在线日韩日本国产亚洲丨少妇伦子伦情品无吗丨欧美性猛交xxxx免费看蜜桃丨精品人妻系列无码一区二区三区丨亚洲精品无码不卡在线播放

Your Good Partner in Biology Research

  1. Home
  2. 抗體
  3. 多克隆抗體
  4. EVC2 Antibody

EVC2 Antibody

  • 中文名稱：
    EVC2兔多克隆抗體
  • 貨號：
    CSB-PA769777LA01HU
  • 規格：
    ￥440
  • 圖片：
    • IHC image of CSB-PA769777LA01HU diluted at 1:200 and staining in paraffin-embedded human prostate 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.
    • IHC image of CSB-PA769777LA01HU diluted at 1:200 and staining in paraffin-embedded human prostate cancer 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.
    • Immunofluorescence staining of A549 cells with CSB-PA769777LA01HU at 1:66, 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) EVC2 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
  • 別名：
    EVC2 antibody; LBN antibody; Limbin antibody; Ellis-van Creveld syndrome protein 2 antibody; EVC2 antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human Limbin protein (30-199AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated

    本頁面中的產品，EVC2 Antibody (CSB-PA769777LA01HU)，的標記方式是Non-conjugated。對于EVC2 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-PA769777LB01HU EVC2 Antibody, HRP conjugated ELISA
    FITC CSB-PA769777LC01HU EVC2 Antibody, FITC conjugated
    Biotin CSB-PA769777LD01HU EVC2 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, IHC, IF
  • 推薦稀釋比：
    Application Recommended Dilution
    IHC 1:200-1:500
    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：
    Component of the EvC complex that positively regulates ciliary Hedgehog (Hh) signaling. Plays a critical role in bone formation and skeletal development. May be involved in early embryonic morphogenesis.
  • 基因功能參考文獻：
    1. he whole exome sequencing (WES) in this family revealed two homozygous variants in EVC2 (c.30dupC; p.Thr11Hisfs*45) and TMC1 (c.1696-1G>A) genes. In family B, WES revealed novel compound heterozygous variants (p.Ser307Pro, c.2894+3A>G) in the EVC gene. PMID: 29321360
    2. sequence analysis identified a novel nonsense mutation (p.Trp234*) in exon 8 of the EVC2 gene and 15 bp duplication in exon 14 of the EVC gene in the two families.. PMID: 26580685
    3. Sequencing of both EVC and EVC2 identified two novel heterozygous splice site mutations c.384+5G>C in intron 3 and c.1465-1G>A in intron 10 in EVC, which were inherited from mother and father, respectively. PMID: 26621368
    4. we identified 2 independent mutations in EVC2 gene in patients with WAD, including one novel. PMID: 23220543
    5. Identification of a novel genotype in EvC will provide clues to phenotype-genotype relations and may assist not only in the clinical diagnosis of EvC but also in the interpretation of genetic information used for prenatal diagnosis and genetic counseling PMID: 23026208
    6. Emerging molecular and developmental studies suggest that EVC and EVC2 proteins may be important for cilia function, which is implicated in the pathogenesis of heterotaxy syndromes. PMID: 21533779
    7. In this study, two novel heterozygous EVC2 mutations, IVS 5-2A > G and c.2653C > T (Arg88 5X), were identified in the patient; the IVS5-2A > G mutation was inherited from the patient's mother and the c.2653C > T from her father PMID: 21815252
    8. Molecular analysis of the EVC and EVC2 genes is helpful in genetic counseling in cases with prenatally detected postaxial polydactyly, thoracic narrowness, short limbs and endocardial cushion defects. PMID: 21199751
    9. STK32b, EVC and EVC2 genes yielded suggestive evidence for linkage and disepuilibrium among cleft palate trios. PMID: 20087401
    10. A novel splice site mutation (c.2047-1G>T) in intron 13 of EVC2 gene was found in a family with child diagnosed with Ellis-van Creveld syndrome in the United Arab Emirates. PMID: 20184732
    11. The expression of a Weyer variant, but not the expression of a truncated protein that mimics an Ellis-van Creveld syndrome mutation, impairs Hedgehog signal transduction in NIH 3T3 cells in keeping with its dominant effect. PMID: 19810119
    12. EVC2 is mutated in an Ashkenazi individual with Ellis-van Creveld syndrome PMID: 12468274
    13. Mutations in this gene cause Ellis-van Creveld syndrome. PMID: 12571802
    14. Data provide conclusive evidence that Weyers acrofacial dysostosis and EvC syndrome are allelic and genetically heterogeneous conditions. PMID: 16404586
    15. EVC and LBN play roles in cardiovascular development and disease. PMID: 19251731

    顯示更多

    收起更多

  • 相關疾病：
    Ellis-van Creveld syndrome (EVC); Acrofacial dysostosis, Weyers type (WAD)
  • 亞細胞定位：
    Cell membrane; Single-pass type I membrane protein. Cytoplasm, cytoskeleton, cilium basal body. Cell projection, cilium. Cell projection, cilium membrane. Nucleus.
  • 組織特異性：
    Found in the heart, placenta, lung, liver, skeletal muscle, kidney and pancreas.
  • 數據庫鏈接：

    HGNC: 19747

    OMIM: 193530

    KEGG: hsa:132884

    STRING: 9606.ENSP00000342144

    UniGene: Hs.87306



主站蜘蛛池模板： 欧美人与动牲交a精品| 大胸美女污污污www网站| 老湿机香蕉久久久久久| 亚洲精品久久久久久中文传媒| 女邻居丰满的奶水| 国内偷自第一区二区三区| 亚洲中文有码字幕青青| 久久99精品久久久久蜜芽| 国产成人精品123区免费视频| 人妻在客厅被c的呻吟| 国产成在线观看免费视频| 性色香蕉av久久久天天网| 97久久精品人人澡人人爽| 国产精品久久久久久久妇| 免费无码又爽又刺激软件下载| 久久大香国产成人av| xx性欧美肥妇精品久久久久久| 欧美丰腴丰满大屁股熟妇| 国产成人欧美日韩在线电影| 欧美精品videossex少妇| 国产午夜精品无码| 亂倫近親相姦中文字幕| 无码区日韩特区永久免费系列| 极品无码国模国产在线观看 | 在线精品国产一区二区三区| 国产亚洲一区二区在线观看| 国产人成网线在线播放va| 无码午夜人妻一区二区不卡视频| 8888四色奇米在线观看| 久久久这里只有免费精品| 西西444www大胆无码视频| 人人超人人超碰超国产| 久久国产成人午夜av影院| 色欲aⅴ亚洲情无码av蜜桃| 国产av偷闻女邻居内裤被发现| 18禁超污无遮挡无码免费游戏| 亚洲综合色自拍一区| 老司机久久一区二区三区| 99在线精品国自产拍| 久久国产精品偷| 137裸交肉体摄影|