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EXTL3 Antibody

  • 货号：
    CSB-PA399518
  • 规格：
    ￥1100
  • 图片：
    • The image on the left is immunohistochemistry of paraffin-embedded Human colon cancer tissue using CSB-PA399518(EXTL3 Antibody) at dilution 1/30, on the right is treated with fusion protein. (Original magnification: ×200)
    • The image on the left is immunohistochemistry of paraffin-embedded Human brain tissue using CSB-PA399518(EXTL3 Antibody) at dilution 1/30, on the right is treated with fusion protein. (Original magnification: ×200)
  • 其他：

产品详情

  • Uniprot No.：
    O43909
  • 基因名：
    EXTL3
  • 别名：
    botv antibody; DKFZp686C2342 antibody; Exostoses (multiple)-like 3 antibody; Exostoses-like 3 antibody; Exostosin-like 3 antibody; EXT-related protein 1 antibody; EXTL1L antibody; EXTL3 antibody; EXTL3_HUMAN antibody; EXTR1 antibody; Glucuronyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferas antibody; Glucuronyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase antibody; Hereditary multiple exostoses gene isolog antibody; KIAA0519 antibody; Multiple exostosis-like protein 3 antibody; Putative tumor suppressor protein EXTL3 antibody; REG antibody; Reg receptor antibody; REGR antibody; RPR antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse
  • 免疫原：
    Fusion protein of Human EXTL3
  • 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,IHC
  • 推荐稀释比：
    Application Recommended Dilution
    ELISA 1:2000-1:5000
    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：
    Glycosyltransferase which regulates the biosynthesis of heparan sulfate (HS). Important for both skeletal development and hematopoiesis, through the formation of HS proteoglycans (HSPGs). Required for the function of REG3A in regulating keratinocyte proliferation and differentiation.
  • 基因功能参考文献：
    1. Human lumenal N-glycosylated EXTL3 (EXTL3DeltaN) was cloned, expressed in human embryonic kidney cells, and purified. Various biophysical and biochemical approaches were then employed to elucidate the N-glycosylation sites and the function of their attached N-glycans. PMID: 29346724
    2. EXTL3 missense mutation is associated with spondylo-epi-metaphyseal dysplasia. PMID: 28331220
    3. EXTL3 mutations as a novel cause of severe immune deficiency with skeletal dysplasia and developmental delay and underline a crucial role of HS in thymopoiesis and skeletal and brain development. PMID: 28148688
    4. We show that biallelic mutations in EXTL3 disturb glycosaminoglycan synthesis and thus lead to a recognizable syndrome characterized by variable expression of skeletal, neurological, and immunological abnormalities. PMID: 28132690
    5. Recombinant EXTL2 showed weak ability to transfer N-acetylgalactosamine to heparan sulfate precursor molecules but also, that EXTL2 exhibited much stronger in vitro N-acetylglucosamine-transferase activity related to elongation of heparan sulfate chains. PMID: 25829497
    6. Regenerating islet-derived 1alpha (Reg-1alpha) protein is new neuronal secreted factor that stimulates neurite outgrowth via exostosin Tumor-like 3 (EXTL3) receptor. PMID: 22158612
    7. Lysosomal glycosaminoglycan levels in mucopolysaccharidosis are reduced by EXTL3 gene silencing. PMID: 19690583
    8. We conclude that EXTL3 promoter methylation and its related loss of EXTL3 expression are involved in the loss of HS expression in mucinous CRCs. PMID: 18543267
    9. HIP enhanced EXTL3 translocation from the membrane to the nucleus, in support of a model whereby EXTL3 mediates HIP signaling for islet neogenesis. PMID: 19158046
    10. A missense mutation involving the exon 3 of the EXTL3 gene in the case of obstructing colon cancer is described in a 31-year-old patient affected by hereditary multiple exostoses. PMID: 19653241
    11. These results suggest that EXTL3/EXTR1 is a cell surface Reg receptor that binds to Reg protein. PMID: 10753861

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  • 相关疾病：
    Immunoskeletal dysplasia with neurodevelopmental abnormalities (ISDNA)
  • 亚细胞定位：
    Endoplasmic reticulum membrane; Single-pass type II membrane protein. Golgi apparatus.
  • 蛋白家族：
    Glycosyltransferase 47 family
  • 组织特异性：
    Ubiquitous. Expressed in keratinocytes.
  • 数据库链接：

    HGNC: 3518

    OMIM: 605744

    KEGG: hsa:2137

    STRING: 9606.ENSP00000220562

    UniGene: Hs.491354