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ETHE1 Antibody

  • 货号：
    CSB-PA808765
  • 规格：
    ￥1100
  • 图片：
    • The image on the left is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using CSB-PA808765(ETHE1 Antibody) at dilution 1/20, on the right is treated with fusion protein. (Original magnification: ×200)
    • Gel: 10%SDS-PAGE, Lysate: 40 μg, Lane 1-2: A549 cells, mouse liver tissue, Primary antibody: CSB-PA808765(ETHE1 Antibody) at dilution 1/200, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 40 seconds
  • 其他：

产品详情

  • Uniprot No.：
    O95571
  • 基因名：
    ETHE1
  • 别名：
    Ethe1 antibody; ETHE1 protein; mitochondrial precursor antibody; ETHE1_HUMAN antibody; ethylmalonic encephalopathy 1 antibody; Ethylmalonic encephalopathy protein 1 antibody; hepatoma subtracted clone one antibody; Hepatoma subtracted clone one protein antibody; HSCO antibody; mitochondrial antibody; Protein ETHE1 antibody; Protein ETHE1 mitochondrial antibody; YF13H12 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse
  • 免疫原：
    Fusion protein of Human ETHE1
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen affinity purification
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB,IHC
  • 推荐稀释比：
    Application Recommended Dilution
    ELISA 1:1000-1:2000
    WB 1:200-1:1000
    IHC 1:25-1:100
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    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.

产品评价

靶点详情

  • 功能：
    Sulfur dioxygenase that plays an essential role in hydrogen sulfide catabolism in the mitochondrial matrix. Hydrogen sulfide (H(2)S) is first oxidized by SQRDL, giving rise to cysteine persulfide residues. ETHE1 consumes molecular oxygen to catalyze the oxidation of the persulfide, once it has been transferred to a thiophilic acceptor, such as glutathione (R-SSH). Plays an important role in metabolic homeostasis in mitochondria by metabolizing hydrogen sulfide and preventing the accumulation of supraphysiological H(2)S levels that have toxic effects, due to the inhibition of cytochrome c oxidase. First described as a protein that can shuttle between the nucleus and the cytoplasm and suppress p53-induced apoptosis by sequestering the transcription factor RELA/NFKB3 in the cytoplasm and preventing its accumulation in the nucleus.
  • 基因功能参考文献：
    1. observations indicate the severe impact of ETHE1 deficiency on cellular physiology and redox state PMID: 27074420
    2. ETHE1 is a major enzyme regulating endogenous glutathione persulfide /GS-(S)n-H and that its activity is controlled by polysulfidation of the Cys247 residue. PMID: 27742479
    3. ETHE1 R163W/R163Q mutations are associated with Ethylmalonic encephalopathy. PMID: 25198162
    4. Case Report: metabolic disturbances in 15-month-old male presenting with typical ethylmalonic encephalopathy associated with a homozygous ETHE1 mutation. PMID: 20978941
    5. T152I mutation of ETHE1 results in a 3-fold lower activity. PMID: 23144459
    6. role of the ETHE1 gene product in mitochondrial homeostasis and energy metabolism PMID: 14732903
    7. Mutations of ETHE1 were detected in all the typical ethylmalonic encephalopathy patients analysed, but no ETHE1 mutations were identified in patients presenting with early onset progressive encephalopathy with ethylmalonic aciduria. PMID: 16183799
    8. structural comparison of human ETHE1 and At1g53580 from Arabidopsis thaliana PMID: 16929096
    9. 14 patients with EE were investigated for mutations in the ETHE1 gene. Of the 14 patients, 5 were found to carry novel mutations. PMID: 18593870
    10. ETHE1 is a mitochondrial sulfur dioxygenase involved in catabolism of sulfide that accumulates to toxic levels in ethylmalonic encephalopathy. PMID: 19136963

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  • 相关疾病：
    Ethylmalonic encephalopathy (EE)
  • 亚细胞定位：
    Cytoplasm. Nucleus. Mitochondrion matrix.
  • 蛋白家族：
    Metallo-beta-lactamase superfamily, Glyoxalase II family
  • 组织特异性：
    Ubiquitously expressed.
  • 数据库链接：

    HGNC: 23287

    OMIM: 602473

    KEGG: hsa:23474

    STRING: 9606.ENSP00000292147

    UniGene: Hs.7486