GM2 Ganglioside Activator, Polyclonal Antibody

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

Polyclonal

IgG

Rabbit

The antibody is a rabbit polyclonal antibody raised against GM2A. It has beenselected for its ability to recognize GM2A in immunohistochemical staining andwestern blotting.

Affinity Chromatography

Liquid

200ug/ml (lot specific)

Store at 4 degree C for frequent use. Stored at -20 degree C to -80 degree C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customer’s specifications, please inquire.

Small volumes of anti-GM2A antibody 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.

Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (EIA)

Western blotting: 0.5-2ug/ml
Immunocytochemistry in formalin fixed cells: 5-20ug/ml
Immunohistochemistry in formalin fixed frozen section: 5-20ug/ml
Immunohistochemistry in paraffin section: 5-20ug/ml
Enzyme-linked Immunosorbent Assay: 0.05-2ug/ml
Optimal working dilutions must be determined by end user.

Western Blot: Sample: Recombinant GM2A, Human.

DABstainingonIHC-P.Samples:HumanTissue)

20,838 Da

GM2 ganglioside activator

ganglioside GM2 activator

Ganglioside GM2 activator

SAP-3  [Similar Products]

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: Activator; Mitochondrial

Chromosomal Location of Human Ortholog: 5q33.1

Cellular Component: apical cortex; cytoplasmic side of plasma membrane; extracellular region; hydrogen:potassium-exchanging ATPase complex; lysosomal lumen; mitochondrion

Molecular Function: beta-N-acetylgalactosaminidase activity; lipid transporter activity; phospholipase activator activity; sphingolipid activator protein activity

Biological Process: ganglioside catabolic process; glycosphingolipid metabolic process; learning and/or memory; lipid storage; lipid transport; neuromuscular process controlling balance; neutrophil degranulation; oligosaccharide catabolic process; positive regulation of hydrolase activity

Disease: Gm2-gangliosidosis, Ab Variant

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