Thermo Fisher Scientific GNS Recombinant Rabbit Monoclonal Antibody (007)

Applications

Tested Dilution

Publications

Western Blot (WB)

1:500-1:2,000

-

ELISA (ELISA)

1:5,000-1:10,000

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Rabbit / IgG

Expression System

HEK293 cells

Class

Recombinant Monoclonal

Type

Antibody

Clone

007

Immunogen

Recombinant Human GNS protein

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Purification

Protein A

Storage buffer

PBS

Contains

no preservative

Storage conditions

-20° C, Avoid Freeze/Thaw Cycles

Shipping conditions

Ambient (domestic); Wet ice (international)

RRID

AB_2898804

Target Information

GNS 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.

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