GM2 Ganglioside Activator, ELISA Kit

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C

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Small volumes of GM2A elisa kit 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.

MBS068278 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the GM2 Ganglioside Activator (GM2A) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing GM2A. The ELISA analytical biochemical technique of the MBS068278 kit is based on GM2A antibody-GM2A antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect GM2A antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, GM2A. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

20,838 Da

GM2 ganglioside activator

ganglioside GM2 activator; shingolipid activator protein 3; sphingolipid activator protein 3; cerebroside sulfate activator protein

Ganglioside GM2 activator

SAP-3  [Similar Products]

SAP3_HUMAN

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]

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: lysosomal lumen; internal side of plasma membrane; apical cortex; mitochondrion; hydrogen:potassium-exchanging ATPase complex

Molecular Function: lipid transporter activity; beta-N-acetylgalactosaminidase activity; phospholipase activator activity

Biological Process: oligosaccharide catabolic process; sequestering of lipid; learning and/or memory; sphingolipid metabolic process; ganglioside catabolic process; positive regulation of hydrolase activity; glycosphingolipid metabolic process; neuromuscular process controlling balance; lipid transport

Disease: Gm2-gangliosidosis, Ab Variant

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