Key Product Details

Species Reactivity

Validated:

Human

Cited:

Human

Applications

Validated:

Western Blot

Cited:

ELISA Detection

Label

Biotin

Antibody Source

Polyclonal Sheep IgG
View all alpha-L-Iduronidase/IDUA Primary Antibodies »

Product Specifications

Immunogen

Mouse myeloma cell line NS0-derived recombinant human alpha ‑L‑Iduronidase/IDUA
Ala26-Pro653 (Ala26Thr)
Accession # AAA81589

Specificity

Detects human alpha ‑L‑Iduronidase/IDUA in Western blots.

Clonality

Polyclonal

Host

Sheep

Isotype

IgG

Applications for Human alpha-L-Iduronidase/IDUA Biotinylated Antibody

Application
Recommended Usage

Western Blot

0.1 µg/mL
Sample: Recombinant Human alpha -L-Iduronidase/IDUA (Catalog # 4119-GH)

Formulation, Preparation, and Storage

Purification

Antigen Affinity-purified

Reconstitution

Reconstitute at 0.2 mg/mL in sterile PBS.

Formulation

Lyophilized from a 0.2 μm filtered solution in PBS with BSA as a carrier protein.

Shipping

The product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature recommended below.

Stability & Storage

Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
  • 12 months from date of receipt, -20 to -70 °C as supplied.
  • 1 month, 2 to 8 °C under sterile conditions after reconstitution.
  • 6 months, -20 to -70 °C under sterile conditions after reconstitution.

Calculators

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

alpha -L-Iduronidase encoded by the IDUA gene is an important enzyme required for the lysosomal degradation of glycosaminoglycans (GAGs). It hydrolyzes the non‑reducing terminal alpha -L-iduronic acid residues in GAGs including dermatan sulfate and heparan sulfate. Mutations in IDUA that result in enzymatic deficiency lead to the autosomal recessive disease mucopolysaccharidosis type I (MPS I) (1). MPS I causes progressive cellular, tissue and organ damage, and several clinical studies using enzyme replacement therapy have shown promising benefits (2).

References

  1. Scott, H.S. et al. (1995) Hum. Mutat. 6:288.
  2. Wraith, J.E. (2005) Expert Opin. Pharmacother. 6:489.

Alternate Names

alphaLIduronidase, IDA, IDUA

Entrez Gene IDs

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

Gene Symbol

IDUA

UniProt

AAA81589

Additional alpha-L-Iduronidase/IDUA Products

Product Documents for Human alpha-L-Iduronidase/IDUA Biotinylated Antibody

Certificate of Analysis

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Note: Certificate of Analysis not available for kit components.

Download SDS

Product Specific Notices for Human alpha-L-Iduronidase/IDUA Biotinylated Antibody

For research use only

Citations for Human alpha-L-Iduronidase/IDUA Biotinylated Antibody

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