Recombinant Mouse alpha-L-Iduronidase/IDUA Protein, CF
R&D Systems, part of Bio-Techne | Catalog # 9348-GH
Key Product Details
Product Specifications
Source
Mouse myeloma cell line, NS0-derived mouse alpha-L-Iduronidase/IDUA protein
Glu17-Ser634, with a C-terminal 6-His tag
Glu17-Ser634, with a C-terminal 6-His tag
Purity
>95%, by SDS-PAGE visualized with Silver Staining and quantitative densitometry by Coomassie® Blue Staining.
Endotoxin Level
<1.0 EU per 1 μg of the protein by the LAL method.
N-terminal Sequence Analysis
Glu17
Predicted Molecular Mass
70 kDa
SDS-PAGE
83-95 kDa, reducing conditions
Activity
Measured by its ability to cleave a fluorogenic substrate, 4-Methylumbelliferyl alpha-L-iduronide.
The specific activity is >7,500 pmol/min/ug, as measured under the described conditions.
The specific activity is >7,500 pmol/min/ug, as measured under the described conditions.
Formulation, Preparation and Storage
9348-GH
| Formulation | Supplied as a 0.2 μm filtered solution in Sodium Acetate, 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: alpha-L-Iduronidase/IDUA
non-reducing terminal alpha-L-iduronic acid residues in GAGS including dermatan sulfate and heparan sulfate. Mature mouse IDUA shares 80% aa identity with human IDUA. Mutations in IDUA that result in enzymatic deficiency lead to the autosomal recessive disease mucopolysaccharidosis type I (MPS I) (1). MPS I can be classified as three clinical subtypes; Hurler syndrome, Hurler-Scheie syndrome, and Scheie syndrome with decreasing severity, respectively. MPS I causes progressive cellular, tissue and organ damage, and several clinical studies using enzyme replacement therapy show positive results (2, 3). Recently, the IDUA gene has been linked to osteoporosis (4, 5).
References
- Scott, H.S. et al. (1995) Hum. Mutat. 6:288.
- Wraith, J.E. (2005) Expert Opin. Pharmacother. 6:489.
- Jameson, E. (2016) Cochrane Database Syst. Rev. 4: CD009354.
- Kodric, K. et al. (2016) Wien Klin Wochenschr. 128:480.
- Niu, T. et al. (2016) J. Bone Miner. Res. 31:358.
Alternate Names
alphaLIduronidase, IDA, IDUA
Gene Symbol
IDUA
UniProt
Additional alpha-L-Iduronidase/IDUA Products
Product Documents for Recombinant Mouse alpha-L-Iduronidase/IDUA Protein, CF
Product Specific Notices for Recombinant Mouse alpha-L-Iduronidase/IDUA Protein, CF
For research use only
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