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AGA Antibody

  • 货号：
    CSB-PA213113
  • 规格：
    ￥1100
  • 图片：
    • Gel: 10%SDS-PAGE, Lysate: 40 μg, Lane: Raji cells, Primary antibody: CSB-PA213113(AGA Antibody) at dilution 1/125, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 30 seconds
  • 其他：

产品详情

  • 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,Rat
  • 免疫原：
    Fusion protein of Human AGA
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen affinity purification
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB
  • 推荐稀释比：
    Application Recommended Dilution
    ELISA 1:1000-1:2000
    WB 1:200-1:1000
  • 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