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, Biotin conjugated

CHST14 Antibody, Biotin conjugated

  • 中文名稱：
    CHST14兔多克隆抗體, Biotin偶聯(lián)
  • 貨號：
    CSB-PA839851LD01HU
  • 規(guī)格：
    ￥880
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)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(yīng)種屬：
    Human
  • 免疫原：
    Recombinant Human Carbohydrate sulfotransferase 14 protein (70-133AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)記方式：
    Biotin
  • 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(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA
  • 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(chǎn)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(xiàn)：
    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(guān)疾病：
    Ehlers-Danlos syndrome, musculocontractural type 1 (EDSMC1)
  • 亞細(xì)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(shù)據(jù)庫鏈接：

    HGNC: 24464

    OMIM: 601776

    KEGG: hsa:113189

    STRING: 9606.ENSP00000307297

    UniGene: Hs.442449



主站蜘蛛池模板： 成在线人免费视频播放| 亚洲国产婷婷香蕉久久久久久| 日日拍夜夜嗷嗷叫|日日摸| 国产欧美日韩亚洲精品专区| 中国白嫩丰满人妻videos| 99re久久精品国产| av大片在线无码永久免费| 最新国产久免费视频在线观看| 97人摸人人澡人人人超碰| 中国china体内裑精亚洲日本| 欧美乱码卡一卡二卡三新区| 久久久久久久综合色一本| 成av人片在线观看www| 99精品国产丝袜在线拍国语| 亚洲精品中国国产嫩草影院美女| 毛片免费视频肛交颜射免费视频| 亚洲国产成人久久综合三区| 久久久精品456亚洲影院| 夜夜躁狠狠躁日日躁视频| 国产精品久久国产精品99 gif| 高清破外女出血av毛片| 综合 欧美 小说 另类 图| 影音先锋亚洲成aⅴ人在| 妺妺窝人体色www在线小说| 免费看美女部位隐私网站| 欧美人成精品网站播放| 大又大粗又爽又黄少妇毛片| 欧美巨大xxxx做受高清| 欧美乱人伦视频在线| 最新国产av无码专区亚洲| 内射无码专区久久亚洲| 国产在线观看黄av免费| 国产精品18久久久久久欧美| 无码丰满熟妇juliaann与黑人| 亚洲第一网站男人都懂| 2018高清日本一道国产-在 | 伊人婷婷六月狠狠狠去| 18禁成人网站免费观看| 久久夜色撩人精品国产小说| 国精产品蘑菇一区一区有限| 成人无码av片在线观看|