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PREPL Antibody

  • 货号：
    CSB-PA018665LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • IHC image of CSB-PA018665LA01HU diluted at 1:400 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.
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) PREPL Polyclonal antibody
  • Uniprot No.：
    Q4J6C6
  • 基因名：
    PREPL
  • 别名：
    PPCEL_HUMAN antibody; prepl antibody; Prolyl endopeptidase-like antibody; Prolylendopeptidase-like antibody; putative prolyl oligopeptidase antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Prolyl endopeptidase-like protein (604-699AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，PREPL Antibody (CSB-PA018665LA01HU)，的标记方式是Non-conjugated。对于PREPL 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-PA018665LB01HU PREPL Antibody, HRP conjugated ELISA
    FITC CSB-PA018665LC01HU PREPL Antibody, FITC conjugated
    Biotin CSB-PA018665LD01HU PREPL 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 1:200-1:500
  • 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：
    Serine peptidase whose precise substrate specificity remains unclear. Does not cleave peptides after a arginine or lysine residue. Regulates trans-Golgi network morphology and sorting by regulating the membrane binding of the AP-1 complex. May play a role in the regulation of synaptic vesicle exocytosis.
  • 基因功能参考文献：
    1. we report the first homozygous PREPL point mutation in a girl with typical PREPL deficiency. This syndrome should be considered in the differential diagnosis of hypotonic neonates exhibiting myasthenic symptoms, hyperphagia, and various degrees of ID. PMID: 29483676
    2. Deletion of PREPL is associated with Hypotonia-cystinuria syndrome. PMID: 23794250
    3. Two novel deletions encompassing the SLC3A1 and PREPL genes have been identified in unrelated hypotonia-cystinuria syndrome patients. PMID: 22796000
    4. description of L-leucine transport into bladder carcinoma cells PMID: 12225859
    5. A 638-residue variant of PREPL, PREPL A, was identifiied, expressed in Escherichia coli and purified; its secondary structure was similar to that of oligopeptidase B. PMID: 16143824
    6. Deletion of PREPL, a gene encoding a putative serine oligopeptidase, in patients with hypotonia-cystinuria syndrome. PMID: 16385448
    7. Deletion of the gene results in hypotonia at birth, failure to thrive and growth hormone deficiency PMID: 16913837
    8. a deletion of PREPL causes atypical hypotonia-cystinuria syndrome PMID: 18234729

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  • 相关疾病：
    Hypotonia-cystinuria syndrome (HCS); Myasthenic syndrome, congenital, 22 (CMS22)
  • 亚细胞定位：
    Cytoplasm, cytosol. Golgi apparatus, trans-Golgi network. Cytoplasm, cytoskeleton. Golgi apparatus. Nucleus.
  • 蛋白家族：
    Peptidase S9A family
  • 组织特异性：
    Expressed in pyramidal neurons of the temporal cortex and neocortex (at protein level). Widely expressed. Expressed at higher level in brain, skeletal muscle, heart and kidney. Expressed at the endplates in the neuromuscular junction.
  • 数据库链接：

    HGNC: 30228

    OMIM: 606407

    KEGG: hsa:9581

    STRING: 9606.ENSP00000260648

    UniGene: Hs.444349