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.
Formulation
0.5mg/ml if reconstituted with 0.2ml sterile DI water
Host
Rabbit
Immunogen Region
A recombinant human protein corresponding to amino acids W238-R355 was used as the immunogen for the GNS antibody.
Isotype
IgG
Predicted Reactivity
Human, Mouse, Rat
Reactivity
Human, Mouse, Rat
Recombinant
No
Subcellular Location
Lysosome
Uniprot
P15586
Buffer
Lyophilized from 1X PBS with 2% Trehalose and 0.025% sodium azide
Format
Antigen affinity purified
Purification
Antigen affinity purified
Storage
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.
Applications
WB, ELISA
Dilution
Western blot: 0.5-1ug/ml, Direct ELISA: 0.1-0.5ug/ml