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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