Recombinant Human alpha-L-Iduronidase/IDUA His Protein, CF

R&D Systems, part of Bio-Techne | Catalog # 11201-GH

CHO-expressed (aa 28-653 H33Q)
R&D Systems, part of Bio-Techne
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11201-GH-020
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Key Product Details

Source

CHO

Accession #

P35475.2

Conjugate

Unconjugated

Applications

Enzyme Activity

View all alpha-L-Iduronidase/IDUA Proteins and Enzymes »

Product Specifications

Source

Chinese Hamster Ovary cell line, CHO-derived human alpha-L-Iduronidase/IDUA protein
Ala28-Pro653 (His33Gln), with a C-terminal 10-His tag

Purity

>95%, by SDS-PAGE visualized with Silver Staining and quantitative densitometry by Coomassie® Blue Staining.

Endotoxin Level

<0.10 EU per 1 μg of the protein by the LAL method.

N-terminal Sequence Analysis

Ala28

Predicted Molecular Mass

71 kDa

SDS-PAGE

80-91 kDa, under reducing conditions.

Activity

Measured by its ability to cleave a fluorogenic substrate, 4-Methylumbelliferyl alpha-L-iduronide.
The specific activity is >6000 pmol/min/μg, as measured under the described conditions.

Scientific Data Images for Recombinant Human alpha-L-Iduronidase/IDUA His Protein, CF

Recombinant Human alpha-L-Iduronidase/IDUA His-tag Protein SDS-PAGE.

2 μg/lane of Recombinant Human alpha-L-Iduronidase/IDUA His-tag Protein (Catalog # 11201-GH) was resolved with SDS-PAGE under reducing (R) and non-reducing (NR) conditions and visualized by Coomassie® Blue staining, showing bands at 80-91 kDa.

Formulation, Preparation and Storage

11201-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.
  • 6 months from date of receipt, -20 to -70 °C as supplied.
  • 3 months, -20 to -70 °C under sterile conditions after opening.

Background: alpha-L-Iduronidase/IDUA

alpha-L-Iduronidase is a member of the glycoside hydrolase family encoded by the IDUA gene (1). It is an important enzyme required for the lysosomal degradation of glycosaminoglycans (GAGS) and hydrolyzes the non-reducing terminal a-L-iduronic acid residues in GAGS including dermatan sulfate and heparan sulfate.  Human IDUA is a 653 aa protein composed of a signal peptide removed in the lysosome for mature form and three domains: a triosephosphate isomerase barrel fold containing the catalytic site, a B-sandwich domain, and an Ig(Ig)-like domain. The protein has six reported N-glycosylation sites and the glycosylation status of the enzyme correlates with its catalytic activity (1). More than a hundred disease-associated variants in the IDUA gene have been identified (1-2). Mutations in IDUA that result in enzymatic deficiency lead to the autosomal recessive disease mucopolysaccharidosis type I (MPS I) (3). MPS I can be classified into 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 (4,5). More recently, the IDUA gene has been linked to osteoporosis (6,7). H33Q, present in the recombinant product, represents a benign and common polymorphism (8).

References

  1. Maita, N. et al. (2013) Proc. Natl. Acad. Sci. 110:14628.
  2. Borges, P. et al. (2021) Front. Mol. Biosci. 8:752797.
  3. Scott, H.S. et al. (1995) Hum. Mutat. 6:288.
  4. Wraith, J.E. (2005) Expert Opin. Pharmacother. 6:489.
  5. Jameson, E. (2016) Cochrane Database Syst. Rev. 4:CD009354.
  6. Kodric, K. et al. (2016) Wien Klin Wochenschr. 128:480.
  7. Niu, T. et al. (2016) J. Bone Miner. Res. 31:358.
  8. Scott, H.S. et al. (1993) Hum. Mol. Genet. 2:1471.

Alternate Names

alphaLIduronidase, IDA, IDUA

Entrez Gene IDs

3425 (Human); 15932 (Mouse); 360904 (Rat); 102116889 (Cynomolgus Monkey)

Gene Symbol

IDUA

UniProt

P35475.2

Additional alpha-L-Iduronidase/IDUA Products

Product Documents for Recombinant Human alpha-L-Iduronidase/IDUA His Protein, CF

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Product Specific Notices for Recombinant Human alpha-L-Iduronidase/IDUA His Protein, CF

For research use only

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