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
Limitations
This GNS antibody is available for research use only.
Storage Conditions
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
Price
Documents
Frequently Asked Questions
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