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

MTO1 Antibody

  • 中文名稱:
    MTO1兔多克隆抗體
  • 貨號:
    CSB-PA015191GA01HU
  • 規格:
    ¥3,900
  • 其他:

產品詳情

  • Uniprot No.:
  • 基因名:
    MTO1
  • 別名:
    MTO1 antibody; CGI-02 antibody; Protein MTO1 homolog antibody; mitochondrial antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human,Mouse,Rat
  • 免疫原:
    Human MTO1
  • 免疫原種屬:
    Homo sapiens (Human)
  • 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 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA,WB
  • 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:
    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:
    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

    顯示更多

    收起更多

  • 相關疾病:
    Combined oxidative phosphorylation deficiency 10 (COXPD10)
  • 亞細胞定位:
    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.
  • 數據庫鏈接:

    HGNC: 19261

    OMIM: 614667

    KEGG: hsa:25821

    UniGene: Hs.347614



主站蜘蛛池模板: 国产精品一区二区av在线观看| 久久久久无码国产精品一区| 久久丫精品国产| 日本少妇高潮喷水视频| 国产亚洲中文日韩欧美综合网| 后进式无遮挡啪啪摇乳动态图| 伊人蕉影院久亚洲高清| 少妇愉情理伦片丰满丰满| 国产无套精品一区二区| 国产av国片精品| 黑人巨茎大战欧美白妇| 欧美亚洲人成网站在线观看 | 韩国午夜福利片在线| 在线黑人抽搐潮喷| 欧美成人一区二区三区片免费| 中文字幕一区二区人妻| 国产麻豆精品av在线观看| 97人妻免费碰视频碰免 | 国产免费又黄又爽又色毛| 亚洲国产精品成人久久| а√天堂8资源中文在线| 热99re久久精品国产首页免费| 性亚洲videofree高清| 国产 | 久你欧洲野花视频欧洲1| 欧美v国产v亚洲v日韩九九| 成在人线av无码免费高潮水老板| 天天综合网亚在线| 国产色秀视频在线播放| 日韩精品视频一区二区三区| 性夜夜春夜夜爽aa片a| 成 人 色综合 综合网站| 日韩欧美国产一区精品| 亚洲精品国产第一综合99久久 | 一本大道无码av天堂| 伊人久久大香线焦av综合影院| 久久99精品久久久久久水蜜桃| 处破痛哭a√18成年片免费 | 免费毛片在线看片免费丝瓜视频| 丰满亚洲大尺度无码无码专线| 老熟女重囗味hdxx69| 国产精品毛片完整版视频|