmembrane isoform consists of 14 extracellular EGF-like domains, 3 extracellular laminin G-like domains, and a Crb cytoplasmic tail domain. PMID: 14767562

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  • 相关疾病：
    Focal segmental glomerulosclerosis 9 (FSGS9); Ventriculomegaly with cystic kidney disease (VMCKD)
  • 亚细胞定位：
    [Isoform 1]: Apical cell membrane; Single-pass type I membrane protein. Cytoplasm. Cell junction.; [Isoform 2]: Secreted.
  • 蛋白家族：
    Crumbs protein family
  • 组织特异性：
    Expressed in glomeruli, podocytes of the glomerular capillary loops, and parietal glomerular epithelial cells in the kidney (at protein level). Expressed in retina, fetal eye and brain. Also expressed in kidney, RPE/choroid, and at low levels in lung, pla
  • 数据库链接：

    HGNC: 18688

    OMIM: 219730

    KEGG: hsa:286204

    STRING: 9606.ENSP00000362734

    UniGene: Hs.568340