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重组RabMAb

重组Anti-GAA抗体[EPR4716(2)] (ab137068)

Submit a review Submit a question References (15)
Western blot - Anti-GAA antibody [EPR4716(2)] (ab137068)
  • Western blot - Anti-GAA antibody [EPR4716(2)] (ab137068)
  • Western blot - Anti-GAA antibody [EPR4716(2)] (ab137068)
  • Anti-GAA antibody [EPR4716(2)] (ab137068)

Key features and details

  • Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
  • Rabbit monoclonal [EPR4716(2)] to GAA
  • Suitable for: WB
  • Reacts with: Human

Conjugates logo Related conjugates and formulations

Carrier Free

概述

  • 产品名称

    Anti-GAA抗体[EPR4716(2)]
    参阅全部 GAA 一抗

  • 描述

    兔单克隆抗体[EPR4716(2)] to GAA

  • 宿主

    Rabbit

  • 经测试应用

    适用于: WBmore details
    不适用于: Flow Cyt,ICC/IF,IHC-P or IP

  • 种属反应性

    与反应: Human
    预测可用于: Mouse, Rat

  • 免疫原

    Synthetic peptide within Human GAA aa 150-250. The exact sequence is proprietary.
    Database link: P10253

  • 阳性对照

    • WB: HEK-293T whole cell lysate. Human fetal liver, placenta, liver, brain, and thymus tissue lysates.

  • 常规说明

    This product is a recombinant monoclonal antibody, which offers several advantages including:

    • - High batch-to-batch consistency and reproducibility
    • - Improved sensitivity and specificity
    • - Long-term security of supply
    • - Animal-free production
    For more information see here.

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.

性能

应用

The Abpromise guarantee

Abpromise™承诺保证使用ab137068于以下的经测试应用

“应用说明”部分 下显示的仅为推荐的起始稀释度；实际最佳的稀释度/浓度应由使用者检定。

应用 Ab评论 说明
WB
1/1000 - 1/10000. Detects a band of approximately 110,95,76 kDa (predicted molecular weight: 105 kDa).
说明
WB
1/1000 - 1/10000. Detects a band of approximately 110,95,76 kDa (predicted molecular weight: 105 kDa).
应用说明
Is unsuitable for Flow Cyt,ICC/IF,IHC-P or IP.

靶标

  • 功能

    Essential for the degradation of glygogen to glucose in lysosomes.

  • 疾病相关

    Defects in GAA are the cause of glycogen storage disease type 2 (GSD2) [MIM:232300]; also called acid alpha-glucosidase (GAA) deficiency or acid maltase deficiency (AMD). GSD2 is a metabolic disorder with a broad clinical spectrum. The severe infantile form, or Pompe disease, presents at birth with massive accumulation of glycogen in muscle, heart and liver. Cardiomyopathy and muscular hypotonia are the cardinal features of this form whose life expectancy is less than two years. The juvenile and adult forms present as limb-girdle muscular dystrophy beginning in the lower limbs. Final outcome depends on respiratory muscle failure. Patients with the adult form can be free of clinical symptoms for most of their life but finally develop a slowly progressive myopathy.

  • 序列相似性

    Belongs to the glycosyl hydrolase 31 family.
    Contains 1 P-type (trefoil) domain.

  • 翻译后修饰

    The different forms of acid glucosidase are obtained by proteolytic processing.
    Phosphorylation of mannose residues ensures efficient transport of the enzyme to the lysosomes via the mannose 6-phosphate receptor.

  • 细胞定位

    Lysosome. Lysosome membrane.
  • Information by UniProt

  • 数据库链接

    • 别名

      • 70 kDa lysosomal alpha-glucosidase antibody
      • Acid alpha glucosidase antibody
      • Acid maltase antibody
      • Aglucosidase alfa antibody
      • Alpha glucosidase antibody
      • GAA antibody
      • Glucosidase alpha acid (Pompe disease glycogen storage disease type II) antibody
      • Glucosidase alpha acid antibody
      • Glucosidase alpha antibody
      • LYAG antibody
      • LYAG_HUMAN antibody
      • Lysosomal alpha glucosidase antibody
      see all

    图片

    • Western blot - Anti-GAA antibody [EPR4716(2)] (ab137068)
      Western blot - Anti-GAA antibody [EPR4716(2)] (ab137068)
      All lanes : Anti-GAA antibody [EPR4716(2)] (ab137068) at 1/1000 dilution

      Lane 1 : HEK-293T (human epithelial cell line from embryonic kidney transformed with large T antigen) cell lysate
      Lane 2 : Human fetal liver tissue lysate
      Lane 3 : Human placenta tissue lysate

      Lysates/proteins at 10 µg per lane.

      Predicted band size: 105 kDa
      Observed band size: 110,75,95 kDa why is the actual band size different from the predicted?

    • Western blot - Anti-GAA antibody [EPR4716(2)] (ab137068)
      Western blot - Anti-GAA antibody [EPR4716(2)] (ab137068)
      Anti-GAA antibody [EPR4716(2)] (ab137068) at 1/1000 dilution + HEK-293 (Human embryonic kidney epithelial cell) whole cell lysates at 15 µg

      Secondary
      Goat Anti-Rabbit IgG H&L (HRP) (ab97051) at 1/20000 dilution

      Predicted band size: 105 kDa


      Exposure time: 20 seconds
    • Western blot - Anti-GAA antibody [EPR4716(2)] (ab137068)
      Western blot - Anti-GAA antibody [EPR4716(2)] (ab137068)
      All lanes : Anti-GAA antibody [EPR4716(2)] (ab137068) at 1/1000 dilution

      Lane 1 : Human liver lysates
      Lane 2 : Human brain lysates
      Lane 3 : Human thymus lysates

      Lysates/proteins at 15 µg per lane.

      Secondary
      All lanes : Goat Anti-Rabbit IgG (HRP) with minimal cross-reactivity with human IgG at 1/2000 dilution

      Predicted band size: 105 kDa
      Observed band size: 110,75,95 kDa why is the actual band size different from the predicted?



      Exposure time:

      Lane 1: 10 seconds
      Lane 2: and 3: 30 seconds

    • Anti-GAA antibody [EPR4716(2)] (ab137068)
      Anti-GAA antibody [EPR4716(2)] (ab137068)

    文献 (15)

    发表研究结果有使用 ab137068？请让我们知道，以便我们可以引用本数据表中的参考文章。

    ab137068 被引用在 15 文献中.

    • Meena NK  et al. AAV-mediated delivery of secreted acid α-glucosidase with enhanced uptake corrects neuromuscular pathology in Pompe mice. JCI Insight 8:N/A (2023). PubMed: 37463048
    • Baudot AD  et al. Glycan degradation promotes macroautophagy. Proc Natl Acad Sci U S A 119:e2111506119 (2022). PubMed: 35737835
    • Hamura R  et al. Suppression of lysosomal acid alpha-glucosidase impacts the modulation of transcription factor EB translocation in pancreatic cancer. Cancer Sci 112:2335-2348 (2021). PubMed: 33931930
    • Kilinc S  et al. Oncogene-regulated release of extracellular vesicles. Dev Cell 56:1989-2006.e6 (2021). PubMed: 34118203
    • Baik AD  et al. Cell type-selective targeted delivery of a recombinant lysosomal enzyme for enzyme therapies. Mol Ther 29:3512-3524 (2021). PubMed: 34400331
    View all Publications for this product

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