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  • Official Full Name

    glucosidase, alpha; acid

  • Overview

    This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompes disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene.
  • Synonyms

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

  • Recombinant Proteins
  • Therapeutic Proteins
  • Cell & Tissue Lysates
  • Protein Pre-coupled Magnetic Beads
  • Assay Kits
  • Human
  • Mouse
  • Rat
  • CHO
  • E.coli
  • HEK293
  • HEK293T
  • In Vitro Cell Free System
  • Mamanlian cells
  • Mammalian Cell
  • Wheat Germ
  • Flag
  • GST
  • His
  • His (Fc)
  • Avi
  • Myc
  • DDK
  • Myc|DDK
  • N/A
Species Cat.# Product name Source (Host) Tag Protein Length Price
Human GAA-002H Active Recombinant Human Acid α-glucosidase (GAA) HEK293T His
Human GAA-173H Active Recombinant Human GAA protein, His-tagged HEK293 His
Human GAA-P026H Recombinant Human GAA therapeutic protein CHO N/A
Human GAA-177H Recombinant Human GAA protein, MYC/DDK-tagged HEK293 Myc/DDK
Human GAA-176H Recombinant Human GAA protein, MYC/DDK-tagged HEK293 Myc/DDK
Human GAA-175H Recombinant Human GAA protein, MYC/DDK-tagged HEK293 Myc/DDK
Human GAA-28090TH Recombinant Human GAA protein, GST-tagged Wheat Germ GST
Human GAA-160H Recombinant Human GAA Protein, His-tagged E.coli His Ala761~Asn919
Human GAA-159H Recombinant Human GAA Protein, His-tagged E.coli His Pro595~Gly770
Human GAA-28087TH Recombinant Human GAA Wheat Germ N/A 102 amino acids
Human GAA-6077HCL Recombinant Human GAA 293 Cell Lysate HEK293 N/A
Human GAA-3385H Recombinant Human GAA Protein, Myc/DDK-tagged, C13 and N15-labeled HEK293T Myc/DDK
Human GAA-4807H Recombinant Human GAA Protein, Myc/DDK-tagged, C13 and N15-labeled HEK293T Myc/DDK
Human GAA-7382HFL Recombinant Full Length Human GAA protein, Flag-tagged Mamanlian cells Flag
Human GAA-5243H Recombinant Human GAA Protein, Myc/DDK-tagged, C13 and N15-labeled HEK293T Myc/DDK
Human GAA-6936HF Recombinant Full Length Human GAA Protein, GST-tagged In Vitro Cell Free System GST 952 amino acids
Mouse Gaa-161M Recombinant Mouse Gaa Protein, His-tagged E.coli His Val755~Ser953
Mouse Gaa-162M Recombinant Mouse Gaa Protein, His-tagged E.coli His Glu70~Phe245
Mouse Gaa-3124M Recombinant Mouse Gaa Protein, Myc/DDK-tagged HEK293T Myc/DDK
Rat Gaa-163R Recombinant Rat Gaa Protein, His-tagged E.coli His Thr782~Ser953
Rat Gaa-164R Recombinant Rat Gaa Protein, His-tagged E.coli His Glu70~Lys225
Rat GAA-2438R Recombinant Rat GAA Protein Mammalian Cell His
Rat GAA-2094R Recombinant Rat GAA Protein, His (Fc)-Avi-tagged HEK293 His (Fc)-Avi
Rat GAA-2094R-B Recombinant Rat GAA Protein Pre-coupled Magnetic Beads HEK293
Kit-0353 GAA Activity Colorimetric Assay Kit N/A
  • Involved Pathway
  • Protein Function
  • Interacting Protein
  • Other Resource

GAA involved in several pathways and played different roles in them. We selected most pathways GAA participated on our site, such as Galactose metabolism, Starch and sucrose metabolism, Metabolic pathways, which may be useful for your reference. Also, other proteins which involved in the same pathway with GAA were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.

Pathway Name Pathway Related Protein
Galactose metabolismHKDC1;B4GALT2;GALE;AKR1B8;PFKM;AKR1B1;AKR1B7;aLA;PFKL
Starch and sucrose metabolismG6PCA.2;GPIB;AMY2A3;GPI1;GPIA;UGT2A3;PYGMA;PGM1;UGP2A

GAA has several biochemical functions, for example, . Some of the functions are cooperated with other proteins, some of the functions could acted by GAA itself. We selected most functions GAA had, and list some proteins which have the same functions with GAA. You can find most of the proteins on our site.

Function Related Protein

GAA has direct interactions with proteins and molecules. Those interactions were detected by several methods such as yeast two hybrid, co-IP, pull-down and so on. We selected proteins and molecules interacted with GAA here. Most of them are supplied by our site. Hope this information will be useful for your research of GAA.

HIVEP1; STAT2; EP300; ypiP; q9wmx2-pro_0000037548; NUMBL; pi3p; cona_canen

Research Area

Related articles

Lukas, J; Pockrandt, AM; et al. Enzyme Enhancers for the Treatment of Fabry and Pompe Disease. MOLECULAR THERAPY 23:456-464(2015).
Guo, MH; Hundseth, K; et al. A Distinct Triplex DNA Unwinding Activity of ChlR1 Helicase. JOURNAL OF BIOLOGICAL CHEMISTRY 290:5174-5189(2015).
  • Q&As
  • Reviews

Q&As (5)

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How does GAA protein relate to other enzymes involved in glycogen metabolism? 11/09/2021

GAA protein is one of several enzymes involved in glycogen metabolism, each playing a specific role in glycogen breakdown.

What are the treatment options for Pompe disease? 02/27/2021

Enzyme replacement therapy (ERT) and gene therapy are two treatment options available for Pompe disease patients to replace or correct the deficient GAA protein.

Are there any other diseases or conditions related to GAA protein deficiency? 09/30/2020

GAA protein deficiency is primarily associated with Pompe disease, but there are other glycogen storage diseases related to deficiencies of different enzymes.

How does ERT work in treating Pompe disease? 05/30/2020

ERT involves the infusion of recombinant GAA protein to replace the missing or deficient enzyme in Pompe disease patients.

Can GAA protein therapy reverse the effects of Pompe disease? 05/03/2016

While GAA protein therapy can improve the symptoms and quality of life for Pompe disease patients, it may not fully reverse the damage caused by the disease.

Customer Reviews (3)

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    Additionally, cost-effectiveness, competitive pricing, and bulk purchase options can also be advantageous for researchers working within limited budgets.


      The ability to customize the GAA protein variants or modifications offered by the manufacturer is also advantageous, as it allows me to tailor the protein to my specific research objectives.


        I appreciate the manufacturer's commitment to maintaining exceptional protein quality standards, which ensures reliable and consistent results in my trials.

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