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

ETFDH Antibody

  • 中文名稱:
    ETFDH兔多克隆抗體
  • 貨號:
    CSB-PA619056ESR2HU
  • 規格:
    ¥440
  • 圖片:
    • Western blot
      All lanes: ETFDH antibody at 3.56µg/ml + 293T whole cell lysate
      Secondary
      Goat polyclonal to rabbit IgG at 1/10000 dilution
      Predicted band size: 69, 63 kDa
      Observed band size: 63 kDa
    • Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA619056ESR2HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human small intestine tissue using CSB-PA619056ESR2HU at dilution of 1:100
    • Immunofluorescent analysis of PC-3 cells using CSB-PA619056ESR2HU at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) ETFDH Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    ETFDH
  • 別名:
    Electron transfer flavoprotein ubiquinone oxidoreductase antibody; Electron transfer flavoprotein-ubiquinone oxidoreductase antibody; electron transferring flavoprotein dehydrogenase antibody; Electron-transferring-flavoprotein dehydrogenase antibody; ETF dehydrogenase antibody; ETF QO antibody; ETF ubiquinone oxidoreductase antibody; ETF-QO antibody; ETF-ubiquinone oxidoreductase antibody; ETFD_HUMAN antibody; Etfdh antibody; MADD antibody; mitochondrial antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human
  • 免疫原:
    Recombinant Human Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial protein (318-617AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    Non-conjugated
  • 克隆類型:
    Polyclonal
  • 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 0.02% sodium azide, 50% glycerol, pH7.3.
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA, WB, IHC, IF
  • 推薦稀釋比:
    Application Recommended Dilution
    WB 1:500-1:2000
    IHC 1:20-1:200
    IF 1:50-1:200
  • 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:
    Accepts electrons from ETF and reduces ubiquinone.
  • 基因功能參考文獻:
    1. Neurite shortening and impairment in neurite growth was caused by a mutation in ETFDH. PMID: 27935074
    2. This study identified three novel compound heterozygous mutations of ETFDH gene in patients with late-onset multiple acyl-CoA dehydrogenase deficiency, and discussed the significant clinical heterogeneity among patients with similar genotype. PMID: 27000805
    3. ETFDH mutation is the causative gene in patients with adult-onset multiple acyl-CoA dehydrogenase deficiency with severe sensory neuropathy. PMID: 26821934
    4. Mtation c.250G>A and mutation c.353G>T in the ETFDH gene are associate with multiple aeyl-CoA dehydrogenase deficiency with severe fatty liver. PMID: 27060313
    5. identified 61 ETFDH mutations, including 31 novel mutations, which were widely distributed within the coding sequence PMID: 24357026
    6. Mutations in SLC22A5 and ETFDH are associated with riboflavin responsive-multiple acyl-CoA dehydrogenase deficiency. PMID: 25119904
    7. Results show that a predicted benign ETFDH missense variationc.158A>G in exon 2 causes exon skipping and degradation of ETFDH protein in patient samples. PMID: 24123825
    8. Case Report: ETF dehydrogenase mutations resulting in mild glutaric aciduria type II and complex II-III deficiency in liver and muscle. PMID: 21088898
    9. folding defects in the variant ETF-QO proteins and multiple acyl-CoA dehydrogenation deficiency PMID: 22611163
    10. a significant reduced expression of ETFDH was identified in the muscle of ETFDH-deficient patients; ETFDH deficiency is a major cause of riboflavin-responsive MADD in southern China, and c.250G>A is an important mutation PMID: 21347544
    11. High frequency of ETFDH c.250G>A mutation in Taiwanese patients with late-onset lipid storage myopathy PMID: 20370797
    12. 3 known (c.250G>A, c380T>A, c.524G>T) and 1 novel (c.1831G>A) ETFDH mutation were detected by high resolution melting analysis. The carrier frequency of the hotspot mutation, c.250G>A, in the Taiwanese population was found to be 1:125. PMID: 20138856
    13. lipid storage myopathy caused by ETFDH gene mutations. PMID: 19758981
    14. expression from a baculovirus vector and kinetic and spectral characteristics PMID: 12049629
    15. Mutations are identified by molecular analysis of 20 ETF:QO-deficient patients. Twenty-one different disease-causing mutations were identified on 36 of the 40 chromosomes. PMID: 12359134
    16. patients had autosomal recessive mutations in ETFDH, suggesting ETFDH deficiency leads to a secondary CoQ10 deficiency; results indicate that the late-onset form of glutaric aciduria type II & the myopathic form of CoQ10 deficiency are allelic diseases PMID: 17412732
    17. study identified ETFDH mutations in all members of a large series of patients with riboflavin-responsive multiple acyl-CoA dehydrogenation deficiency PMID: 17584774
    18. We performed mutation analysis in four Taiwanese MADD patients. Three novel ETFDH mutations were identified in four patients and all harbored the p.A84T mutation PMID: 19249206
    19. Four novel mutations (3 missenses and 1 deletion) in ETFDH were found in Chinese families that presented with riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency. PMID: 19265687

    顯示更多

    收起更多

  • 相關疾?。?/div>
    Glutaric aciduria 2C (GA2C)
  • 亞細胞定位:
    Mitochondrion inner membrane.
  • 蛋白家族:
    ETF-QO/FixC family
  • 數據庫鏈接:

    HGNC: 3483

    OMIM: 231675

    KEGG: hsa:2110

    STRING: 9606.ENSP00000426638

    UniGene: Hs.155729



主站蜘蛛池模板: 国产精品情侣呻吟对白视频| 欧美群妇大交群| 无码国产精品一区二区免费i6| 女人被弄到高潮的免费视频 | 国产精品igao视频| 国产午夜亚洲精品不卡| 亚洲国产日韩在线人成蜜芽| 国产午夜鲁丝片av无码| 国产亚av手机在线观看| 国产成人亚洲精品| 国产v综合v亚洲欧| 亚洲综合色88综合天堂| 亚洲乱码国产乱码精品精| 国自产拍偷拍精品啪啪模特 | 国产成人免费永久在线平台| 老妇肥熟凸凹丰满刺激| 137裸交肉体摄影| 欧美两根一起进3p在线观看| 无码一区二区三区亚洲人妻| 久久精品国产一区二区三区 | 亚洲精品无码av人在线观看| 日本免费一本一二区三区| 性色av无码久久一区二区三区| 日韩超碰人人爽人人做人人添 | 国产卡一卡二卡三卡免费| 国产精品免费久久久久影院仙踪林| 18禁网站免费无遮挡无码中文| 国产成人综合色视频精品| 国产亚洲曝欧美曝妖精品| 亚洲成av人片在www色猫咪| www国产精品内射老熟女| 老司机免费的精品视频| 亚洲综合在线一区二区三区| 亚洲国产婷婷六月丁香| 日产乱码一区二区三区在线| 久青草久青草视频在线观看| 69sex久久精品国产麻豆| 中文在线а√在线天堂中文| 国产亚洲精品自在久久蜜tv| 男人j进入女人j内部免费网站| 久久久久久久久免费看无码 |