Human alpha-L-Iduronidase/IDUA Biotinylated Antibody
R&D Systems, part of Bio-Techne | Catalog # BAF4119
Key Product Details
Species Reactivity
Validated:
Cited:
Applications
Validated:
Cited:
Label
Antibody Source
Product Specifications
Immunogen
Ala26-Pro653 (Ala26Thr)
Accession # AAA81589
Specificity
Clonality
Host
Isotype
Applications for Human alpha-L-Iduronidase/IDUA Biotinylated Antibody
Western Blot
Sample: Recombinant Human alpha-L-Iduronidase/IDUA (Catalog # 4119-GH)
Formulation, Preparation, and Storage
Purification
Reconstitution
Formulation
Shipping
Stability & Storage
- 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.
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
- Scott, H.S. et al. (1995) Hum. Mutat. 6:288.
- Wraith, J.E. (2005) Expert Opin. Pharmacother. 6:489.
Alternate Names
Gene Symbol
UniProt
Additional alpha-L-Iduronidase/IDUA Products
Product Documents for Human alpha-L-Iduronidase/IDUA Biotinylated Antibody
Product Specific Notices for Human alpha-L-Iduronidase/IDUA Biotinylated Antibody
For research use only