alpha-L-Iduronidase/IDUA Antibody [Alexa Fluor™ Plus 647]

• Reactivity: Hu
• Clonality: Polyclonal
• Host: Sheep
• Conjugate: Alexa Fluor Plus 647

alpha-L-Iduronidase/IDUA Antibody [Alexa Fluor™ Plus 647] Summary

• Specificity: Detects human alpha ‑L‑Iduronidase/IDUA in direct ELISAs and Western blots.
• Isotype: IgG
• Clonality: Polyclonal
• Host: Sheep

Packaging, Storage & Formulations

• Storage: Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied
• Buffer: Supplied 0.2 mg/mL in a saline solution containing BSA and Sodium Azide.

Notes

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

Alternate Names for alpha-L-Iduronidase/IDUA Antibody [Alexa Fluor™ Plus 647]

• alphaLIduronidase
• alpha-L-Iduronidase
• IDA
• IDUA
• MPS1
• MPSI

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).

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

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.