Western Blot (WB)
(Western Blot analysis of GAA expression in transfected 293T cell line using 127076. Lane 1: GAA transfected lysate (105.3kD). Lane 2: Non-transfected lysate.)
Rabbit anti-Human, Mouse GAA Polyclonal Antibody | anti-GAA antibody
GAA (Lysosomal alpha-glucosidase, Acid Maltase, Aglucosidase alfa) (MaxLight 750)
Western Blot (WB)
(Western Blot analysis of GAA expression in transfected 293T cell line using 127076. Lane 1: GAA transfected lysate (105.3kD). Lane 2: Non-transfected lysate.)
Western Blot (WB)
(Western Blot analysis of GAA expression in transfected 293T cell line using 127076. Lane 1: GAA transfected lysate (105.3kD). Lane 2: Non-transfected lysate.)
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: Carbohydrate Metabolism - galactose; Carbohydrate Metabolism - starch and sucrose; Contractile; EC 3.2.1.20; Hydrolase
Chromosomal Location of Human Ortholog: 17q25.3
Cellular Component: lysosomal lumen; lysosomal membrane; lysosome; membrane
Molecular Function: alpha-glucosidase activity; oligo-1,6-glucosidase activity
Biological Process: cardiac muscle contraction; diaphragm contraction; glucose metabolic process; glycogen catabolic process; lysosome organization and biogenesis; maltose metabolic process; sucrose metabolic process; vacuolar sequestering
Disease: Glycogen Storage Disease Ii
Research Articles on GAA
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Product Notes
The GAA gaa (Catalog #AAA6379129) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The GAA (Lysosomal alpha-glucosidase, Acid Maltase, Aglucosidase alfa) (MaxLight 750) reacts with Human, Mouse and may cross-react with other species as described in the data sheet. AAA Biotech's GAA can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Applications are based on unconjugated antibody. 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, 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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