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Mouse Anti-GM2A Recombinant Antibody (2C8) (CBMAB-G3991-LY)

This product is antibody recognizes GM2A. The antibody 2C8 immunoassay techniques such as: ELISA.
See all GM2A antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
2C8
Antibody Isotype
IgG2b, κ
Application
ELISA

Basic Information

Specificity
Human
Antibody Isotype
IgG2b, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Concentration
1 mg/mL
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
GM2 Ganglioside Activator
Introduction
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]
Entrez Gene ID
2760
UniProt ID
P17900
Alternative Names
GM2 Ganglioside Activator; Cerebroside Sulfate Activator Protein; Sphingolipid Activator Protein 3; GM2-AP; SAP-3; GM2 Ganglioside Activator Protein; Shingolipid Activator Protein 3; Ganglioside GM2 Activator;
Function
The large binding pocket can accommodate several single chain phospholipids and fatty acids, GM2A also exhibits some calcium-independent phospholipase activity (By similarity).

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 (By similarity).

Has cholesterol transfer activity (PubMed:17552909).
Biological Process
Ganglioside catabolic process Source: GO_Central
Glycosphingolipid metabolic process Source: Reactome
Learning or memory Source: Ensembl
Lipid storage Source: Ensembl
Lipid transport Source: GO_Central
Neuromuscular process controlling balance Source: Ensembl
Oligosaccharide catabolic process Source: Ensembl
Positive regulation of hydrolase activity Source: Ensembl
Cellular Location
Lysosome
Involvement in disease
GM2-gangliosidosis AB (GM2GAB):
An autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. It is characterized by GM2 gangliosides accumulation in the presence of both normal hexosaminidase A and B.
PTM
The serines in positions 32 and 33 are absent in 80% of the sequenced protein.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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