HEXA polyclonal antibody

Product Specifications

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.

CAS Number

9007-83-4

Structure Composition

1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Product Name Alternative

HEXA; TSD

Swiss Prot

P06865

Reactivity

Human, Mouse, Rat

Immunogen

Recombinant fusion protein of human HEXA (NP_000511.2) .

Conjugation

Unconjugated

Applications

WB, IHC

Dilution

WB,1:500 - 1:2000|IHC,1:50 - 1:200

Purity

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .

Modification

Unmodification

Molecular Weight

Refer to figures

Storage Conditions

Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Notes

For research use only, not for use in diagnostic procedure.

Specificity

Polyclonal Antibodies

Applications Notes

Immunohistochemistry of paraffin-embedded rat brain using HEXA antibody at dilution of 1:100 .Perform microwave antigen retrieval with 10 mM PBS buffer pH 7.2 before commencing with IHC staining protocol.