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GNS Antibody / Glucosamine (N-acetyl) -6-sulfatase

Selected Variant

RQ4270

Size: 100 µg

N-acetylglucosamine-6-sulfatase, also known as glucosamine (N-acetyl) -6-sulfatase, is an enzyme that in humans is encoded by the GNS gene. 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.

Product Specifications

CAS Number

9007-83-4

Specifications

Western blot: 0.5-1 µg/mL, Direct ELISA: 0.1-0.5 µg/mL

UniProt

P15586

Host

Rabbit

Reactivity

Human, Mouse, Rat

Immunogen

A recombinant human protein corresponding to amino acids W238-R355 was used as the immunogen for the GNS antibody.

Clonality

Polyclonal

Isotype

IgG

Applications

WB, ELISA

Purity

Antigen affinity purified

Format

Antigen affinity purified

Buffer

Lyophilized from 1X PBS with 2% Trehalose and 0.025% sodium azide

Reconstitution

After reconstitution, the GNS antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.

Limitations

This GNS antibody is available for research use only.

Storage Conditions

After reconstitution, the GNS antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.

Formulation

0.5 mg/mL if reconstituted with 0.2ml sterile DI water

Applications Notes

Optimal dilution of the GNS antibody should be determined by the researcher.

Location

Lysosome

Image Legend

Western blot testing of human 1) HepG2, 2) PANC-1 and 3) SGC-7901 cell lysate with GNS antibody at 0.5ug/ml. Predicted molecular weight ~62 kDa, can be observed at ~94 kDa.

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