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FAM20C Antibody

  • 货号：
    CSB-PA816901LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western Blot
      Positive WB detected in: HepG2 whole cell lysate
      All lanes: FAM20C antibody at 4.1µg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 67, 30 kDa
      Observed band size: 67 kDa
    • IHC image of CSB-PA816901LA01HU diluted at 1:500 and staining in paraffin-embedded human liver tissue 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.
    • IHC image of CSB-PA816901LA01HU diluted at 1:500 and staining in paraffin-embedded human brain tissue 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) FAM20C Polyclonal antibody
  • Uniprot No.：
    Q8IXL6
  • 基因名：
    FAM20C
  • 别名：
    C76981 antibody; Dentin matrix protein 4 antibody; DKFZp547C074 antibody; DMP-4 antibody; DMP4 antibody; DMP4_HUMAN antibody; Extracellular serine/threonine protein kinase Fam20C antibody; Fam20c antibody; Family with sequence similarity 20 member C antibody; GEF CK antibody; Golgi enriched fraction casein kinase antibody; Protein FAM20C antibody; RNS antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Extracellular serine/threonine protein kinase FAM20C protein (107-224AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，FAM20C Antibody (CSB-PA816901LA01HU)，的标记方式是Non-conjugated。对于FAM20C 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-PA816901LB01HU FAM20C Antibody, HRP conjugated ELISA
    FITC CSB-PA816901LC01HU FAM20C Antibody, FITC conjugated
    Biotin CSB-PA816901LD01HU FAM20C 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, WB, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:500-1:1000
  • 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：
    Golgi serine/threonine protein kinase that phosphorylates secretory pathway proteins within Ser-x-Glu/pSer motifs and plays a key role in biomineralization of bones and teeth. Constitutes the main protein kinase for extracellular proteins, generating the majority of the extracellular phosphoproteome. Mainly phosphorylates proteins within the Ser-x-Glu/pSer motif, but also displays a broader substrate specificity. Phosphorylates casein as well as a number of proteins involved in biomineralization such as AMELX, AMTN, ENAM and SPP1. In addition to its role in biomineralization, also plays a role in lipid homeostasis, wound healing and cell migration and adhesion.
  • 基因功能参考文献：
    1. that FAM20C may affect the biomineralization by the means more than local phosphorylation of extracellular matrix proteins and systemic phosphorus homeostasis PMID: 28926103
    2. Histidine-rich Ca-binding protein (HRC) was phosphorylated by family with sequence similarity 20C (Fam20C) both in vitro and in vivo. PMID: 28784772
    3. These results suggest that TET1 potentially promotes the cytodifferentiation potential of human dental pulp cells through its DNA demethylation machinery and upregulation of FAM20C protein expression. PMID: 29277934
    4. findings clarify FAM20C's role in hard tissue formation and mineralization, and show that Raine syndrome is congenital sclerosing osteomalacia with cerebral calcification. PMID: 27862258
    5. Alterations of Fam20C activity, promoted by myriocin and sphingolipids, are not accompanied by any significant change in Fam20C protein. These data provide the proof of concept that Fam20C activity is under the control of sphingolipid signaling PMID: 28236661
    6. Our report reinforces that Raine syndrome is compatible with life, and that mild hypophosphatemia and amelogenesis imperfecta are key features of the attenuated form. PMID: 27667191
    7. The Fam20C-and VLK-family of kinases mediate the phosphorylation of proteins in the secretory pathway and extracellular space.Mutation in several secretory pathway kinases cause human disease PMID: 25862977
    8. by treating Fam20C expressing HEK293T cells with myriocin, a potent inhibitor of the sphingosine biosynthetic pathway, the activity of Fam20C released into the conditioned medium is substantially decreased corroborating the concept that sphingosine PMID: 25936777
    9. Using CRISPR/Cas9 genome editing, mass spectrometry, and biochemistry, study identifies more than 100 secreted phosphoproteins as genuine Fam20C substrates; further, study shows that Fam20C exhibits broader substrate specificity than previously appreciated. PMID: 26091039
    10. phenotype in two families with non-lethal Raine syndrome with FAM20C mutations PMID: 25928877
    11. Findings suggest that certain homozygous FAM20C mutations can cause FGF23-related hypophosphatemic osteomalacia and indicate the multiple roles of FAM20C in bone. PMID: 24982027
    12. Results suggest that FAM20C suppresses FGF23 production by enhancing DMP1 expression, and inactivating mutations in FAM20C cause FGF23-related hypophosphatemia by decreasing transcription of DMP1. PMID: 25026495
    13. We report on a child who is homozygous for a 487-kb deletion in 7p22.3 that contains FAM20C PMID: 24039075
    14. Fam20C phosphorylates FGF23, which promotes FGF23 proteolysis by furin by blocking O-glycosylation by polypeptide N-acetylgalactosaminyltransferase 3. PMID: 24706917
    15. mutations in FAM20C provide a putative new mechanism in human subjects leading to dysregulated FGF23 levels, hypophosphatemia, hyperphosphaturia, dental anomalies, intracerebral calcifications and osteosclerosis of the long bones PMID: 23325605
    16. Our results identify FAM20C as a kinase for secreted phosphoproteins and establish a biochemical basis for Raine syndrome. PMID: 22900076
    17. Fam20C appears to be the Golgi casein kinase that phosphorylates secretory pathway proteins within S-x-E motifs; Fam20C phosphorylates caseins and several secreted proteins implicated in biomineralization; mutations in Fam20C cause an osteosclerotic bone dysplasia known as Raine syndrome PMID: 22582013
    18. Osteosclerotic bone dysplasia in siblings with a Fam20C mutation PMID: 20825432
    19. This study defines the causative role of FAM20C in this lethal osteosclerotic disorder and its crucial role in normal bone development. PMID: 17924334
    20. Mutation of FAM20C does not always lead to the infantile lethality previously seen as a prerequisite for Raine syndrome diagnosis. PMID: 19250384

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  • 相关疾病：
    Raine syndrome (RNS)
  • 亚细胞定位：
    Secreted. Golgi apparatus.
  • 蛋白家族：
    FAM20 family
  • 组织特异性：
    Widely expressed.
  • 数据库链接：

    HGNC: 22140

    OMIM: 259775

    KEGG: hsa:56975

    STRING: 9606.ENSP00000322323

    UniGene: Hs.134742