HEXB chain A Rabbit anti-Human∣Mouse∣Rat, Polyclonal, Bioss

Catalog No.	Quantity
50-198-0892	100 μL

Catalog No. 50-198-0892 Supplier Bioss Supplier No. BS10463R

Description

Rabbit Polyclonal Antibody

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 chain A
• Applications: Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
• Classification: Polyclonal
• Concentration: 1 μg/mL
• Conjugate: Unconjugated
• Formulation: PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH
• Gene: Hexb
• Gene Accession No.: P07686, P20060, Q6AXR4
• Gene Alias: Beta-hexosaminidase subunit beta; Beta-hexosaminidase subunit beta chain A; Beta-hexosaminidase subunit beta chain B; Beta-N-acetylhexosaminidase subunit beta; Cervical cancer proto-oncogene 7 protein; ENC-1AS; epididymis luminal protein 248; epididymis secretory protein Li 111; HCC7; HCC-7; HEL-248; HEL-S-111; Hexb; hexosaminidase B; hexosaminidase B (beta polypeptide); Hexosaminidase subunit B; hexosaminidase subunit beta; N-acetyl-beta-glucosaminidase subunit beta
• Gene Symbols: Hexb
• Host Species: Rabbit
• Immunogen: KLH conjugated synthetic peptide derived from human HEXB chain A.
• Purification Method: Protein A
• Quantity: 100 μL
• Regulatory Status: RUO
• Primary or Secondary: Primary
• Gene ID (Entrez): 15212, 294673, 3074
• Target Species: Human, Mouse, Rat
• Content And Storage: -20°C
• Product Type: Antibody
• Form: Liquid
• Isotype: IgG