alpha-L-Iduronidase/IDUA Antibody (452619) [Alexa Fluor® 594]

• Applications: WB
• Clone: 452619
• Clonality: Monoclonal
• Host: Mouse
• Conjugate: Alexa Fluor 594

alpha-L-Iduronidase/IDUA Antibody (452619) [Alexa Fluor® 594] Summary

• Immunogen: Mouse myeloma cell line NS0-derived recombinant human α‑L‑Iduronidase/IDUA
  Ala26-Pro653 (Ala26Thr)
  Accession # P35475.2
• Specificity: Detects human alpha ‑L‑Iduronidase/IDUA in direct ELISAs and Western blots.
• Isotype: IgG1
• Clonality: Monoclonal
• Host: Mouse
• Purity Statement: Protein A or G purified

Packaging, Storage & Formulations

• Storage: Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied
• Buffer: Supplied 0.2mg/ml in 1X PBS with RDF1 and 0.09% Sodium Azide

Notes

This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.

Background

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). The sequence of human IDUA shares 79% amino acid identity with mouse IDUA.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.