GM2A antibody pair
GM2A Antibody Pair
Detection: Rabbit
Detection: IgG
(Standards are recommendations only. Compatibility of the standards in the user developed application needs to be established and confirmed by the researcher.
Detection: 50% Glycerol, 0.01M PBS, PH 7.4
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a small glycolipid transport protein which acts as a substrate specific co-factor for the lysosomal enzyme beta-hexosaminidase A. Beta-hexosaminidase A, together with GM2 ganglioside activator, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Mutations in this gene result in GM2-gangliosidosis type AB or the AB variant of Tay-Sachs disease. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Nov 2009]
Uniprot Description
GM2A: The large binding pocket can accommodate several single chain phospholipids and fatty acids, GM2A also exhibits some calcium-independent phospholipase activity. Binds gangliosides and stimulates ganglioside GM2 degradation. It stimulates only the breakdown of ganglioside GM2 and glycolipid GA2 by beta-hexosaminidase A. It extracts single GM2 molecules from membranes and presents them in soluble form to beta- hexosaminidase A for cleavage of N-acetyl-D-galactosamine and conversion to GM3. Defects in GM2A are the cause of GM2-gangliosidosis type AB (GM2GAB); also known as Tay-Sachs disease AB variant. GM2-gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. GM2GAB is characterized by GM2 gangliosides accumulation in the presence of both hexosaminidase A and B.
Protein type: Activator; Mitochondrial
Chromosomal Location of Human Ortholog: 5q33.1
Cellular Component: apical cortex; hydrogen:potassium-exchanging ATPase complex; internal side of plasma membrane; lysosomal lumen; mitochondrion
Molecular Function: beta-N-acetylgalactosaminidase activity; lipid transporter activity; phospholipase activator activity; sphingolipid activator protein activity
Biological Process: ganglioside catabolic process; glycosphingolipid metabolic process; learning and/or memory; lipid transport; neuromuscular process controlling balance; oligosaccharide catabolic process; positive regulation of hydrolase activity; sequestering of lipid
Disease: Gm2-gangliosidosis, Ab Variant
Research Articles on GM2A
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Product Notes
The GM2A gm2a (Catalog #AAA7041786) is an Antibody Pair produced from Capture: Rabbit Detection: Rabbit and is intended for research purposes only. The product is available for immediate purchase. The GM2A Antibody Pair reacts with Mouse, Guinea Pig, Bovine, Pig, Sheep and may cross-react with other species as described in the data sheet. AAA Biotech's GM2A can be used in a range of immunoassay formats including, but not limited to, Sandwich ELISA (EIA). We recommend using the capture antibody at a concentration of 0.4ug/ml and the detection antibody at a concentration of 0.34ug/ml. Researchers should empirically determine the suitability of the GM2A gm2a 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 "GM2A, Polyclonal Antibody Pair" 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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