Thermo Fisher Scientific HEXB Polyclonal Antibody

Applications

Tested Dilution

Publications

Western Blot (WB)

1:500-1:1,000

-

Immunohistochemistry (Paraffin) (IHC (P))

1:50-1:100

-

Product Specifications

Host/Isotype

Rabbit / IgG

Class

Polyclonal

Type

Antibody

Immunogen

A synthetic peptide derived from the C-terminal region of human HEXB if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:HEXB,uniProtId:P07686-1,ncbiNodeId:9606,antigenRange:556,antigenLength:556,antigenImageFileName:PA5-38571_HEXB_P07686-1_Rabbit.svg,antigenImageFileNamePDP:PA5-38571_HEXB_P07686-1_Rabbit_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

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

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

1 mg/mL

Storage conditions

-20°C

Shipping conditions

Wet ice

RRID

AB_2555170

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.