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

UROS Antibody, FITC conjugated

  • 中文名稱:
    UROS兔多克隆抗體, FITC偶聯
  • 貨號:
    CSB-PA025679LC01HU
  • 規格:
    ¥880
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) UROS Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    UROS
  • 別名:
    congenital erythropoietic porphyria antibody; HEM4_HUMAN antibody; Hydroxymethylbilane hydrolyase [cyclizing] antibody; Hydroxymethylbilane hydrolyase antibody; OTTHUMP00000020709 antibody; OTTHUMP00000020710 antibody; UROIIIS antibody; Uroporphyrinogen III cosynthetase antibody; Uroporphyrinogen III synthase (congenital erythropoietic porphyria) antibody; Uroporphyrinogen III synthase antibody; Uroporphyrinogen-III cosynthase antibody; Uroporphyrinogen-III synthase antibody; UROS antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human
  • 免疫原:
    Recombinant Human Uroporphyrinogen-III synthase protein (1-265AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    FITC
  • 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
  • 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 cyclization of the linear tetrapyrrole, hydroxymethylbilane, to the macrocyclic uroporphyrinogen III, the branch point for the various sub-pathways leading to the wide diversity of porphyrins. Porphyrins act as cofactors for a multitude of enzymes that perform a variety of processes within the cell such as methionine synthesis (vitamin B12) or oxygen transport (heme).
  • 基因功能參考文獻:
    1. we show evidence that abnormal protein homeostasis is a prevalent mechanism responsible for UROS deficiency and that modulators of UROS proteolysis such as proteasome inhibitors or chemical chaperones may represent an attractive therapeutic option to reduce porphyrin accumulation and prevent skin photosensitivity in Congenital erythropoietic porphyria (CEP)patients when the genotype includes a missense variant. PMID: 28334762
    2. A, T to C change at nucleotide 34313, leading to a substitution of Leucine by Proline at codon 237, was observed in the homozygous state in a family with congenital erythropoietic porphyria. PMID: 22350154
    3. REVIEW: Structural, thermodynamic, and mechanistical studies in uroporphyrinogen III synthase and molecular basis of congenital erythropoietic porphyria PMID: 21570665
    4. Intracellular rescue of the uroporphyrinogen III synthase activity in enzymes carrying the hotspot mutation C73R. PMID: 21343304
    5. Data show that branchpoint sequence (BPS)mutation reduced the wild-type transcript and UROS enzyme activity in CEP lymphoblasts to approximately 10% and 15% of normal, respectively. PMID: 19965637
    6. two novel misense mutations in the UROS gene. PMID: 17298225
    7. NMR analyses of URO-synthase titrated with competitive inhibitors N(D)-methyl-1-formylbilane or URO'gen III, revealed resonance perturbations of specific residues lining the cleft between 2 major domains of URO synthase that mapped enzyme's active site. PMID: 18004775
    8. The decrease in the expression of ubiquitous HMBS and UROS mRNAs under hypoxia is associated with accumulation of hypoxia-inducible factor 1alpha protein. PMID: 19021769
    9. UROS mutations related to erythropoietic porphyria identify a key helix for protein stability. PMID: 19099412

    顯示更多

    收起更多

  • 相關疾病:
    Congenital erythropoietic porphyria (CEP)
  • 蛋白家族:
    Uroporphyrinogen-III synthase family
  • 組織特異性:
    Ubiquitous.
  • 數據庫鏈接:

    HGNC: 12592

    OMIM: 263700

    KEGG: hsa:7390

    STRING: 9606.ENSP00000357775

    UniGene: Hs.501376



主站蜘蛛池模板: 久久www免费人成_看片中文| 先锋影音xfyy5566男人资源| 国产av国内精品jk制服| 无码av在线一本无码| 国产麻传媒精品国产av| 无码中文字幕日韩专区视频| 欧美粗大猛烈老熟妇| 99久久无码一区人妻a黑| 国产精品99爱免费视频| 99久久亚洲精品日本无码| 娇妻玩4p被三个男人伺候电影| 天天综合亚洲色在线精品| 2020久久天天躁狠狠躁夜夜| 亚洲国产精品久久久久久久| 美女张开腿黄网站免费| 精品国产小视频在线观看| 巨大乳女人做爰视频在线看| 少妇人妻偷人精品视频| 国产免费久久久久久无码| 无码国产激情在线观看| 国产人在线成免费视频| 国产人妻人伦精品1国产盗摄| 国产亚洲精品久久久久久大师| 亚洲精品无码av中文字幕电影网站| 成熟老妇女毛茸茸的做性| 在线欧美中文字幕农村电影| 蜜桃一区二区三区| 女m羞辱调教视频网站| 国产精品爽爽va吃奶在线观看 | 国产天堂亚洲国产碰碰| 亚洲精品成人片在线观看精品字幕| 日本少妇高潮喷水免费可以看| 日产精品卡二卡三卡四卡区满十八| 精品国产制服丝袜高跟| 乱码午夜-极国产极内射| 国产精品午夜成人免费观看 | 性av盈盈无码天堂| 夜夜高潮天天爽欧美| 日本熟妇人妻xxxxx| 日本亚洲欧洲另类图片| 少妇人妻大乳在线视频|