Background:

This 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.

Clonality:

polyclonal

Classification:

Primary antibody

Alternative names:

N/A

Specificity:

Human, Mouse, Rat

Host:

Rabbit

Isotype:

IgG

Immunogen:

hexosaminidase A (alpha polypeptide)

Purification:

Immunogen affinity purified

Purity:

≥95% as determined by SDS-PAGE

Calculated molecular weight:

Refer to figures

Form:

liquid

Applications:

ELISA, WB, IHC

Recommended dilution:

WB: 1:500 - 1:2000; IHC: 1:50 - 1:200

Storage:

PBS with 0.02% sodium azide and 50% glycerol pH 7.3 , -20℃ for 12 months (Avoid repeated freeze / thaw cycles.)

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