Goat GM2 Ganglioside Activator protein ELISA Kit | GM2AP elisa kit

Goat GM2 Ganglioside Activator protein ELISA Kit

Cat. Num. AAA753773

• Gene Names: GM2A; SAP-3; GM2-AP
• Reactivity: Goat
• Synonyms: GM2 Ganglioside Activator protein; Goat GM2 Ganglioside Activator protein ELISA Kit; GM2AP elisa kit

• Reactivity: Goat

• Samples: Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate
• Assay Type: Competitive
• Sensitivity: 0.1 ng/mL.
• Preparation and Storage: Store all reagents at 2-8 degree C.

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for GM2AP elisa kit

Intended Uses: This GM2AP ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Goat GM2AP. This ELISA kit for research use only!

Principle of the Assay: GM2AP ELISA kit applies the competitive enzyme immunoassay technique utilizing a monoclonal anti-GM2AP antibody and an GM2AP-HRP conjugate. The assay sample and buffer are incubated together with GM2AP-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the GM2AP concentration since GM2AP from samples and GM2AP-HRP conjugate compete for the anti-GM2AP antibody binding site. Since the number of sites is limited, as more sites are occupied by GM2AP from the sample, fewer sites are left to bind GM2AP-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The GM2AP concentration in each sample is interpolated from this standard curve.

Product Categories/Family for GM2AP elisa kit

Signal Transduction

NCBI and Uniprot Product Information

• NCBI GI #: 263190474
• NCBI GeneID: 2760
• NCBI Accession #: NP_001161079.1
• NCBI GenBank Nucleotide #: NM_001167607.1
• Molecular Weight: 20,838 Da
• NCBI Official Full Name: ganglioside GM2 activator isoform 2
• NCBI Official Synonym Full Names: GM2 ganglioside activator
• NCBI Official Symbol: GM2A
• NCBI Official Synonym Symbols: SAP-3; GM2-AP
• NCBI Protein Information: ganglioside GM2 activator; shingolipid activator protein 3; sphingolipid activator protein 3; cerebroside sulfate activator protein
• UniProt Protein Name: Ganglioside GM2 activator
• UniProt Gene Name: GM2A
• UniProt Synonym Gene Names: SAP-3
• UniProt Entry Name: SAP3_HUMAN

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: Mitochondrial; Activator

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

Disease: Gm2-gangliosidosis, Ab Variant