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P3H2 Antibody

  • 货号：
    CSB-PA815555HA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western Blot
      Positive WB detected in: Mouse lung tissue, Mouse kidney tissue
      All lanes: LEPREL1 antibody at 3μg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 81, 61 kDa
      Observed band size: 81, 61 kDa
    • Immunohistochemistry of paraffin-embedded human liver tissue using CSB-PA815555HA01HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human lung tissue using CSB-PA815555HA01HU at dilution of 1:100
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) P3H2 Polyclonal antibody
  • Uniprot No.：
    Q8IVL5
  • 基因名：
    P3H2
  • 别名：
    4832416N06 antibody; AW553532 antibody; Leprecan like 1 antibody; Leprecan-like protein 1 antibody; Leprel1 antibody; MCVD antibody; MLAT4 antibody; Myxoid liposarcoma-associated protein 4 antibody; P3H2 antibody; P3H2_HUMAN antibody; Prolyl 3 hydroxylase 3 antibody; Prolyl 3-hydroxylase 2 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human, Mouse
  • 免疫原：
    Recombinant Human Prolyl 3-hydroxylase 2 protein (301-528AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，P3H2 Antibody (CSB-PA815555HA01HU)，的标记方式是Non-conjugated。对于P3H2 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-PA815555HB01HU P3H2 Antibody, HRP conjugated ELISA
    FITC CSB-PA815555HC01HU P3H2 Antibody, FITC conjugated
    Biotin CSB-PA815555HD01HU P3H2 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 1:2000-1:5000
    IHC 1:20-1:200
  • 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：
    Prolyl 3-hydroxylase that catalyzes the post-translational formation of 3-hydroxyproline on collagens. Contributes to proline 3-hydroxylation of collagen COL4A1 and COL1A1 in tendons, the eye sclera and in the eye lens capsule. Has high activity with the type IV collagen COL4A1, and lower activity with COL1A1. Catalyzes hydroxylation of the first Pro in Gly-Pro-Hyp sequences where Hyp is 4-hydroxyproline. Has no activity on substrates that lack 4-hydroxyproline in the third position.
  • 基因功能参考文献：
    1. Data identified an enhancer region within the TP63/LEPREL1 locus containing genetic variants associated with bladder cancer risk. PMID: 29956121
    2. Recessive LEPREL1 mutations should be recognized as part of the differential diagnosis of lens subluxation. PMID: 25469533
    3. LEPREL1 plays an important role in eye development and homozygous loss-of-function mutation of this gene can cause severely high myopia and early-onset cataract. PMID: 24172257
    4. mutation in LRPAP1 is associated with high myopia. Further studies are expected to evaluate the pathogenicity of the variants in CTSH, LEPREL1, ZNF644, SLC39A5, and SCO2. PMID: 25525168
    5. High myopia is caused by a mutation in LEPREL1, encoding prolyl 3-hydroxylase 2. PMID: 21885030
    6. P3H2 has preferred substrate sequences among the classes of 3Hyp sites in clade A collagen chains PMID: 21757687
    7. P3H2 is responsible for the hydroxylation of collagen IV, which has the highest 3-hydroxyproline content of all collagens. It is thus possible that P3H2 mutations may lead to a disease with changes in basement membranes. PMID: 18487197
    8. The restriction of silencing in P3H2 to breast carcinomas, and its association with oestrogen-receptor-positive cases, suggests that P3H2 may be a breast-cancer-specific tumour suppressor. PMID: 19436308

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  • 相关疾病：
    Myopia, high, with cataract and vitreoretinal degeneration (MCVD)
  • 亚细胞定位：
    Endoplasmic reticulum. Sarcoplasmic reticulum. Golgi apparatus.
  • 蛋白家族：
    Leprecan family
  • 组织特异性：
    Expression localized to the epithelia of bile ducts and to the sacroplasm of heart muscle and skeletal muscle. In the pancreas, localized to a subpopulation of Langerhans islet cells and in the salivary gland, expressed in acinar cells (at protein level).
  • 数据库链接：

    HGNC: 19317

    OMIM: 610341

    KEGG: hsa:55214

    STRING: 9606.ENSP00000316881

    UniGene: Hs.374191