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

Product Code. 16895784 Shop All Proteintech Products
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150 μL
20 μL
Unit Size:
150µL
20µL
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16895784 20 μL 20µL
16885784 150 μL 150µL
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Product Code. 16895784 Supplier Proteintech Supplier No. 162291AP20UL

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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
Applications Western Blot, Immunohistochemistry (Paraffin)
Classification Polyclonal
Concentration 0.35 mg/mL
Conjugate Unconjugated
Formulation PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
Gene HEXB
Gene Accession No. P07686, P20060, Q6AXR4
Gene Alias ENC 1AS, HCC 7, HEXB, Hexosaminidase subunit B
Gene Symbols Hexb
Host Species Rabbit
Immunogen HEXB Fusion Protein Ag8683
Purification Method Antigen Affinity Chromatography
Quantity 20 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 15212, 294673, 3074
Target Species Rat, Mouse, Human
Content And Storage -20°C
Product Type Antibody
Form Liquid
Isotype IgG
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