Recombinant Human Glucosamine (N-acetyl)-6-Sulfatase, CF
R&D Systems, part of Bio-Techne | Catalog # 2484-SUC
Key Product Details
Product Specifications
Source
Val37-Leu552 & Thr44-Leu552, both with a C-terminal 10-His tag
Purity
Endotoxin Level
N-terminal Sequence Analysis
Predicted Molecular Mass
SDS-PAGE
Activity
The specific activity is >250 pmol/min/μg, as measured under the described conditions.
Reviewed Applications
Read 1 review rated 5 using 2484-SUC in the following applications:
Formulation, Preparation and Storage
2484-SUC
Formulation | Supplied as a 0.2 μm filtered solution in Tris, NaCl and Glycerol. |
Shipping | The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below. |
Stability & Storage | Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
|
Background: Glucosamine (N-acetyl)-6-Sulfatase/GNS
A member of the sulfatase family, GNS is required for the lysosomal degradation of the glycosaminoglycans (GAG) heparan sulfate and keratan sulfate (1, 2). It hydrolyzes the 6-sulfate group of the N-acetyl-D-glucosamine 6-sulfate units of the GAG. GNS deficiency results in mucopolysaccharidosis type IIID (MPS IIID or Sanfilippo D Syndrome), an inborn error leading to lysosomal accumulation of heparan sulfate. MPS IIID has profound mental deterioration, hyperactivity, and relatively mild somatic manifestations. The deduced amino acid sequence of human GNS consists of a signal peptide (residues 1-36) and a mature chain (residues 37-552) that may be further processed into N-terminal and C-terminal fragments (3). rhGNS corresponds to the single chain and has sulfatase activity described in Activity Assay Protocol.
References
- Parenti, G. et al. (1997) Curr. Opin. Genet. & Dev. 7:386.
- Neufeld, E.F. and J. Muenzer (2001) in The Metabolic and Molecular Basis of Inherited Disease, Scriver, C.R. et al. (eds.) pp. 3421, New York, McGraw-Hill.
- Robertson, D.A. et al. (1992) Biochem. J. 288:539.
Alternate Names
Entrez Gene IDs
Gene Symbol
UniProt
Additional Glucosamine (N-acetyl)-6-Sulfatase/GNS Products
Product Documents for Recombinant Human Glucosamine (N-acetyl)-6-Sulfatase, CF
Product Specific Notices for Recombinant Human Glucosamine (N-acetyl)-6-Sulfatase, CF
For research use only