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

Catalog #: AVI11613 Datasheet / COA / SDS
Fc Chimera
Catalog # Availability Size / Price Qty
AVI11613-010
Biotinylated Recombinant Human alpha‑L‑Iduronidase/ IDUA Fc Chimera Avi-tag Protein Binding Activity.
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Summary Product Datasheets Carrier Free Data Images Reconstitution Calculator Background Related Research Areas

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

Product Specifications

Endotoxin Level
<0.10 EU per 1 μg of the protein by the LAL method.
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.
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-terminusC-terminus
N-terminal Sequence
Analysis
Ala26
Structure / Form
Biotinylated via Avi-tag
Predicted Molecular Mass
98 kDa, under reducing conditions
SDS-PAGE
104-115 kDa, under reducing conditions

Product Datasheets

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AVI11613

Product Datasheet
COA
SDS

Carrier Free

What does CF mean?

CF stands for Carrier Free (CF). We typically add Bovine Serum Albumin (BSA) as a carrier protein to our recombinant proteins. Adding a carrier protein enhances protein stability, increases shelf-life, and allows the recombinant protein to be stored at a more dilute concentration. The carrier free version does not contain BSA.

What formulation is right for me?

In general, we advise purchasing the recombinant protein with BSA for use in cell or tissue culture, or as an ELISA standard. In contrast, the carrier free protein is recommended for applications, in which the presence of BSA could interfere.

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.

Scientific Data

Binding Activity View Larger

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.

SDS-PAGE View Larger

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.

Reconstitution Calculator

Reconstitution Calculator

The reconstitution calculator allows you to quickly calculate the volume of a reagent to reconstitute your vial. Simply enter the mass of reagent and the target concentration and the calculator will determine the rest.

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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.
Entrez Gene IDs
3425 (Human); 15932 (Mouse); 360904 (Rat); 102116889 (Cynomolgus Monkey)
Alternate Names
alphaLIduronidase; alpha-L-Iduronidase; IDA; IDUA; MPS1; MPSI

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