Western Blot (WB)
(Western Blot: Sample: Recombinant GaA, Rat.)
Rabbit anti-Rat Glucosidase Alpha Polyclonal Antibody | anti-GaA antibody
Glucosidase Alpha, Acid (GaA) Polyclonal Antibody
Immunohistochemistry in formalin fixed frozen section: 1:50-500
Enzyme-linked Immunosorbent Assay: 1:100-200
Usage: 10uL per well when 3,3'-Diaminobenzidine(DAB) as the substrate.
5uL per well when used in enhanced chemilumescent (ECL).
Note: The quality control is specifically manufactured as the positive control.Not used for other purposes.
Loading Buffer: 100mM Tris(pH8.8), 2% SDS, 200mM NaCl, 50% glycerol,BPB 0.01%, NaN3 0.02%.
Western Blot (WB)
(Western Blot: Sample: Recombinant GaA, Rat.)
Western Blot (WB)
(Western Blot: Sample: Recombinant GaA, Rat.)
Immunohistochemistry (IHC)
(DAB staining on fromalin fixed paraffin- embedded Kidney tissue))
Immunohistochemistry (IHC)
(DAB staining on fromalin fixed paraffin- embedded Kidney tissue))
NCBI and Uniprot Product Information
NCBI Description
human homolog catalyzes the degradation of glycogen [RGD, Feb 2006]
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: 10q32.3
Cellular Component: integral component of membrane; lysosomal membrane; lysosome; membrane
Molecular Function: alpha-1,4-glucosidase activity; carbohydrate binding; maltase activity; oligo-1,6-glucosidase activity
Biological Process: cardiac muscle contraction; diaphragm contraction; glycogen catabolic process; glycogen metabolic 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; striated muscle contraction; tissue development; vacuolar sequestering
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Product Notes
The GaA gaa (Catalog #AAA2032509) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Glucosidase Alpha, Acid (GaA) Polyclonal Antibody reacts with Rat and may cross-react with other species as described in the data sheet. AAA Biotech's Glucosidase Alpha can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (EIA). Western Blot: 1:50-400 Immunohistochemistry in formalin fixed frozen section: 1:50-500 Enzyme-linked Immunosorbent Assay: 1:100-200. 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 "Glucosidase Alpha, 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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