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Invitrogen™ HEXB Polyclonal Antibody
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Catalog No. PIPA5101082
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Quantity:
100 μL
Catalog No. Quantity
PIPA5101082 100 μL
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Catalog No. PIPA5101082

Supplier: Invitrogen™ PA5101082

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Description

Rabbit Polyclonal Antibody

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.
TRUSTED_SUSTAINABILITY

Specifications

Antigen HEXB
Applications Immunohistochemistry (Paraffin), Western Blot, Immunocytochemistry
Classification Polyclonal
Concentration 1 mg/mL
Conjugate Unconjugated
Formulation PBS with 50% glycerol and 0.02% sodium azide; pH 7.4
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 A synthesized peptide derived from human HEXB(Accession P07686), corresponding to amino acid residues T496-L546.
Purification Method Affinity chromatography
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
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