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

  1. Home
  2. 抗體
  3. 多克隆抗體
  4. GATM Antibody

GATM Antibody

  • 中文名稱：
    GATM兔多克隆抗體
  • 貨號：
    CSB-PA009284ESR1HU
  • 規格：
    ￥440
  • 圖片：
    • Western Blot
      Positive WB detected in: THP-1 whole cell lysate(20μg), 293 whole cell lysate(20μg), Mouse Liver tissue lysate(20μg), Mouse Kidney tissue lysate(20μg)
      All lanes: GATM antibody at 1:1000
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 49, 45 kDa
      Observed band size: 49 kDa
      Exposure time: 120s
    • IHC image of CSB-PA009284ESR1HU diluted at 1:300 and staining in paraffin-embedded human liver tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB. Secondary antibody only control: uses 1% BSA instead of primary antibody
    • IHC image of CSB-PA009284ESR1HU diluted at 1:300 and staining in paraffin-embedded human kidney tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB. Secondary antibody only control: uses 1% BSA instead of primary antibody
    • IHC image of CSB-PA009284ESR1HU diluted at 1:300 and staining in paraffin-embedded human pancreatic cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB. Secondary antibody only control: uses 1% BSA instead of primary antibody
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) GATM Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    GATM
  • 別名：
    AGAT antibody; AT antibody; GATM antibody; GATM_HUMAN antibody; Glycine amidinotransferase antibody; Glycine amidinotransferase, mitochondrial antibody; L-arginine:glycine amidinotransferase antibody; mitochondrial antibody; Transamidinase antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human, Mouse
  • 免疫原：
    Recombinant Human Glycine amidinotransferase, mitochondrial protein (124-423AA)
  • 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
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:20-1:200
  • 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：
    Catalyzes the biosynthesis of guanidinoacetate, the immediate precursor of creatine. Creatine plays a vital role in energy metabolism in muscle tissues. May play a role in embryonic and central nervous system development. May be involved in the response to heart failure by elevating local creatine synthesis.
  • 基因功能參考文獻：
    1. Measurements of creatine and guanidinoacetate in plasma are recommended for the diagnosis of AGAT and GAMT deficiency.Definitive confirmation of the diagnosis requires DNA sequencing of the appropriate gene and (if molecular analysis is ambiguous) measurement of AGAT or GAMT enzyme activity or of CRTR-mediated transport . PMID: 28055022
    2. Results show the functional characterization of rare missense variants in GATM which cause GATM deficiency. Seven of them report 0% of wild-type GATM activity indicating a putative pathogenicity. PMID: 27233232
    3. AGAT deficiency is a treatable intellectual disability. PMID: 26490222
    4. Meta-analysis yielded a marginal, but null, association of GATM rs9806699 with statin-induced myopathy. PMID: 25863251
    5. Genome-wide association reveals that plasma homoarginine is strongly associated with single nucleotide polymorphisms in the AGAT gene. PMID: 24004504
    6. promiscuous activity of AGAT, a key enzyme in creatine synthesis, plays a pivotal role in homoarginine synthesis PMID: 23010440
    7. GATM sequencing revealed a homozygous single nucleotide insertion 1111_1112insA, producing a frame-shift at Met-371 and premature termination at codon 376. PMID: 20682460
    8. AGAT mRNA expression was significantly elevated in all heart failure patients and returned to normal levels after recovery, suggesting a specific response to heart failure involving elevated local creatine synthesis. PMID: 16820567

    顯示更多

    收起更多

  • 相關疾病：
    Cerebral creatine deficiency syndrome 3 (CCDS3)
  • 亞細胞定位：
    [Isoform 1]: Mitochondrion inner membrane; Peripheral membrane protein; Intermembrane side. Note=Probably attached to the outer side of the inner membrane.; [Isoform 2]: Cytoplasm.
  • 蛋白家族：
    Amidinotransferase family
  • 組織特異性：
    Expressed in brain, heart, kidney, liver, lung, salivary gland and skeletal muscle tissue, with the highest expression in kidney. Biallelically expressed in placenta and fetal tissues.
  • 數據庫鏈接：

    HGNC: 4175

    OMIM: 602360

    KEGG: hsa:2628

    STRING: 9606.ENSP00000379895

    UniGene: Hs.560354



主站蜘蛛池模板： 亚洲 欧美 中文 日韩aⅴ| 亚洲国产精品无码专区| 国内精品免费久久久久软件| 亚洲人成电影综合网站色www| 中文字幕乱码免费| 国产99久久久国产精品～～牛| 女女同性av片在线观看免费| 久久人午夜亚洲精品无码区| 鲁鲁狠狠狠7777一区二区| 波多野吉衣av无码| 久久97国产超碰青草| 在线|国产精品女主播阳台| 日本熟妇中文字幕三级| 97色伦图片97综合影院| 欧美成人精品三级一二三在线观看| 人妻 偷拍 无码 中文字幕| 好大好硬好爽免费视频| 欧美亚洲色综久久精品国产| 在线观看日本亚洲一区| 久久久婷婷五月亚洲97色| 夜夜高潮夜夜爽夜夜爱| 久久久久久久无码高潮| 久久er99国产精品免费| 国产成人久久av免费| 熟妇的味道hd中文字幕| 高清破外女出血av毛片| 欧美人妻久久精品| 国产精品污www一区二区三区| 国产精品成人久久久久久久| 乱人伦视频中文字幕| 护士被强女千到高潮视频| 波多野结衣av手机在线观看| 久久婷婷五月综合色首页| 国产做a爰片久久毛片a片美国| 国产精品国产三级国产专区53| 熟妇熟女乱妇乱女网站| 亚洲另类欧美在线电影| 内射精品无码中文字幕| 国产区图片区小说区亚洲区| 色一情一乱一乱一区99av| 亚洲午夜精品久久久久久app|