Thermo Fisher Scientific HEXB Polyclonal Antibody

Applications

Tested Dilution

Publications

Western Blot (WB)

1:500-1:1,000

View 1 publication 1 publication

Immunohistochemistry (Paraffin) (IHC (P))

1:50-1:200

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Product Specifications

Species Reactivity

Human

Published species

Mouse

Host/Isotype

Rabbit / IgG

Class

Polyclonal

Type

Antibody

Immunogen

Synthetic peptide corresponding to amino acids 481-530 of human Hexb if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:HEXB,uniProtId:P07686-1,ncbiNodeId:9606,antigenRange:481-530,antigenLength:556,antigenImageFileName:PA5-36146_HEXB_P07686-1_Rabbit.svg,antigenImageFileNamePDP:PA5-36146_HEXB_P07686-1_Rabbit_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

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Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

1 mg/mL

Purification

Antigen affinity chromatography

Storage buffer

PBS, pH 7.2, with 50% glycerol

Contains

0.02% sodium azide

Storage conditions

Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.

Shipping conditions

Wet ice

RRID

AB_2553391

Product Specific Information

This antibody detects endogenous protein at a molecular weight of 63 kDa.

Purity is >95% by SDS-PAGE.

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.