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AGL Antibody

  • 货号：
    CSB-PA001446LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunofluorescence staining of A549 cells with CSB-PA001446LA01HU at 1:100, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) AGL Polyclonal antibody
  • Uniprot No.：
    P35573
  • 基因名：
    AGL
  • 别名：
    1110061O17Rik antibody; 4-1 antibody; 4-glucantransferase antibody; 6-glucosidase antibody; 9430004C13Rik antibody; 9630046L06Rik antibody; AGL antibody; AI850929 antibody; Amylo 1 6 glucosidase 4 alpha glucanotransferase antibody; Amylo-1 antibody; Amylo-alpha-1 antibody; C77197 antibody; Dextrin 6-alpha-D-glucosidase antibody; GDE antibody; GDE_HUMAN antibody; Glycogen debrancher antibody; Glycogen debranching enzyme antibody; Glycogen storage disease type III antibody; Oligo-1 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Glycogen debranching enzyme protein (1217-1378AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，AGL Antibody (CSB-PA001446LA01HU)，的标记方式是Non-conjugated。对于AGL 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-PA001446LB01HU AGL Antibody, HRP conjugated ELISA
    FITC CSB-PA001446LC01HU AGL Antibody, FITC conjugated
    Biotin CSB-PA001446LD01HU AGL 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, IF
  • 推荐稀释比：
    Application Recommended Dilution
    IF 1:50-1:200
  • 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：
    Multifunctional enzyme acting as 1,4-alpha-D-glucan:1,4-alpha-D-glucan 4-alpha-D-glycosyltransferase and amylo-1,6-glucosidase in glycogen degradation.
  • 基因功能参考文献：
    1. This report of patients with GSD-III in Iran with 2 uncommon clinical presentations and 5 novel mutations in the AGL gene. PMID: 29794575
    2. The study identified 31 novel mutations and extended the mutation spectrum of AGL in Chinese patients with glycogen storage disease type III. PMID: 26984562
    3. Our study establishes HAS2-mediated HA synthesis as a driver of growth of bladder cancer with low AGL and provides preclinical rationale for personalized targeting of HAS2/HA signaling in patients with low amylo-alpha-1-6-glucosidase-4-alpha-glucanotransferase -expressing tumors. PMID: 26490312
    4. AGL loss causes high SHMT2 expression and consequently increases glycine-dependent nucleotide synthesis leading to bladder cancer growth. PMID: 26975021
    5. Point mutations in AGL gene are associated with glycogen storage disease type IIIa in a Chinese family. PMID: 26252094
    6. Haplotype analysis revealed that the mutation arises as a result of founder effect, not an independent event. PMID: 25827695
    7. AGL haplotype analyses suggested that c.1019delA and c.958+1G>A are founder mutations in Turkish patients, while p.R864X is a recurrent mutation. PMID: 25451950
    8. A homozygous frameshift deletion, c.4456delT, in exon 33 of the AGL gene in Inuit children determines the cause of glycogen storage disease type IIIa and confirms a founder effect. PMID: 25602008
    9. study identified 10 different mutations in 8 Korean Glycogen storage disease type III patients; 5 mutations are novel and include 1 nonsense (c.1461G>A, p.W487X), 3 splicing (c.293+4_293+6delAGT in IVS4, c.460+1G>T in IVS5, c.2682-8A>G in IVS21) and 1 missense mutation (c.2591G>C, p.R864P) PMID: 24257475
    10. Characterization of a novel homozygous single point mutation at the polypyrimidine tract of intron 21 of the AGL gene in two consanguineous siblings with glycogen storage disease type III. PMID: 23649758
    11. We found that most patients with macular telangiectasia-2 possess retinal autoantibodies, the most prevalent of which were directed against AGL, RBP3, and CK-B. PMID: 23882694
    12. A founder effect discovered amongst Tunisian patients with glycogen storage disease type III and a c.3216_3217delGA mutation in the AGL gene. PMID: 22035446
    13. Mutations in amylo-1,6-glucosidase is associated with Glycogen Storage Disease Type III. PMID: 20648714
    14. The present patient was found to be deficient in GDE activity and homozygous for a novel 1 bp deletion in AGL. This mutation is predicted to cause premature termination at codon 834 due to frame shift. PMID: 20158661
    15. GSD-III patients have variable phenotypic characteristics. Administration of raw-corn-starch can effectively improve the disease outcome. We identified 8 new mutations on AGL gene through nucleotide sequence analysis. PMID: 15833157
    16. Nine AGL mutations: six nonsense mutations , one deletion and two splicing mutation were identified in Turkish GSD III patients. PMID: 19834502
    17. Mutations associated with GSD III include R34X and Y1148X. PMID: 11924557
    18. it is likely that the AMPK-GDE association is a novel mechanism regulating AMPK activity and the resultant fatty acid oxidation and glucose uptake PMID: 15886229
    19. AGL gene mutations may have roles in glycogen storage disease type III PMID: 17047887
    20. These results indicate that binding to glycogen crucially regulates the stability of AGL and, further, that its ubiquitination may play an important role in the pathophysiology of both Lafora and Cori's disease. PMID: 17908927
    21. Current clinical and molecular knowledge about glycogenosis 3 and phenotype and genotype levels of this enzyme. [REVIEW] PMID: 17915576
    22. a homozygous p.W1327X mutation leads to severe generalized glycogenosis types 3a & 3b within the same family; heterozygous p.W1327X mutation carriers may present with mild non-progressive neuromuscular symptoms, such as exercise-induced myalgia & fatigue PMID: 18924225
    23. Mutations in the carbohydrate-binding domain of AGL lead to loss of all enzymatic activities and enhancing targeting for proteasomal degradation. PMID: 19299494
    24. Six novel AGL mutations were identified. PMID: 19754354

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  • 相关疾病：
    Glycogen storage disease 3 (GSD3)
  • 亚细胞定位：
    Cytoplasm. Note=Under glycogenolytic conditions localizes to the nucleus.
  • 蛋白家族：
    Glycogen debranching enzyme family
  • 组织特异性：
    Liver, kidney and lymphoblastoid cells express predominantly isoform 1; whereas muscle and heart express not only isoform 1, but also muscle-specific isoform mRNAs (isoforms 2, 3 and 4). Isoforms 5 and 6 are present in both liver and muscle.
  • 数据库链接：

    HGNC: 321

    OMIM: 232400

    KEGG: hsa:178

    STRING: 9606.ENSP00000294724

    UniGene: Hs.904