Human alpha -L-Iduronidase/IDUA Antibody

  
  • Species Reactivity
    Human
  • Specificity
    Detects human alpha ‑L‑Iduronidase/IDUA in direct ELISAs and Western blots.
  • Source
    Polyclonal Sheep IgG
  • Purification
    Antigen Affinity-purified
  • Immunogen
    Mouse myeloma cell line NS0-derived recombinant human alpha ‑L‑Iduronidase/IDUA
    Ala26-Pro653 (Ala26Thr)
    Accession # AAA81589
  • Formulation
    Lyophilized from a 0.2 μm filtered solution in PBS with Trehalose. *Small pack size (SP) is supplied as a 0.2 µm filtered solution in PBS.
  • Label
    Unconjugated
Applications
  •  
    Recommended
    Concentration
    Sample
Please Note: Optimal dilutions should be determined by each laboratory for each application. General Protocols are available in the Technical Information section on our website.
Data Examples
Immunocytochemistry
alpha ‑L‑Iduronidase/IDUA in HepG2 Human Cell Line.

alpha ‑L‑Iduronidase/IDUA was detected in immersion fixed HepG2 human hepatocellular carcinoma cell line using Sheep Anti-Human alpha ‑L‑Iduronidase/IDUA Antigen Affinity-purified Polyclonal Antibody (Catalog # AF4119) at 15 µg/mL for 3 hours at room temperature. Cells were stained using the NorthernLights™ 557-conjugated Anti-Sheep IgG Secondary Antibody (red; Catalog # NL010) and counterstained with DAPI (blue). Specific staining was localized to cytoplasm. View our protocol for Fluorescent ICC Staining of Cells on Coverslips.

Preparation and Storage
  • Reconstitution
    Reconstitute at 0.2 mg/mL in sterile PBS.
  • Shipping
    The product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature recommended below. *Small pack size (SP) is shipped with polar packs. Upon receipt, store it immediately at -20 to -70 °C
  • 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:
    1. Scott, H.S. et al. (1995) Hum. Mutat. 6:288.
    2. Wraith, J.E. (2005) Expert Opin. Pharmacother. 6:489.
  • Entrez Gene IDs:
    3425 (Human); 15932 (Mouse); 360904 (Rat)
  • Alternate Names:
    alphaLIduronidase; alpha-L-Iduronidase; IDA; IDUA
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