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HEXB Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech
Rabbit Polyclonal Antibody
Brand: Proteintech 16229-1-AP-150UL
Additional Details : Weight : 0.00010kg
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DescriptionHexosaminidase 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.
|ENC 1AS, HCC 7, HEXB, Hexosaminidase subunit B|
|Antigen Affinity Chromatography|
|15212, 294673, 3074|
|Western Blot, Immunohistochemistry (Paraffin)|
|PBS with 50% glycerol and 0.02% sodium azide; pH 7.3|
|P07686, P20060, Q6AXR4|
|HEXB Fusion Protein Ag8683|
|Rat, Mouse, Human|