Product Description

Hexb (S516) Polyclonal Antibody

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

Catalogue Numbers: BS1917-50, BS1917-100

Sizes: 50µl, 100µl

Swiss-Prot: P07686

Host: Rabbit

Reactivity: Human

Applications: WB, IHC

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.

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

Purification and Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).

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

Note: For research use only, not for use in diagnostic procedure.

Extra Notes: Western blot (WB) analysis of Hexb (S516) pAb at 1:500 dilution
Lane 1: A549 whole cell lysate (40ug)
Lane 2: MCF-7 whole cell lysate (40ug)
Lane 3: U-87MG whole cell lysate (40ug), Immunohistochemistry (IHC) analyzes of Hexb (S516) pAb in paraffin-embedded human breast carcinoma tissue at 1:100.

Bioworld Molecular Weight: ~ 63 kDa

Storage and Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Alternative Name: 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: Unmodified