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

Rabbit Polyclonal Antibody

1656.73 SEK - 4721.67 SEK

Specifications

Antigen HEXB
Concentration 0.35 mg/mL
Applications Immunohistochemistry (Paraffin), Western Blot
Classification Polyclonal
Conjugate Unconjugated
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Products 2
Product Code Brand Quantity Price Quantity & Availability  
Product Code Brand Quantity Price Quantity & Availability  
16895784
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Proteintech
16229-1-AP-20UL
20 μL
1656.73 SEK
20µL
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Estimated Shipment: 02-04-2024
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16885784
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Proteintech
16229-1-AP-150UL
150 μL
4721.67 SEK
150µL
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Estimated Shipment: 02-04-2024
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Description

Description

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

Specifications

HEXB
Immunohistochemistry (Paraffin), Western Blot
Unconjugated
Rabbit
Human, Mouse, Rat
P07686, P20060, Q6AXR4
15212, 294673, 3074
HEXB Fusion Protein Ag8683
Primary
-20°C
HEXB
0.35 mg/mL
Polyclonal
Liquid
RUO
PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
ENC 1AS, HCC 7, HEXB, Hexosaminidase subunit B
Hexb
IgG
Antigen Affinity Chromatography
Antibody
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SDS
Documents

Documents

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