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Antibodies

HEXB Antibody

Product Sizes
100 ug
£671.00
3074-MSM2-P1-100UG
About this Product
SKU:
3074-MSM2-P1
Additional Names:
Beta-N-acetylhexosaminidase subunit beta | Cervical cancer proto-oncogene 7 protein | N-acetyl-beta-glucosaminidase subunit beta
Application:
Immunohistochemistry
translate.label.attr.clone:
HEXB/7762
Clonality:
Monoclonal
Extra Details:
Hexosaminidase B (HEXB); also designated β-hexosaminidase B; is a hexosaminidase B (HEXB); also designated β-hexosaminidase B; is a tetramer of two β-A and two β-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 β 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.
Host:
Mouse
Immunogen:
Recombinant fragment human HEXB protein (exact sequence is proprietary)
Isotype:
IgG2
Molecular Weight:
63kDa
Research Area:
Cardiovascular; Immunology; Infectious Disease
Shipping Conditions:
Blue Ice
Storage Conditions:
Antibody with azide - store at 2 to 8°C. Antibody without azide - store at -20 to -80°C.Antibody is stable for 24 months. Non-hazardous. No MSDS required.
Supplier:
NeoBiotechnologies
Type:
Antibodies: Monoclonal Antibody