GM2A Human; GM2 Ganglioside Activator Human Recombinant; Ganglioside GM2 activator; Cerebroside sulfate activator protein; GM2-AP; Sphingolipid activator protein 3; SAP-3; GM2A

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

E Coli

Purity as determined by densitometric image analysis is greater than 95%.

GM2A filtered (0.4 um) and lyophilized from 0.5mg/ml in 0.05M Tris buffer and 0.1M NaCl, pH-8.0.
Filtered White lyophilized (freeze-dried) powder.

Store lyophilized protein at -20 degree C. Aliquot the product after reconstitution to avoid repeated freezing/thawing cycles. Reconstituted protein can be stored at 4 degree C for a limited period of time; it does not show any change after two weeks at 4 degree C.

Small volumes of GM2A recombinant protein 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.

Description: GM2A Human Recombinant produced in E Coli is a single, non-glycosylated, polypeptide chain (a.a 33-193) containing 170 amino acids including a 9 a.a N-terminal His tag. The total molecular mass is 18.7kDa (calculated).

Introduction: Ganglioside GM2 activator (GM2A) is a small glycolipid transport protein which functions as a substrate specific co-factor for the lysosomal enzyme beta-hexosaminidase A. GM2A participates in lipid metabolism. GM2A protein is localized in lysosomes. The GM2A protein forms a large binding pocket for a number of single chain phospholipids and fatty acids. GM2A demonstrates calcium-independent phospholipase activity. GM2A stimulates ganglioside GM2 degradation by presenting GM2 to beta-hexosaminidase A. The Beta-hexosaminidase A, along with GM2A, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. GM2A gene mutations result in GM2-gangliosidosis type AB or the AB variant of Tay-Sachs disease.

RECOMBINANT & NATURAL PROTEINS; Recombinant Proteins

20,838 Da

GM2 ganglioside activator

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

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: Mitochondrial; Activator

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; sphingolipid metabolic process; learning and/or memory; 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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