1-832-868-1888
order@swbio.com

Catalog Number: |
30947 |
| other_names: | GNS; G6S; N-acetylglucosamine-6-sulfatase |
Amount: |
100μg |
| calculated_mw: | 62kDa |
| host_species: | Rabbit |
Price: |
$319 |
Swiss-Prot No: |
Swiss-Prot#:P15586 |
Form of Antibody: |
Avoid freeze / thaw cycles.|Buffer: PBS with 50% glycerol, pH7.4. |
Storage/Stability: |
|
Immunogen: |
Recombinant fusion protein of human GNS (NP_002067.1). |
Purification: |
Affinity purification |
Specificity/Sensitivity: |
|
Applications: |
WB |
Background: |
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. |
References: |
|
appl_detail: |
WB 1:500 - 1:2000 |
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