国产午夜福利片1000无码丨人妻国产成人久久av免费高清丨午夜做受视频试看6次丨日韩av不卡一区在线免费观看丨在线观看精品三级欧美

Your Good Partner in Biology Research

PORCN Antibody

  • 中文名稱:
    PORCN兔多克隆抗體
  • 貨號:
    CSB-PA887958LA01HU
  • 規格:
    ¥440
  • 圖片:
    • 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:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) PORCN Polyclonal antibody
  • Uniprot No.:
  • 基因名:
  • 別名:
    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
  • 反應種屬:
    Human, Mouse
  • 免疫原:
    Recombinant Human Protein-serine O-palmitoleoyltransferase porcupine protein (269-332AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    Non-conjugated

    本頁面中的產品,PORCN Antibody (CSB-PA887958LA01HU),的標記方式是Non-conjugated。對于PORCN Antibody,我們還提供其他標記。見下表:

    可提供標記
    標記方式 貨號 產品名稱 應用
    HRP CSB-PA887958LB01HU PORCN Antibody, HRP conjugated ELISA
    FITC CSB-PA887958LC01HU PORCN Antibody, FITC conjugated
    Biotin CSB-PA887958LD01HU PORCN Antibody, Biotin conjugated ELISA
  • 克隆類型:
    Polyclonal
  • 抗體亞型:
    IgG
  • 純化方式:
    >95%, Protein G purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA, WB, IHC, IF
  • 推薦稀釋比:
    Application Recommended Dilution
    WB 1:500-1:2000
    IHC 1:20-1:200
    IF 1:50-1:200
  • Protocols:
  • 儲存條件:
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期:
    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.
  • 用途:
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 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.
  • 基因功能參考文獻:
    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

    顯示更多

    收起更多

  • 相關疾病:
    Focal dermal hypoplasia (FODH)
  • 亞細胞定位:
    Endoplasmic reticulum membrane; Multi-pass membrane protein.
  • 蛋白家族:
    Membrane-bound acyltransferase family, Porcupine subfamily
  • 組織特異性:
    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
  • 數據庫鏈接:

    HGNC: 17652

    OMIM: 300651

    KEGG: hsa:64840

    STRING: 9606.ENSP00000322304

    UniGene: Hs.386453



主站蜘蛛池模板: 璧山县| 和静县| 曲松县| 攀枝花市| 汤阴县| 蓬溪县| 揭阳市| 大化| 宣恩县| 阳江市| 青神县| 库尔勒市| 琼结县| 闸北区| 南川市| 新化县| 工布江达县| 红原县| 华池县| 博客| 武威市| 乐昌市| 孝感市| 永嘉县| 乐山市| 鄂尔多斯市| 绥棱县| 错那县| 廊坊市| 都匀市| 和硕县| 六盘水市| 三江| 砚山县| 青铜峡市| 基隆市| 万山特区| 桃园县| 双牌县| 乡宁县| 大关县|