Product Name
Ganglioside GM2 Activator (GM2A), Recombinant Protein
Full Product Name
Recombinant Human Ganglioside GM2 Activator Protein
Product Synonym Names
Ganglioside GM2 activator; Cerebroside sulfate activator protein; GM2-AP; Sphingolipid activator protein 3; SAP-3
Product Gene Name
GM2A recombinant protein
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Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for P17900
Purity/Purification
>95% as determined by reducing SDS-PAGE.
Form/Format
Lyophilized from a 0.2 mum filtered solution of 20mM TrisHCl, 150mM NaCl, pH7.5.
Endotoxin
<1.0 EU per ug as determined by LAL test.
Preparation and Storage
Lyophilized protein should be stored at < -20 degree C, though stable at room temperature for 3 weeks.
Reconstituted protein solution can be stored at 4-7 degree C for 2-7 days.
Aliquots of reconstituted samples are stable at < -20 degree C for 3 months.
ISO Certification
Manufactured in an ISO 9001:2015 Certified Laboratory.
Other Notes
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.
Related Product Information for
GM2A recombinant protein
Protein Construction: Recombinant Human Ganglioside GM2 activator is produced by our Mammalian expression system and the target gene encoding Ser32-Ile193 is expressed with a 6His tag at the C-terminus.
Background: Ganglioside GM2 activator (GM2A) is a small glycolipid transport protein which acts as a substrate specific co-factor for the lysosomal enzyme beta-hexosaminidase A (HEXB). HEXB together with GM2A, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. GM2A accommodate several single chain phospholipids and fatty acids, is a lipid transfer protein that stimulates the enzymatic processing of gangliosides, and also T-cell activation through lipid presentation. 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.
NCBI/Uniprot data below describe general gene information for GM2A. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000396.2
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NCBI GenBank Nucleotide #
NM_000405.4
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UniProt Primary Accession #
P17900
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UniProt Secondary Accession #
Q14426; Q14428; Q6LBL5; B2R699; D3DQH6[Other Products]
UniProt Related Accession #
P17900[Other Products]
Molecular Weight
Molecular Mass: 18.6kDa
Actual Protein?Molecular Mass: 20kDa[Actual+Protein?Molecular+Mass:+20kDa" title="Find similar products with Molecular Mass: 18.6kDa
Actual Protein?Molecular Mass: 20kDa">Similar Products]
NCBI Official Full Name
ganglioside GM2 activator isoform 1
NCBI Official Synonym Full Names
GM2 ganglioside activator
NCBI Official Symbol
GM2A??[Similar Products]
NCBI Official Synonym Symbols
SAP-3; GM2-AP
??[Similar Products]
NCBI Protein Information
ganglioside GM2 activator
UniProt Protein Name
Ganglioside GM2 activator
UniProt Synonym Protein Names
Cerebroside sulfate activator protein; GM2-AP; Sphingolipid activator protein 3; SAP-3
Protein Family
Ganglioside GM2 activator
UniProt Gene Name
GM2A??[Similar Products]
UniProt Synonym Gene Names
SAP-3??[Similar Products]
NCBI Summary for GM2A
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 Comments for 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.
Research Articles on GM2A
1. Review of GM2A mutations causing GM2 activator protein deficiency and GM2 gangliosidosis-AB variant.
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.
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