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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