Your Good Partner in Biology Research

  1. Home
  2. 抗体
  3. 多克隆抗体
  4. PORCN Antibody

PORCN Antibody

  • 货号：
    CSB-PA887958LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western blot
      All lanes: PORCN antibody at 3μg/ml + Mouse thymus tissue
      Secondary
      Goat polyclonal to rabbit IgG at 1/10000 dilution
      Predicted band size: 53, 52, 43 kDa
      Observed band size: 53 kDa
    • Immunohistochemistry of paraffin-embedded human testis tissue using CSB-PA887958LA01HU at dilution of 1:100
    • Immunofluorescence staining of A549 cells with CSB-PA887958LA01HU at 1:100, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) PORCN Polyclonal antibody
  • Uniprot No.：
    Q9H237
  • 基因名：
  • 别名：
    DHOF antibody; FODH antibody; MG61 antibody; MGC29687 antibody; por antibody; PORC antibody; PORCN antibody; PORCN_HUMAN antibody; PPN antibody; Probable protein-cysteine N-palmitoyltransferase porcupine antibody; Protein MG61 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human, Mouse
  • 免疫原：
    Recombinant Human Protein-serine O-palmitoleoyltransferase porcupine protein (269-332AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，PORCN Antibody (CSB-PA887958LA01HU)，的标记方式是Non-conjugated。对于PORCN 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-PA887958LB01HU PORCN Antibody, HRP conjugated ELISA
    FITC CSB-PA887958LC01HU PORCN Antibody, FITC conjugated
    Biotin CSB-PA887958LD01HU PORCN 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, IF
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:2000
    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：
    Protein-serine O-palmitoleoyltransferase that acts as a key regulator of the Wnt signaling pathway by mediating the attachment of palmitoleate, a 16-carbon monounsaturated fatty acid (C16:1), to Wnt proteins. Serine palmitoleylation of WNT proteins is required for efficient binding to frizzled receptors.
  • 基因功能参考文献：
    1. Case Report: mosaicism for PORCN mutations in focal dermal hypoplasia (Goltz Syndrome). PMID: 28293688
    2. Data suggest that PORCN exhibits substrate specificity that includes a Wnt3a peptide fragment (residues 199-219, with disulfide bonds); recombinant PORCN containing a point mutation (R228C) associated with focal dermal hypoplasia exhibits impaired acylation activity toward Wnt3a peptide fragment. (PORCN = porcupine O-acyltransferase; Wnt3a = Wnt family member 3A) PMID: 28655768
    3. Inhibition of Wnt signaling by PORCN inhibition holds promise as differentiation therapy in genetically defined human cancers PMID: 26257057
    4. porcupine-mediated production of Wnts is context dependent and is not required for all Wnts production, suggesting that alternative mechanisms exist for Wnts production. PMID: 24647048
    5. We describe the first case of non-mosaic males affected with syndromic microphthalmia because of a non-synonymous variant in the PORCN gene. PMID: 25026905
    6. the Wnt amino acid residues required for recognition and palmitoylation by PORCN PMID: 25451226
    7. We describe the ophthalmologic findings in an 18-month-old boy with mosaicism of a novel mutation in PORCN. PMID: 24698628
    8. a novel variant in the PORCN gene (c.1250T>C:p.F417S) in the focal dermal hypoplasia with spinal anomaly PMID: 23696273
    9. We report a typical focal dermal hypoplasia (FDH) patient with a recurrent PORCN mutation, which was previously identified, and a second female, with an almost unilateral FDH and a novel postzygotic PORCN mutation. PMID: 23399492
    10. To the best of our knowledge, this is the second case report that reveals a mutation of the PORCN gene in a patient with almost unilateral focal dermal hypoplasia. PMID: 22735390
    11. PORCN protein thus appears to moonlight in a novel signaling pathway that is rate-limiting for cancer cell growth and tumorigenesis independent of its enzymatic function in Wnt biosynthesis and secretion PMID: 22509316
    12. review of the published mutations in the PORCN gene and report on 7 new mutations identified in Goltz-Gorlin syndrome patients PMID: 21472892
    13. 12 novel PORCN mutations and 6 previously reported mutations were found in 53 unrelated focal dermal hypoplasia patients. PMID: 20854095
    14. Porcupine might contribute to non-small cell lung carcinoma development by ranscriptional activation of cancer-related genes such as s100P. PMID: 20198348
    15. Three de novo mutations were identified in PORCN gene in patients with focal dermal hypoplasia. PMID: 19863546
    16. Sequence deletions and point mutations cause focal dermal hypoplasia. PMID: 17546030
    17. PORCN, encoding a putative O-acyltransferase potentially crucial for cellular export of Wnt signaling proteins, is the gene mutated in focal dermal hypoplasia. PMID: 17546031
    18. Overexpression of PORCN is associated with lung cancer PMID: 18193088
    19. 3 novel mutations in PORCN, c.373+1G>A, c.737_738insA & c.1094G>A (p.R365Q), were identified in focal dermal hypoplasia patients(FDH); study shows PORCN is gene responsible for FDH in different populations & extends number of confirmed mutations to 26 PMID: 18325042
    20. defective PORCN does not lead to selective growth disadvantage PMID: 19277062
    21. Molecular characterization of 24 unrelated patients from different ethnic backgrounds revealed 23 different mutations of the PORCN gene in Xp11.23. PMID: 19309688
    22. Mutations within the PORCN gene are associated with Goltz-Gorlin syndrome. PMID: 19586929
    23. Focal dermal hypoplasia illustrates the phenotypic consequences of defective modulation of Wnt signaling in utero and highlights the important roles of PORCN and Wnt signaling pathways in embryogenesis. PMID: 19681149

    显示更多

    收起更多

  • 相关疾病：
    Focal dermal hypoplasia (FODH)
  • 亚细胞定位：
    Endoplasmic reticulum membrane; Multi-pass membrane protein.
  • 蛋白家族：
    Membrane-bound acyltransferase family, Porcupine subfamily
  • 组织特异性：
    Isoform 1 is expressed in fetal brain, brain, amygdala, caudate nucleus, cerebellum, hippocampus, pituitary, thalamus, heart, skeletal muscle and testis. Isoform 4 is expressed in amygdala, corpus callosum, hippocampus, spinal cord, kidney, liver, lung, s
  • 数据库链接：

    HGNC: 17652

    OMIM: 300651

    KEGG: hsa:64840

    STRING: 9606.ENSP00000322304

    UniGene: Hs.386453