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HEXB polyclonal antibody

Product Specifications

Background

Hexosaminidase B is the beta subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes. Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases. Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioside in neurons and neurodegenerative disorders termed the GM2 gangliosidoses. Beta subunit gene mutations lead to Sandhoff disease (GM2-gangliosidosis type II) . Alternatively spliced transcript variants encoding different isoforms have been found for this gene.

CAS Number

9007-83-4

Structure Composition

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

Product Name Alternative

ENC-1AS; HEL-248; HEL-S-111; HEXB

Swiss Prot

P07686

Reactivity

Mouse

Immunogen

Recombinant fusion protein of human HEXB (NP_000512.1) .

Conjugation

Unconjugated

Applications

IF/ICC

Dilution

IF/ICC,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

Unmodification

Host or Source

Rabbit

Available Sizes

Curated Selection

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