Invitrogen™ HEXB Polyclonal Antibody

Rabbit Polyclonal Antibody

Supplier:  Invitrogen™ PA563236

Catalog No. PIPA563236

Description

Immunogen sequence: FGFYKWHHEP AEFQAKTQVQ QLLVSITLQS ECDAFPNISS DESYTLLVKE PVAVLKANRV W Highest antigen sequence identity to the following orthologs: Mouse - 72%, Rat - 69%.

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: Antigen affinity chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 3074
• Content And Storage: Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
• Form: Liquid

• Applications: Immunohistochemistry (Paraffin)
• Concentration: 0.05 mg/mL
• Formulation: PBS with 40% glycerol and 0.02% sodium azide; pH 7.2
• Gene Accession No.: P07686
• Gene Symbols: Hexb
• Immunogen: Recombinant protein corresponding to Human HEXB. Recombinant protein control fragment (Product #RP-100907).
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG