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-PA005407GA01HU
  • 規格：
    ￥3,900
  • 其他：

產品詳情

  • 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,Mouse,Rat
  • 免疫原：
    Human CHST14
  • 免疫原種屬：
    Homo sapiens (Human)
  • 抗體亞型：
    IgG
  • 純化方式：
    Antigen Affinity purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 產品提供形式：
    Liquid
  • 應用范圍：
    ELISA,WB,IHC
  • 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： 美女视频黄频a美女大全| 国产最新精品自产在线观看| 国产山东熟女48嗷嗷叫| 少妇无码av无码专区| 欧美激性欧美激情在线| 国产巨大爆乳在线观看| 影视av久久久噜噜噜噜噜三级| 国产乱色国产精品播放视频| 国产乱视频在线观看| 饥渴少妇av无码影片| 亚洲欧美中文高清在线专区| 熟妇无码乱子成人精品| 小蜜被两老头吸奶头在线观看| 午夜a理论片在线播放| 米奇影院888奇米色99在线| 人妻人人做人碰人人添| 麻豆精品国产精华精华液好用吗 | 无码制服丝袜人妻在线视频精品| 人妻av无码专区| 少妇av一区二区三区无码| 国产做爰xxxⅹ高潮视频12p| 国产午夜亚洲精品国产成人| 久久久久久久性潮| 亚洲码国产精品高潮在线| 国语自产拍精品香蕉在线播放| 四虎成人国产精品永久在线| 337p日本欧洲亚洲大胆裸体艺术| 亚洲爆乳无码一区二区三区| 国产成人最新三级在线视频| 小嫩妇好紧好爽18禁视频| 欧美人与动性xxxxx杂| 妺妺跟我一起洗澡没忍住| 亚洲午夜久久久无码精品网红a片| 免费看欧美成人a片无码| 性色欲情网站iwww| 亚洲色欲色欲天天天www| 欧洲无码一区二区三区在线观看| 欧美大浪妇猛交饥渴大叫| 日本妇人成熟免费中文字幕| 中文字字幕在线成人av电影| 亚洲色成人网站www永久四虎|