Thermo Fisher Scientific HEXB Polyclonal Antibody

Applications

Tested Dilution

Publications

Western Blot (WB)

0.1-0.5 µg/mL

-

Immunohistochemistry (Paraffin) (IHC (P))

0.5-1 µg/mL

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Rabbit / IgG

Class

Polyclonal

Type

Antibody

Immunogen

E.coli-derived human HEXB recombinant protein (Position: K381-M556). if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:HEXB,uniProtId:P07686-1,ncbiNodeId:9606,antigenRange:381-556,antigenLength:556,antigenImageFileName:PA5-79359_HEXB_P07686-1_Rabbit.svg,antigenImageFileNamePDP:PA5-79359_HEXB_P07686-1_Rabbit_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

View immunogen .st0{fill:#FFFFFF;} .st1{fill:#1E8AE7;}

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Lyophilized

Concentration

500 µg/mL

Purification

Antigen affinity chromatography

Storage buffer

PBS with 5mg BSA

Contains

0.05mg sodium azide

Storage conditions

-20°C

Shipping conditions

Ambient (domestic); Wet ice (international)

RRID

AB_2746475

Product Specific Information

Reconstitute with 0.2 mL of distilled water to yield a concentration of 500 µg/mL.

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.