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. ISPD Antibody, HRP conjugated

ISPD Antibody, HRP conjugated

  • 中文名稱：
    ISPD兔多克隆抗體, HRP偶聯
  • 貨號：
    CSB-PA388827LB01HU
  • 規格：
    ￥880
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) ISPD Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    ISPD
  • 別名：
    2-C-methyl-D-erythritol 4-phosphate cytidylyltransferase-like protein antibody; Isoprenoid synthase domain-containing protein antibody; ispD antibody; ISPD_HUMAN antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human Isoprenoid synthase domain-containing protein (1-264AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    HRP
  • 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
  • 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：
    Cytidylyltransferase required for protein O-linked mannosylation. Catalyzes the formation of CDP-ribitol nucleotide sugar from D-ribitol 5-phosphate. CDP-ribitol is a substrate of FKTN during the biosynthesis of the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1), which is required for binding laminin G-like domain-containing extracellular proteins with high affinity. Shows activity toward other pentose phosphate sugars and mediates formation of CDP-ribulose or CDP-ribose using CTP and ribulose-5-phosphate or ribose-5-phosphate, respectively. Not Involved in dolichol production.
  • 基因功能參考文獻：
    1. ISPD and FKTN are essential for the incorporation of ribitol into alpha-dystroglycan. PMID: 27194101
    2. data suggest that the genetic heterogeneity of Limb Girdle Muscular Dystrophy with and without alpha-DG defects is greater than previously realized. PMID: 26404900
    3. ISPD gene homozygous deletion as a prenatal manifestation of Walker-Warburg syndrome has been found in 3 female fetuses of one family. PMID: 26087224
    4. Reduced levels of GYLTL1B and ISPD mRNA associated with increased patient mortality and are the likely cause of alphaDG hypoglycosylation in ccRCC. PMID: 26220087
    5. study describes a new homozygous missense mutation c.367G>A (p.Gly123Arg) in the ISPD gene in a family of Pakistani origin with 2 cousins from consanguineous parents affected with a congenital muscular dystrophy (CMD) /early limb-girdle muscular dystrophy intermediate phenotype and CMD respectively PMID: 25444434
    6. study report the involvement of the ISPD gene in milder dystroglycanopathy phenotypes ranging from congenital muscular dystrophy to limb-girdle muscular dystrophy and identified allelic ISPD variants in nine cases belonging to seven families PMID: 23288328
    7. we identified a novel homozygous c.161G>C/p.G54A variant in ISPD in patients with limb-girdle muscular dystrophy PMID: 23390185
    8. TMEM5 mutations were frequently associated with gonadal dysgenesis and neural tube defects, and ISPD mutations were frequently associated with brain vascular anomalies. PMID: 23217329
    9. ISPD loss-of-function mutations disrupt dystroglycan O-mannosylation and cause Walker-Warburg syndrome. PMID: 22522420
    10. Mutations in ISPD cause Walker-Warburg syndrome and defective glycosylation of alpha-dystroglycan. PMID: 22522421
    11. Observational study of gene-disease association. (HuGE Navigator) PMID: 19913121
    12. Observational study of gene-disease association, gene-environment interaction, and pharmacogenomic / toxicogenomic. (HuGE Navigator) PMID: 20628086
    13. Clinical trial of gene-disease association and gene-environment interaction. (HuGE Navigator) PMID: 20379614

    顯示更多

    收起更多

  • 相關疾病：
    Muscular dystrophy-dystroglycanopathy congenital with brain and eye anomalies A7 (MDDGA7); Muscular dystrophy-dystroglycanopathy limb-girdle C7 (MDDGC7)
  • 亞細胞定位：
    Cytoplasm, cytosol.
  • 蛋白家族：
    IspD/TarI cytidylyltransferase family, IspD subfamily
  • 組織特異性：
    Ubiquitously expressed, with high expression in brain.
  • 數據庫鏈接：

    HGNC: 37276

    OMIM: 614631

    KEGG: hsa:729920

    STRING: 9606.ENSP00000385478

    UniGene: Hs.636502



主站蜘蛛池模板： 鲁一鲁av2019在线| 亚洲精品一区国产精品| 牛牛在线视频| 一出一进一爽一粗一大视频免费的| 亚洲a成人无码网站在线| 亚洲精品无码伊人久久| 国产精品久久久久高潮| 亚欧免费无码aⅴ在线观看 | 色一情一区二区三区四区| 天天综合亚洲色在线精品| 国产美女露脸口爆吞精| 2019最新中文字幕在线观看| 日日碰狠狠添天天爽| 国产精品刮毛| 国内精品久久久久影院蜜芽| 成人网站www污污污网站| 67194熟妇在线观看线路1 | 无码国产乱人伦偷精品视频| 99久久无色码中文字幕人妻蜜柚| 成人网站免费高清视频在线观看 | 久久久无码精品国产一区 | 性av无码天堂vr专区| 丰满熟妇被猛烈进入高清片| 97人妻精品一区二区三区| 精品国际久久久久999| 18禁男女污污污午夜网站免费暖暖| 嫩草国产露脸精品国产软件| 九九久久精品无码专区| 影音先锋男人站| 免费能直接看黄的视频| 久久精晶国产99久久6| 国产精品久久国产精品99 gif| 国产av高清无亚洲| 亚洲日韩中文字幕久热| 国产精品熟妇一区二区三区四区| 精品人伦一区二区三区潘金莲 | 日本韩国的免费观看视频| 噜噜久久噜噜久久鬼88| 国产国语老龄妇女a片| 亚洲第一无码专区天堂| 国内精品免费网站牛牛|