Product Name
Ganglioside GM2 activator (GM2A), Polyclonal Antibody
Full Product Name
Rabbit anti- human Ganglioside GM2 activator polyclonal Antibody, HRP conjugated
Product Gene Name
anti-GM2A antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for P17900
Species Reactivity
Human, Mouse
Purity/Purification
Caprylic Acid Ammonium Sulfate Precipitation Purified
Immunogen
Recombinant human Ganglioside GM2 activator protein
Preparation and Storage
Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of anti-GM2A antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-GM2A antibody
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.
Applications Tested/Suitable for anti-GM2A antibody
ELISA (EIA)
NCBI/Uniprot data below describe general gene information for GM2A. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000396.2
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NCBI GenBank Nucleotide #
NM_000405.4
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UniProt Primary Accession #
P17900
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UniProt Secondary Accession #
Q14426; Q14428; Q6LBL5; B2R699; D3DQH6[Other Products]
UniProt Related Accession #
P17900[Other Products]
Molecular Weight
20,838 Da[Similar Products]
NCBI Official Full Name
ganglioside GM2 activator isoform 1
NCBI Official Synonym Full Names
GM2 ganglioside activator
NCBI Official Symbol
GM2A??[Similar Products]
NCBI Official Synonym Symbols
SAP-3; GM2-AP
??[Similar Products]
NCBI Protein Information
ganglioside GM2 activator
UniProt Protein Name
Ganglioside GM2 activator
UniProt Synonym Protein Names
Cerebroside sulfate activator protein; GM2-AP; Sphingolipid activator protein 3; SAP-3
Protein Family
Ganglioside GM2 activator
UniProt Gene Name
GM2A??[Similar Products]
UniProt Synonym Gene Names
SAP-3??[Similar Products]
UniProt Entry Name
SAP3_HUMAN
NCBI Summary for GM2A
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 Comments for GM2A
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
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; sphingolipid metabolic process
Disease: Gm2-gangliosidosis, Ab Variant
Research Articles on GM2A
1. Gene polymorphisms of MD2 and GM2A were associated with the occurrence or severity of neonatal necrotizing enterocolitis.
Precautions
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Disclaimer
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