Human alpha-L-Iduronidase/IDUA Biotinylated Antibody
R&D Systems, part of Bio-Techne | Catalog # BAF4119
Conjugate
Catalog #
Key Product Details
Species Reactivity
Validated:
Human
Cited:
Human
Applications
Validated:
Western Blot
Cited:
ELISA Detection
Label
Biotin
Antibody Source
Polyclonal Sheep IgG
Product Specifications
Immunogen
Mouse myeloma cell line NS0-derived recombinant human alpha‑L‑Iduronidase/IDUA
Ala26-Pro653 (Ala26Thr)
Accession # AAA81589
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)
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.
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
alphaLIduronidase, IDA, IDUA
Gene Symbol
IDUA
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
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