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. CHST14 Antibody

CHST14 Antibody

  • 中文名稱：
    CHST14兔多克隆抗體
  • 貨號：
    CSB-PA839851LA01HU
  • 規格：
    ￥440
  • 圖片：
    • Western Blot
      Positive WB detected in: A549 whole cell lysate
      All lanes: CHST14 antibody at 4.8µg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 43 kDa
      Observed band size: 43 kDa
    • IHC image of CSB-PA839851LA01HU diluted at 1:800 and staining in paraffin-embedded human pancreatic 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.
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) CHST14 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    CHST14
  • 別名：
    2600016L03Rik antibody; ATCS antibody; Carbohydrate (N acetylgalactosamine 4 0) sulfotransferase 14 antibody; Carbohydrate sulfotransferase 14 antibody; CHST14 antibody; CHSTE_HUMAN antibody; D4ST-1 antibody; D4st1 antibody; Dermatan 4-sulfotransferase 1 antibody; hD4ST1 antibody; RP23-286G12.2 antibody; UNQ1925/PRO4400 antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human Carbohydrate sulfotransferase 14 protein (70-133AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated

    本頁面中的產品，CHST14 Antibody (CSB-PA839851LA01HU)，的標記方式是Non-conjugated。對于CHST14 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-PA839851LB01HU CHST14 Antibody, HRP conjugated ELISA
    FITC CSB-PA839851LC01HU CHST14 Antibody, FITC conjugated
    Biotin CSB-PA839851LD01HU CHST14 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
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:500-1:1000
  • 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：
    Catalyzes the transfer of sulfate to position 4 of the N-acetylgalactosamine (GalNAc) residue of dermatan sulfate. Plays a pivotal role in the formation of 4-0-sulfated IdoA blocks in dermatan sulfate. Transfers sulfate to the C-4 hydroxyl of beta1,4-linked GalNAc that is substituted with an alpha-linked iduronic acid (IdoUA) at the C-3 hydroxyl. Transfers sulfate more efficiently to GalNAc residues in -IdoUA-GalNAc-IdoUA- than in -GlcUA-GalNAc-GlcUA-sequences. Has preference for partially desulfated dermatan sulfate. Addition of sulfate to GalNAc may occur immediately after epimerization of GlcUA to IdoUA. Appears to have an important role in the formation of the cerebellar neural network during postnatal brain development.
  • 基因功能參考文獻：
    1. Dermatan sulfate does not appear in urine of patients with Ehlers-Danlos syndrome caused by a CHST14/D4ST1 deficiency. PMID: 28238810
    2. patients with CHST14/D4ST1 deficiency develop progressive multisystem fragility-related manifestations, establishment of a comprehensive and detailed natural history and health-care guidelines as well as further elucidation of the pathophysiology in view of future etiology-based therapy are crucial PMID: 26646600
    3. CHST14 gene mutations are associated with musculocontractural type of Ehlers-Danlos syndrome. PMID: 26373698
    4. We report on the detailed clinical characterization of two sisters with musculocontractural Ehlers-Danlos syndrome caused by a homozygous mutation in the CHST14 gene. PMID: 22581468
    5. Loss of dermatan-4-sulfotransferase 1 (D4ST1/CHST14) function represents the first dermatan sulfate biosynthesis defect, "dermatan sulfate-deficient adducted thumb-clubfoot syndrome". PMID: 21309034
    6. Musculocontractural Ehlers-Danlos Syndrome (former EDS type VIB) and adducted thumb clubfoot syndrome (ATCS) represent a single clinical entity caused by mutations in the dermatan-4-sulfotransferase 1 encoding CHST14 gene. PMID: 20842734
    7. A homozygous CHST14 (carbohydrate sulfotransferase 14) mutation in the two familial cases and compound heterozygous mutations in four sporadic cases of Ehlers-Danlos syndrome, were identified. PMID: 20533528
    8. D4ST-1 is a key enzyme and is indispensable in the formation of important functional domains in dermatan sulfate and cannot be compensated by other 4-O-sulfotransferases PMID: 19661164
    9. dermatan-4-sulfotransferase 1 has a role in adducted thumb-clubfoot syndrome PMID: 20004762
    10. D4ST-1 is encoded by a single exon located on human chromosome 15q14; type II membrane protein of 376 amino acids with a 43-amino acid cytoplasmic domain and a 316-amino acid luminal domain containing two potential N-linked glycosylation sites PMID: 11470797

    顯示更多

    收起更多

  • 相關疾病：
    Ehlers-Danlos syndrome, musculocontractural type 1 (EDSMC1)
  • 亞細胞定位：
    Golgi apparatus membrane; Single-pass type II membrane protein.
  • 蛋白家族：
    Sulfotransferase 2 family
  • 組織特異性：
    Widely expressed. Expressed at high level in pituitary gland, placenta, uterus and thyroid.
  • 數據庫鏈接：

    HGNC: 24464

    OMIM: 601776

    KEGG: hsa:113189

    STRING: 9606.ENSP00000307297

    UniGene: Hs.442449



主站蜘蛛池模板： 玩弄少妇人妻| 成人视频在线观看| 国产伦子系列沙发午睡| 大学生被内谢粉嫩无套| 高清国产亚洲精品自在久久| 久久无码潮喷a片无码高潮 | 国产在线拍偷自揄拍无码| 在线偷着国产精选视频| 亚洲国产精品va在线观看麻豆| 欧美z0zo人禽交免费观看99| 无码专区久久综合久中文字幕| 久久av无码精品人妻系列| yy111111少妇无码理论片| 国产男生午夜福利免费网站| 日本亚洲欧洲另类图片| 免费国产va在线观看中文字| 国精产品一区一区三区免费视频| 国产成人免费视频| 美女裸体跪姿扒开屁股无内裤| 人人玩人人添人人澡超碰偷拍| 国产在线观看免费视频软件 | 精品人妻无码专区在线无广告视频 | 久久永久免费专区人妻精品| 久久国产福利播放| 偷国产乱人伦偷精品视频 | 免费无码又爽又刺激高潮的动态图| 超清无码波多野吉衣中文| 国产精品久久久午夜夜伦鲁鲁| 又黄又爽又无遮挡免费的网站| 亚洲精品无码mv在线观看| 中国china体内裑精亚洲日本| 亚洲精品无码鲁网中文电影| 色噜噜狠狠狠狠色综合久| 国产精品久久久久久久久久久不卡 | 日韩精品一区二区三区在线观看l| 18禁无遮挡肉动漫在线播放观看| 狠狠色噜噜狠狠狠狠五月婷| 色狠狠成人综合网| 国产人妻aⅴ色偷| 亚洲美女高清aⅴ视频免费| 国产熟睡乱子伦午夜视频麻豆|