BackgroundThis gene encodes a member of the glycosyl hydrolase 20 family of proteins. The encoded preproprotein is proteolytically processed to generate the alpha subunit of the lysosomal enzyme beta-hexosaminidase. This enzyme, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Mutations in this gene lead to an accumulation of GM2 ganglioside in neurons, the underlying cause of neurodegenerative disorders termed the GM2 gangliosidoses, including Tay-Sachs disease (GM2-gangliosidosis type I). Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed.
DescriptionHEXA Monoclonal Antibody. Unconjugated. Raised in: Rabbit.
FormulationBuffer: PBS containing 50% glycerol and 0.05% BSA, preserved with proclin300 or sodium azide, pH 7.3.
SpecificityHuman, Mouse, Rat
IsotypeIgG
Uniprot IDP06865
PurificationAffinity Purified
ImmunogenRecombinant protein (or fragment). Please contact EpigenTek for more information.
StorageStore at -20°C. Avoid freeze / thaw cycles.
Alternative NamesTSD; HEXA
ApplicationWB, IF-P, IHC-P, ELISA; Recommended dilution: WB, 1:500 - 1:1000; IF-P, 1:50 - 1:200; IHC-P, 1:1000 - 1:5000; ELISA, Recommended starting concentration is 1 µg/mL. Please optimize the concentration based on your specific assay requirements.
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