Anti-GAA抗体(ab55049)

概述

  • 产品名称Anti-GAA抗体
    参阅全部 GAA 一抗
  • 描述
    小鼠单克隆抗体to GAA
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Recombinant Fragment
    预测可用于: Human
  • 免疫原

    Recombinant fragment, corresponding to amino acids 851-953 of Human GAA

性能

应用

Our Abpromise guarantee covers the use of ab55049 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB Use a concentration of 1 - 5 µg/ml. This antibody has only been tested in WB against the recombinant fragment used as immunogen. We have no data on the detection of endogenous protein.

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

Anti-GAA antibody 图像

  • Western blot against tagged recombinant protein immunogen using ab55049 GAA antibody at 1ug/ml. Predicted band size of immunogen is 37 kDa.

    This antibody has only been tested in WB against the recombinant fragment used as immunogen. We have no data on the detection of endogenous protein.

Anti-GAA antibody (ab55049)参考文献

ab55049 has not yet been referenced specifically in any publications.

Product Wall

Thank you for your inquiry. The immunogen of the antibody is GAA (AAH40431, 851 a.a. ~ 953 a.a) partial recombinant protein with GST tag. According to the description on paper, this part of GAA, 851 a.a. ~ 953 a.a, will be cleaved in the matur...

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"