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： 奇米影视7777久久精品人人爽| 日本免费一区二区三区最新| 美女裸体自慰在线观看| 97无码免费人妻超级碰碰夜夜| 中文字幕三级人妻无码视频| 色舞月亚洲综合一区二区| 欧美乱强伦xxxx孕妇| 中文天堂网www新版资源在线| 免费又黄又爽又色的视频| 51久久成人国产精品麻豆| 国产mv在线天堂mv免费观看| 国产在线观看免费视频软件| 性色av一区二区三区咪爱四虎| 国产精品三级一区二区| 蜜桃av抽搐高潮一区二区| k频道国产在线观看| 国产极品美女到高潮无套| 欧美黑人巨大videos极品| 国产一起色一起爱| 日日摸日日碰夜夜爽av| 中国极品少妇xxxxx| 老司机亚洲精品影院无码| 女人爽到喷水的视频大全| 久久人人爽人人爽爽久久小说 | 国产午夜永久福利视频在线观看| 亚洲精品成人区在线观看| 久久精品丝袜高跟鞋| 国产成人精品福利一区二区三区 | 性动态图av无码专区| 免费久久人人爽人人爽av| 国产97在线 | 日韩| 国产精品久久人妻无码网站蜜臀| 天天做日日做天天添天天欢公交车| 荫蒂被男人添的好舒服爽免费视频| 中文字幕人成乱码熟女香港 | 人妻少妇无码专视频在线| 亚洲女同一区二区| 非洲黑人最猛性xxxx交| 色爱情人网站| 一本色道久久综合亚州精品蜜桃| 狠狠色噜噜狠狠狠狠777米奇小说 又黄又爽的60分钟视频 |