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ECEL1 Antibody

  • 货号：
    CSB-PA007373LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA007373LA01HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human cervical cancer using CSB-PA007373LA01HU at dilution of 1:100
    • Immunofluorescent analysis of HepG2 cells using CSB-PA007373LA01HU at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
    • Immunofluorescent analysis of Hela cells using CSB-PA007373LA01HU at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
  • 其他：

产品详情

  • 产品描述：
    ECEL1 polyclonal antibody CSB-PA007373LA01HU was produced in the rabbit immunized by using the Recombinant Human Endothelin-converting enzyme-like 1 protein (425-775AA) as the immunogen. The target protein ECEL1 is a member of the M13 family of endopeptidases. Members of this family are zinc-containing type II integral-membrane proteins that are important regulators of neuropeptide and peptide hormone activity. Diseases associated with ECEL1 include Arthrogryposis, Distal, Type 5D and Distal Arthrogryposis.
    This Rabbit anti-Homo sapiens (Human) ECEL1 Polyclonal antibody was tested in the IF, IHC and ELISA applications. The non-conjugated IgG got purified by protein G and reached up to 95% in purity. It only reacts with the ECEL1 proteins of human-origin and may be used to detect the endogenous levels of ECEL1 protein.
  • 产品名称：
    Rabbit anti-Homo sapiens (Human) ECEL1 Polyclonal antibody
  • Uniprot No.：
    O95672
  • 基因名：
    ECEL1
  • 别名：
    ECEL1; XCE; UNQ2431/PRO4991; Endothelin-converting enzyme-like 1; Xce protein
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Endothelin-converting enzyme-like 1 protein (425-775AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，ECEL1 Antibody (CSB-PA007373LA01HU)，的标记方式是Non-conjugated。对于ECEL1 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-PA007373LB01HU ECEL1 Antibody, HRP conjugated ELISA
    FITC CSB-PA007373LC01HU ECEL1 Antibody, FITC conjugated
    Biotin CSB-PA007373LD01HU ECEL1 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 1:50-1:200
  • 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：
    May contribute to the degradation of peptide hormones and be involved in the inactivation of neuronal peptides.
  • 基因功能参考文献：
    1. Mutation of a conserved residue in ECEL1 is linked with fetal arthrogryposis multiplex congenita. PMID: 25708584
    2. Our clinical findings are consistent with recessive ECEL1 mutations causing variably penetrant orbital dysinnervation phenotypes (ptosis and/or complex strabismus with abnormal synkinesis) PMID: 25173900
    3. Three novel ECEL1 mutations have been identified in consanguineous pedigrees of Saudi Arabian origin presenting with distal arthrogryposis type 5D. PMID: 23829171
    4. A novel missense c.1819G>A mutation (G607S) in the ECEL1 gene has been identified in a consanguineous pedigree of Turkish origin presenting with congenital contracture syndromes. PMID: 23808592
    5. We described a new and homogenous phenotype of DA associated with ECEL1 that resulted in symptoms involving rather the peripheral than the central nervous system and suggesting a developmental dysfunction PMID: 23236030
    6. Mutations in ECEL1 cause distal arthrogryposis type 5D. PMID: 23261301
    7. Sp1 recruits ATF3, c-Jun, and STAT3 to obtain the requisite synergistic effect in neuronal injury through DINE neuronal injury-inducible gene PMID: 18192274

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  • 相关疾病：
    Arthrogryposis, distal, 5D (DA5D)
  • 亚细胞定位：
    Membrane; Single-pass type II membrane protein.
  • 蛋白家族：
    Peptidase M13 family
  • 组织特异性：
    Highly expressed in the CNS, in particular in putamen, spinal cord, medulla and subthalamic nucleus. A strong signal was also detected in uterine subepithelial cells and around renal blood vessels. Detected at lower levels in amygdala, caudate, thalamus,
  • 数据库链接：

    HGNC: 3147

    OMIM: 605896

    KEGG: hsa:9427

    STRING: 9606.ENSP00000302051

    UniGene: Hs.26880