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Hexb (S516) polyclonal antibody

Product Specifications

Background

Hexosaminidase B (HEXB), also designated beta-hexosaminidase B, is a Hexosaminidase B (HEXB), also designated b-hexosaminidase B, is a tetramer of two b-A and two b-B chains and is found in the lysosomes of cells. Sandhoff disease (SD), also known as GM2-gangliosidosis type II, is caused by mutations in the HEXB gene that affect the b subunit. These mutations disrupt the activity of HEXB and HEXA, which prevents the breakdown of GM2 ganglioside, a fatty material found in the brain, therby rendering both the HEXA and HEXB enzymes deficient. SD is a rare autosomal recessive disorder characterized by an accumulation of GM2 ganglioside, which causes progressive destruction of the central nervous system. Sandhoff disease is similar to Tay-Sachs disease, which is caused by mutations in the HEXA gene, although SD is more severe.

CAS Number

9007-83-4

Structure Composition

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

Product Name Alternative

Beta-hexosaminidase subunit beta; Beta-N-acetylhexosaminidase subunit beta; Hexosaminidase subunit B; Cervical cancer proto-oncogene 7 protein; HCC-7; N-acetyl-beta-glucosaminidase subunit beta; HEXB; HCC7

Swiss Prot

P07686

Reactivity

Human

Immunogen

Synthetic peptide, corresponding to amino acids 481-530 of Human Hexb.

Conjugation

Unconjugated

Applications

WB IHC

Dilution

WB: 1:500~1:1000 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

~ 63 kDa

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

Hexb (S516) polyclonal antibody detects endogenous levels of Hexb protein.

Applications Notes

Western blot (WB) analysis of Hexb (S516) pAb at 1:500 dilution Lane1:A549 whole cell lysate (40ug) Lane2:MCF-7 whole cell lysate (40ug) Lane3:U-87MG whole cell lysate (40ug)

Host or Source

Rabbit

Available Sizes

Frequently Asked Questions

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