GM2A recombinant protein

GM2A Protein, Human, Recombinant (His Tag)

Cat. Num. AAA8121354

• Gene Names: GM2A; SAP-3; GM2-AP
• Purity: >96% as determined by SDS-PAGE
• Synonyms: GM2A; GM2A Protein; Human; Recombinant (His Tag); Human GM2A Protein (His Tag); GM2-AP Protein; SAP-3 Protein; GM2 ganglioside activator; GM2A recombinant protein

• Host: Baculovirus-Insect Cells
• Purity/Purification: >96% as determined by SDS-PAGE
• Form/Format: Lyophilized from sterile 20mM Tris, 500mM NaCl, pH7.4, 10% gly. Normally 5%-8% trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization. Please refer to the specific buffer information in the hard copy of CoA.
• Sequence: Met1-Ile193

• Species: Human
• Endotoxin: <1.0EU per ug of the protein as determined by the LAL method
• Predicted N Terminal: Val 22
• Tag: C-His
• Protein Construction: A DNA sequence encoding the human GM2A (AAA35907.1)(Met 1-Ile 193) was fused with a polyhistidine tag at the C-terminus.
• Reconstitution: A hardcopy of COA with reconstitution instruction is sent along with the products. Please refer to it for detailed information.
• Preparation and Storage: Samples are stable for up to twelve months from date of receipt at -20 degree C to -80 degree C. Store it under sterile conditions at -20 degree C to -80 degree C. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.; In general, recombinant proteins are provided as lyophilized powder which are shipped at ambient temperature. Bulk packages of recombinant proteins are provided as frozen liquid.; They are shipped out with blue ice.

SDS-Page

Related Product Information for GM2A recombinant protein

Background: GM2A (GM2 ganglioside activator), is a lipid transfer protein which belongs to the ML domain family. GM2A can accommodate several single chain phospholipids and fatty acids. It also exhibits some calcium-independent phospholipase activity. GM2A binds gangliosides and stimulates ganglioside GM2 degradation. It stimulates only the breakdown of ganglioside GM2 and glycolipid GA2 by beta-hexosaminidase A. GM2A 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. 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.

References

Wright CS, et al. (2003) Structural analysis of lipid complexes of GM2-activator protein. J Mol Biol. 331(4):951-64.

NCBI and Uniprot Product Information

• NCBI GI #: 183357
• NCBI GeneID: 2760
• Molecular Weight: 20,838 Da
• NCBI Official Full Name: G-M2 activator protein
• 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
• Protein Family: Ganglioside GM2 activator

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]