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AGA Antibody

  • 货号：
    CSB-PA001423LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western blot
      All lanes: AGA antibody at 10µg/ml
      Lane 1: 293T whole cell lysate
      Lane 2: Mouse muscle tissue
      Secondary
      Goat polyclonal to rabbit IgG at 1/10000 dilution
      Predicted band size: 38 kDa
      Observed band size: 38 kDa
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) AGA Polyclonal antibody
  • Uniprot No.：
    P20933
  • 基因名：
    AGA
  • 别名：
    Aga antibody; AGU antibody; Aspartylglucosaminidase antibody; Aspartylglucosylamine deaspartylase antibody; Aspartylglycosaminuria antibody; ASPG_HUMAN antibody; ASRG antibody; GA antibody; Glycosylasparaginase antibody; Glycosylasparaginase beta chain antibody; N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase antibody; N4 (N acetyl beta glucosaminyl) L asparagine amidase antibody; N4-(N-acetyl-beta-glucosaminyl)-L-asparagine amidase antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human, Mouse
  • 免疫原：
    Recombinant Human N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase protein (206-346AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，AGA Antibody (CSB-PA001423LA01HU)，的标记方式是Non-conjugated。对于AGA Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA001423LB01HU AGA Antibody, HRP conjugated ELISA
    FITC CSB-PA001423LC01HU AGA Antibody, FITC conjugated
    Biotin CSB-PA001423LD01HU AGA Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, WB
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:1000-1:5000
  • 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：
    Cleaves the GlcNAc-Asn bond which joins oligosaccharides to the peptide of asparagine-linked glycoproteins.
  • 基因功能参考文献：
    1. 1.8A resolution crystal structure of mature G172D mutant of a model missense GA corresponding to a Canadian aspartylglucosaminuria allele; studied the effect of its single amino acid change on substrate processing PMID: 28457719
    2. We show that gene-silenced cells show specifically reduced AGA activity and store globotriaosylceramide. In gene-silenced cells, release of the neurotransmitter acetylcholine is significantly reduced, demonstrating that this model may be used to study specific neuronal functions such as neurotransmitter release in Fabry disease PMID: 27471012
    3. study reports 2 novel aspartylglucosaminidase gene mutations, one in Qatari twins with an early, perinatal presentation not previously described for aspartylglucosaminuria and the other in 3 Turkish children with newly diagnosed aspartylglucosaminuria and a more classical disease course PMID: 23271757
    4. [review] Natural killer (NK) cell tumors, subtypes of myeloid leukemias and T-cell lymphomas respond to ASNase; ovarian carcinomas and other solid tumors have been proposed as additional targets for ASNase, with a potential role for glutaminase. activity. PMID: 21854356
    5. Molecular mechanism for the autoproteolytic activation of aspartylglucosaminidase. PMID: 14616088
    6. A new point mutation, c.44T>G, found in a Finnish compound heterozygote causes a L15R AA substitution in the signal sequence of the AGA enzyme, affecting AGA translocation by altering a critical hydrophobic core structure in the signal sequence. PMID: 15365992
    7. aspartylglucosaminidase may have a role in development of congenital disorders of glycosylation type I PMID: 16435229
    8. The amino acid substitutions in aspartylglucosaminidase responsible for aspartylglucosaminuria were classified and divided in three groups. PMID: 18992224
    9. Increased AGA plasma activity, although a consistent finding in congenital disorders of glycosylation patients, is not specific to this group of disorders. PMID: 19100247

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  • 相关疾病：
    Aspartylglucosaminuria (AGU)
  • 亚细胞定位：
    Lysosome.
  • 蛋白家族：
    Ntn-hydrolase family
  • 数据库链接：

    HGNC: 318

    OMIM: 208400

    KEGG: hsa:175

    STRING: 9606.ENSP00000264595

    UniGene: Hs.207776