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PREPL Antibody

  • 货号：
    CSB-PA018665GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • 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,Mouse,Rat
  • 免疫原：
    Human PREPL
  • 免疫原种属：
    Homo sapiens (Human)
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity Purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB
  • 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