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MTO1 Antibody

  • 中文名稱(chēng)：
    MTO1兔多克隆抗體
  • 貨號(hào)：
    CSB-PA846064ESR2HU
  • 規(guī)格：
    ￥440
  • 促銷(xiāo)：
    小規(guī)格抗體限時(shí)一口價(jià)
  • 圖片：
    • Western blot
      All lanes: MTO1 antibody at 2.25µg/ml
      Lane 1: Rat heart tissue
      Lane 2: Jurkat whole cell lysate
      Secondary
      Goat polyclonal to rabbit IgG at 1/10000 dilution
      Predicted band size: 80, 67, 35, 78, 73, 82 kDa
      Observed band size: 80, 67, 35 kDa
    • Immunohistochemistry of paraffin-embedded human brain tissue using CSB-PA846064ESR2HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA846064ESR2HU at dilution of 1:100
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱(chēng)：
    Rabbit anti-Homo sapiens (Human) MTO1 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    MTO1
  • 別名：
    MTO1 antibody; CGI-02 antibody; Protein MTO1 homolog antibody; mitochondrial antibody
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human, Rat
  • 免疫原：
    Recombinant Human Protein MTO1 homolog, mitochondrial protein (420-680AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)記方式：
    Non-conjugated
  • 克隆類(lèi)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(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA, WB, IHC
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:1000-1:5000
    IHC 1:20-1:200
  • Protocols：
  • 儲(chǔ)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.

產(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

  • 功能：
    Involved in the 5-carboxymethylaminomethyl modification (mnm(5)s(2)U34) of the wobble uridine base in mitochondrial tRNAs.
  • 基因功能參考文獻(xiàn)：
    1. Two novel variants (c.[253G > A];[938G > A]) in the MTO1 gene were identified in a child with cardiomyopathy with early-onset brain disease. PMID: 29440775
    2. MTO1 deficiency is lethal in some but not all cases, and a genotype-phenotype relation is suggested. Aside from lactic acidosis and cardiomyopathy, developmental delay and other phenotypic features affecting multiple organ systems are often present in these patients, suggesting a broader spectrum than hitherto reported. PMID: 29331171
    3. Defects in the mitochondrial tRNA modification enzymes MTO1 and GTPBP3 promote different metabolic reprogramming through a HIF-PPARgamma-UCP2-AMPK axis. PMID: 29348686
    4. Whole-exome sequencing (WES) indicated a double homozygous mutation in the mitochondrial tRNA translation optimization 1 protein (MTO1) gene (p.R504C and p.V557M) in two of the patients and the homozygous mutation p.R504C in the other. PMID: 27256614
    5. Optic neuropathy, cardiomyopathy, and cognitive disability was found in patients with a homozygous mutation in the nuclear MTO1 and a mitochondrial MT-TF variant. PMID: 26061759
    6. MTO1 mediates tRNA modification and controls mitochondrial translation rate in a highly tissue-specific manner associated with tissue-specific oxidative phosphorylation defects. PMID: 25552653
    7. Results show that post-transcriptional expression of GTPBP3, MTO1 and TRMU genes is down-regulated, leading to mt-tRNA hypomodification and contributing to mitochondrial dysfunction in MELAS cybrids. PMID: 25149473
    8. Nuclear-encoded mitochondrial MTO1 and MRPL41 are regulated in an opposite epigenetic mode based on estrogen receptor status in breast cancer. PMID: 24160266
    9. MTO1 mutations are commonly associated with a presentation of hypertrophic cardiomyopathy, lactic acidosis, and MRC deficiency. PMID: 23929671
    10. MTO1 mutations have roles in hypertrophic cardiomyopathy and lactic acidosis PMID: 22608499
    11. identification of full-length cDNA and elucidation of genomic organization of the human MTO1 homolog PMID: 12011058
    12. Phenotype of non-syndromic deafness associated with the mitochondrial A1555G mutation is modulated by mitochondrial RNA modifying enzymes MTO1 and GTPBP3. PMID: 15542390
    13. proposed linkage in chromosome 8 and the association with TRMU and MTO1 genes were studied in A1555G deafness PMID: 18391568

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  • 相關(guān)疾?。?/div>
    Combined oxidative phosphorylation deficiency 10 (COXPD10)
  • 亞細(xì)胞定位：
    Mitochondrion.
  • 蛋白家族：
    MnmG family
  • 組織特異性：
    Ubiquitously expressed in various tissues, but with a markedly elevated expression in tissues of high metabolic rates including cochlea.
  • 數(shù)據(jù)庫(kù)鏈接：

    HGNC: 19261

    OMIM: 614667

    KEGG: hsa:25821

    UniGene: Hs.347614