BW
Hexb (S516) polyclonal Antibody | BS1917
- SKU:
- BW-BS1917
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- Usually ships in 5 working days
Description
Hexb (S516) polyclonal Antibody | BS1917 | Gentaur UK, US & Europe Distribution
Host: Rabbit
Reactivity: Human
Application: WB IHC
Application Range: WB: 1:500~1:1000 IHC: 1:50~1:200
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.
Storage & Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Specificity: Hexb (S516) polyclonal Antibody detects endogenous levels of Hexb protein.
Molecular Weight: ~ 63 kDa
Note: For research use only, not for use in diagnostic procedure.
Alternative Names: 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
Immunogen: Synthetic peptide, corresponding to amino acids 481-530 of Human Hexb.
Conjugate: Unconjugated
Modification: Unmodification
Purification & Purity: The Antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .
Pathway: