Your Good Partner in Biology Research

中文简体

CSF2｜ CTLA4｜ DLL3｜ EGFR｜ IL4｜ IL4R｜ LAG3｜ MSTN｜ NECTIN4｜ NT5E｜ SEZ6｜ TM4SF1｜ TNFSF13｜ TPO

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

GATM Antibody

说明书
  • 中文名称：
    GATM兔多克隆抗体
  • 货号：
    CSB-PA009284ESR1HU
  • 规格：
    ￥440
  • 图片：
    • 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：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) GATM Polyclonal antibody
  • Uniprot No.：
    P50440
  • 基因名：
    GATM
  • 别名：
    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
  • 反应种属：
    Human, Mouse
  • 免疫原：
    Recombinant Human Glycine amidinotransferase, mitochondrial protein (124-423AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 克隆类型：
    Polyclonal
  • 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 0.02% sodium azide, 50% glycerol, pH7.3.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, WB, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:20-1:200
  • Protocols：
    ELISA Protocol
    Western Blotting (WB) Protocol
    Immunohistochemistry (IHC) Protocol
  • 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.
  • 用途：
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

产品评价

相关产品

GATM antibodies

GATM Proteins

靶点详情

  • 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.
  • 基因功能参考文献：
    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

    显示更多

    收起更多

  • 相关疾病：
    Cerebral creatine deficiency syndrome 3 (CCDS3)
  • 亚细胞定位：
    [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
  • 组织特异性：
    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.
  • 数据库链接：

    HGNC: 4175

    OMIM: 602360

    KEGG: hsa:2628

    STRING: 9606.ENSP00000379895

    UniGene: Hs.560354

Most popular with customers

产品中心
特别关注


X
九游平台-九游(中国) | 米兰体育-米兰体育（中国） | 开云手机在线登陆入口 | 江南官方体育网页版 | 开云网页版 | 开云官方在线入口 | 开云电子体育 | 开云官方链接 | 江南网页版页面登录 |