GNS polyclonal antibody

Product Specifications

Background

The product of this gene is a lysosomal enzyme found in all cells. It is involved in the catabolism of heparin, heparan sulphate, and keratan sulphate. Deficiency of this enzyme results in the accumulation of undegraded substrate and the lysosomal storage disorder mucopolysaccharidosis type IIID (Sanfilippo D syndrome) . Mucopolysaccharidosis type IIID is the least common of the four subtypes of Sanfilippo syndrome.

CAS Number

9007-83-4

Structure Composition

1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Product Name Alternative

GNS; G6S

Swiss Prot

P15586

Reactivity

Human, Mouse, Rat

Immunogen

Recombinant fusion protein of human GNS (NP_002067.1) .

Conjugation

Unconjugated

Applications

Dilution

WB,1:500 - 1:2000

Purity

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .