Human GM2 Ganglioside Activator protein ELISA Kit | MyBioSource MBS722287 product information

product summary

company name :

MyBioSource

product type :

ELISA/assay

product name :

Human GM2 Ganglioside Activator protein ELISA Kit

catalog :

MBS722287

quantity :

48-Strip-Wells

price :

470 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS722287

products type :

ELISA Kit

products full name :

Human GM2 Ganglioside Activator protein ELISA Kit

products short name :

GM2 Ganglioside Activator protein

other names :

ganglioside GM2 activator isoform 2; Ganglioside GM2 activator; ganglioside GM2 activator; shingolipid activator protein 3; sphingolipid activator protein 3; cerebroside sulfate activator protein; GM2 ganglioside activator; Cerebroside sulfate activator protein; GM2-AP; Sphingolipid activator protein 3; SAP-3

products gene name :

GM2AP

other gene names :

GM2A; GM2A; SAP-3; GM2-AP; SAP-3

uniprot entry name :

SAP3_HUMAN

reactivity :

Human

specificity :

This assay has high sensitivity and excellent specificity for detection of GM2. No significant cross-reactivity or interference between GM2 and analogues was observed. NOTE: Limited by current skills and knowledge, it is impossible for us to complete the cross-reactivity detection between GM2 and all the analogues, therefore, cross reaction may still exist in some cases.

storage stability :

Store all reagents at 2-8 degree C.

other info1 :

Samples: Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate. Assay Type: Competitive. Sensitivity: 0.1 ng/mL.

other info2 :

Intended Uses: This GM2 ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Human GM2. This ELISA kit for research use only, not for therapeutic or diagnostic applications!

products categories :

Signal Transduction

products description :

Principle of the assay: GM2 ELISA kit applies the competitive enzyme immunoassay technique utilizing a monoclonal anti-GM2 antibody and an GM2-HRP conjugate. The assay sample and buffer are incubated together with GM2-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 GM2 concentration since GM2 from samples and GM2-HRP conjugate compete for the anti-GM2 antibody binding site. Since the number of sites is limited, as more sites are occupied by GM2 from the sample, fewer sites are left to bind GM2-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The GM2 concentration in each sample is interpolated from this standard curve.

ncbi gi num :

263190474

ncbi acc num :

NP_001161079.1

ncbi gb acc num :

NM_001167607.1

ncbi mol weight :

20,838 Da

ncbi pathways :

Glycosphingolipid Metabolism Pathway (530751); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolism Pathway (477135); Metabolism Of Lipids And Lipoproteins Pathway (160976); Sphingolipid Metabolism Pathway (119543)

ncbi summary :

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 summary :

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; mitochondrion; apical cortex; 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; lipid transport; neuromuscular process controlling balance. Disease: Gm2-gangliosidosis, Ab Variant

size1 :

48-Strip-Wells

price1 :

470 USD

size2 :

96-Strip-Wells

price2 :

675

more info or order :

MyBioSource product webpage