Description
GNS Antibody / Glucosamine (N-acetyl)-6-sulfatase | RQ4270 | Gentaur US, UK & Europe Disrtribition
Family: Primary antibody
Formulation: 0.5mg/ml if reconstituted with 0.2ml sterile DI water
Format: Antigen affinity purified
Clone: N/A
Host Animal: Rabbit
Clonality: Polyclonal (rabbit origin)
Species Reactivity: Human, Mouse, Rat
Application: WB, ELISA
Buffer: Lyophilized from 1X PBS with 2% Trehalose and 0.025% sodium azide
Limitation: This GNS antibody is available for research use only.
Purity: Antigen affinity purified
Description: 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.
Immunogen: A recombinant human protein corresponding to amino acids W238-R355 was used as the immunogen for the GNS antibody.
Storage: After reconstitution, the GNS antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20 °C. Avoid repeated freezing and thawing.
