Immunohistochemistry (IHC)
(Anti-GAA antibody IHC staining of human prostate. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody dilution 1:50.)
Rabbit anti-Human GAA / Alpha-Glucosidase, Acid Polyclonal Antibody | anti-GAA antibody
Anti-GAA / Alpha-Glucosidase, Acid Antibody (aa173-203) IHC-plus
WB (1:1000)
For long term storage store at -20 degree C.
Immunohistochemistry (IHC)
(Anti-GAA antibody IHC staining of human prostate. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody dilution 1:50.)
Immunohistochemistry (IHC)
(Anti-GAA antibody IHC staining of human prostate. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody dilution 1:50.)
Western Blot (WB)
(GAA Antibody western blot of HL-60 cell line lysates (35 ug/lane). The GAA antibody detected the GAA protein (arrow).)
Western Blot (WB)
(GAA Antibody western blot of HL-60 cell line lysates (35 ug/lane). The GAA antibody detected the GAA protein (arrow).)
Western Blot (WB)
(Western blot of lysates from A549, MCF-7, SW620, PC-3 cell line (from left to right), using GAA Antibody. Antibody was diluted at 1:1000 at each lane. A goat anti-rabbit IgG H&L (HRP) at 1:10000 dilution was used as the secondary antibody. Lysates at 20ug per lane.)
Western Blot (WB)
(Western blot of lysates from A549, MCF-7, SW620, PC-3 cell line (from left to right), using GAA Antibody. Antibody was diluted at 1:1000 at each lane. A goat anti-rabbit IgG H&L (HRP) at 1:10000 dilution was used as the secondary antibody. Lysates at 20ug per lane.)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2016]
Uniprot Description
GAA: Essential for the degradation of glygogen to glucose in lysosomes. Defects in GAA are the cause of glycogen storage disease type 2 (GSD2); 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. Belongs to the glycosyl hydrolase 31 family.
Protein type: Hydrolase; EC 3.2.1.20; Carbohydrate Metabolism - galactose; Carbohydrate Metabolism - starch and sucrose; Contractile
Chromosomal Location of Human Ortholog: 17q25.2-q25.3
Cellular Component: lysosomal lumen; lysosomal membrane; lysosome; membrane
Molecular Function: alpha-glucosidase activity; carbohydrate binding; maltase activity
Biological Process: carbohydrate metabolic process; cardiac muscle contraction; diaphragm contraction; glucose metabolic process; glycogen catabolic process; heart morphogenesis; locomotory behavior; lysosome organization and biogenesis; maltose metabolic process; muscle maintenance; neuromuscular process controlling balance; neuromuscular process controlling posture; regulation of the force of heart contraction; sucrose metabolic process; tissue development; vacuolar sequestering
Disease: Glycogen Storage Disease Ii
Research Articles on GAA
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Product Notes
The GAA gaa (Catalog #AAA2400046) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Anti-GAA / Alpha-Glucosidase, Acid Antibody (aa173-203) IHC-plus reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's GAA / Alpha-Glucosidase, Acid can be used in a range of immunoassay formats including, but not limited to, Immunohistochemistry (IHC) Paraffin, Western Blot (WB). IHC-P (1:50) WB (1:1000). Researchers should empirically determine the suitability of the GAA gaa for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "GAA / Alpha-Glucosidase, Acid, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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