Invitrogen™ HEXB Polyclonal Antibody

Rabbit Polyclonal Antibody

Supplier:  Invitrogen™ PA5101082

Catalog No. PIPA5101082

Description

Antibody detects endogenous levels of total HEXB.

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: 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
• Host Species: Rabbit
• Purification Method: Affinity chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 15212, 294673, 3074
• Content And Storage: -20°C
• Form: Liquid

• Applications: Immunohistochemistry (Paraffin), Western Blot, Immunocytochemistry
• Concentration: 1 mg/mL
• Formulation: PBS with 50% glycerol and 0.02% sodium azide; pH 7.4
• Gene Accession No.: P07686, P20060, Q6AXR4
• Gene Symbols: Hexb
• Immunogen: A synthesized peptide derived from human HEXB(Accession P07686), corresponding to amino acid residues T496-L546.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human, Mouse, Rat
• Product Type: Antibody
• Isotype: IgG

Safety and Handling

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