HEXB antibody

Catalog No. ABIN7881035

This Mouse Monoclonal antibody specifically detects HEXB in IHC (p). It exhibits reactivity toward Human.

Quick Overview for HEXB antibody (ABIN7881035)

Target: HEXB (Hexosaminidase B (Beta Polypeptide) (HEXB))

Reactivity: Human

Host: Mouse

Clonality: Monoclonal

Conjugate: This HEXB antibody is un-conjugated

Application: Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Clone: HEXB-7762

Product Details anti-HEXB Antibody

Purpose: Hexosaminidase B Antibody / HEXB

Purification: Protein A/G affinity

Immunogen: A recombinant fragment of the human protein was used as the immunogen for the Hexosaminidase B antibody.

Isotype: IgG2, kappa

Application Details

Application Notes: Optimal dilution of the Hexosaminidase B antibody should be determined by the researcher.

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 0.2 mg/mL

Buffer: 0.2 mg/mL in 1X PBS with 0.1 mg/mL BSA (US sourced), 0.05 % sodium azide

Preservative: Sodium azide

Precaution of Use: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Aliquot the Hexosaminidase B antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.

Target Details for HEXB

Target: HEXB (Hexosaminidase B (Beta Polypeptide) (HEXB))

Alternative Name: Hexosaminidase B

Background: Hexosaminidase B (HEXB), also designated beta-hexosaminidase B, is a hexosaminidase B (HEXB), also designated beta-hexosaminidase B, is a tetramer of two beta-A and two beta-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 beta 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.

UniProt: P07686

Pathways: Sensory Perception of Sound, Glycosaminoglycan Metabolic Process