HEXB Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech

Rabbit Polyclonal Antibody

Brand:  Proteintech 16229-1-AP-20UL

Product Code. 16895784

Description

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.

Specifications

• Antigen: HEXB
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: HEXB
• Gene Alias: ENC 1AS, HCC 7, HEXB, Hexosaminidase subunit B
• Host Species: Rabbit
• Purification Method: Antigen Affinity Chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 15212, 294673, 3074
• Content And Storage: -20°C
• Form: Liquid

• Applications: Western Blot, Immunohistochemistry (Paraffin)
• Concentration: 0.35 mg/mL
• Formulation: PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
• Gene Accession No.: P07686, P20060, Q6AXR4
• Gene Symbols: Hexb
• Immunogen: HEXB Fusion Protein Ag8683
• Quantity: 20 μL
• Primary or Secondary: Primary
• Target Species: Rat, Mouse, Human
• Product Type: Antibody
• Isotype: IgG