Hexb (S516) polyclonal Antibody | BS1917

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: