Thermo Fisher Scientific HEXB Monoclonal Antibody (HEXB/7762)

Applications

Tested Dilution

Publications

Immunohistochemistry (Paraffin) (IHC (P))

1-2 µg/mL

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Mouse / IgG2, kappa

Class

Monoclonal

Type

Antibody

Clone

HEXB/7762

Immunogen

Recombinant fragment human HEXB protein (exact sequence is proprietary)

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

200 µg/mL

Purification

Protein A/G

Storage buffer

PBS with 0.05% BSA

Contains

0.05% sodium azide

Storage conditions

4° C

Shipping conditions

Ambient (domestic); Wet ice (international)

Product Specific Information

Positive Control:Human kidney or lung.

Cellular Location: Lysosome.

Target Information

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.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.