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

R&D Systems, part of Bio-Techne | Catalog # AVI11613

Fc Chimera
R&D Systems, part of Bio-Techne
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AVI11613-010
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Key Product Details

Source

CHO

Structure / Form

Biotinylated via Avi-tag

Conjugate

Biotin

Applications

Bioactivity, 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
Human IDUA
(Ala26-Pro653)
Accession # P35475.2		 Human IgG1
(Pro100-Lys330)		 Avi-tag
N-terminus C-terminus

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

Ala26

Predicted Molecular Mass

98 kDa, under reducing conditions

SDS-PAGE

104-115 kDa, under reducing conditions

Activity

Measured by its binding ability in a functional ELISA.
Recombinant Human alpha‑L‑Iduronidase/IDUA Fc Chimera Avi-tag binds to Human alpha -L-Iduronidase/IDUA Antibody (Catalog # AF4119) with an ED50 of 2.00-20.0 ng/mL.

Measured by its ability to cleave a fluorogenic substrate, 4-Methylumbelliferyl alpha-L-iduronide.
The specific activity is >4500 pmol/min/μg, as measured under the described conditions.

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

Biotinylated Recombinant Human alpha‑L‑Iduronidase/ IDUA Fc Chimera Avi-tag Protein Binding Activity.

Measured by its binding ability in a functional ELISA. Recombinant Human alpha-L-Iduronidase/IDUA Fc Chimera Avi-tag Protein (Catalog # AVI11613) binds to Human alpha -L-Iduronidase/ IDUA Antibody (AF4119) with an ED50 of 2.00-20.0 ng/mL.

Biotinylated Recombinant Human alpha‑L‑Iduronidase/IDUA Fc Chimera Avi-tag Protein SDS-PAGE.

2 μg/lane of Biotinylated Recombinant Human alpha‑L‑Iduronidase/IDUA Fc Chimera Avi-tag Protein (Catalog # AVI11613) was resolved with SDS-PAGE under reducing (R) and non-reducing (NR) conditions and visualized by Coomassie® Blue staining, showing bands at 104-115 kDa and 210-230 kDa, respectively.

Formulation, Preparation and Storage

AVI11613
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

a-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). 

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.

Alternate Names

alphaLIduronidase, IDA, IDUA

Entrez Gene IDs

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

Gene Symbol

IDUA

Additional alpha-L-Iduronidase/IDUA Products

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

Product Datasheets

Certificate of Analysis

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Note: Certificate of Analysis not available for kit components.

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

For research use only

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