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GTPBP3 Antibody

  • 货号：
    CSB-PA010034GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    Q969Y2
  • 基因名：
    GTPBP3
  • 别名：
    GTPBP3 antibody; MTGP1 antibody; tRNA modification GTPase GTPBP3 antibody; mitochondrial antibody; GTP-binding protein 3 antibody; Mitochondrial GTP-binding protein 1 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human GTPBP3
  • 免疫原种属：
    Homo sapiens (Human)
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity Purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB,IHC
  • 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：
    GTPase involved in the 5-carboxymethylaminomethyl modification (mnm(5)s(2)U34) of the wobble uridine base in mitochondrial tRNAs.
  • 基因功能参考文献：
    1. Defects in the mitochondrial tRNA modification enzymes MTO1 and GTPBP3 promote different metabolic reprogramming through a HIF-PPARgamma-UCP2-AMPK axis. PMID: 29348686
    2. GTPBP3 defective expression is associated with an mitochondrial-tRNA hypomodification status.GTPBP3 plays a role in the regulation of UCP2 and MCP1 proteins through AMPK signaling. PMID: 26642043
    3. Most individuals with GTPBP3 mutations developed neurological symptoms and MRI involvement of thalamus, putamen, and brainstem resembling Leigh syndrome. PMID: 25434004
    4. GTPBP3 localizes in the mitochondria and is a deafness-associated homolog of yeast MSS1. PMID: 12370316
    5. Phenotype of non-syndromic deafness associated with the mitochondrial A1555G mutation is modulated by mitochondrial RNA modifying enzymes MTO1 and GTPBP3. PMID: 15542390
    6. Data show that the two most abundant GTPBP3 isoforms exhibit moderate affinity for guanine nucleotides like their bacterial homologue, MnmE, although they hydrolyze GTP at a 100-fold lower rate. PMID: 18852288

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  • 相关疾病：
    Combined oxidative phosphorylation deficiency 23 (COXPD23)
  • 亚细胞定位：
    Mitochondrion.
  • 蛋白家族：
    TRAFAC class TrmE-Era-EngA-EngB-Septin-like GTPase superfamily, TrmE GTPase family
  • 组织特异性：
    Ubiquitously expressed.
  • 数据库链接：

    HGNC: 14880

    OMIM: 580000

    KEGG: hsa:84705

    UniGene: Hs.334885