Human alpha -L-Iduronidase/IDUA Antibody

Catalog # Availability Size / Price Qty
AF4119
AF4119-SP
Detection of Human  alpha ‑L‑Iduronidase/IDUA by Western Blot.
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Product Details
Citations (4)
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Human alpha -L-Iduronidase/IDUA Antibody Summary

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 either lyophilized or as a 0.2 µm filtered solution in PBS.
Label
Unconjugated

Applications

Recommended Concentration
Sample
Western Blot
1 µg/mL
See below
Immunoprecipitation
25 µg/mL
Conditioned cell culture medium spiked with Recombinant Human alpha -L-Iduronidase/IDUA (Catalog # 4119-GH), see our available Western blot detection antibodies
Immunocytochemistry
5-15 µg/mL
See below

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.

Scientific Data

Western Blot Detection of Human a-L-Iduronidase/IDUA antibody by Western Blot. View Larger

Detection of Human alpha ‑L‑Iduronidase/IDUA by Western Blot. Western blot shows lysates of human kidney tissue and IMR-90 human lung fibroblast cell line. PVDF membrane was probed with 1 µg/mL of Sheep Anti-Human a-L-Iduronidase/IDUA Antigen Affinity-purified Polyclonal Antibody (Catalog # AF4119) followed by HRP-conjugated Anti-Sheep IgG Secondary Antibody (Catalog # HAF016). Specific bands were detected for a-L-Iduronidase/IDUA at approximately 74 kDa (as indicated). This experiment was conducted under reducing conditions and using Immunoblot Buffer Group 1.

Immunocytochemistry a-L-Iduronidase/IDUA antibody in HepG2 Human Cell Line by Immunocytochemistry (ICC). View Larger

alpha ‑L‑Iduronidase/IDUA in HepG2 Human Cell Line. a-L-Iduronidase/IDUA was detected in immersion fixed HepG2 human hepatocellular carcinoma cell line using Sheep Anti-Human a-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.
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Shipping
Lyophilized product is shipped at ambient temperature. Liquid small pack size (-SP) is shipped with polar packs. Upon receipt, store 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
  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); 102116889 (Cynomolgus Monkey)
Alternate Names
alphaLIduronidase; alpha-L-Iduronidase; IDA; IDUA; MPS1; MPSI

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Citations for Human alpha -L-Iduronidase/IDUA Antibody

R&D Systems personnel manually curate a database that contains references using R&D Systems products. The data collected includes not only links to publications in PubMed, but also provides information about sample types, species, and experimental conditions.

4 Citations: Showing 1 - 4
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  1. STEP� vectors for rapid generation of stable transfected CHO cell pools and clones with high expression levels and product quality homogeneity of difficult-to-express proteins
    Authors: A Luthra, RA Spanjaard, S Cheema, N Veith, L Kober, Y Wang, T Jing, Y Zhao, F Hoeksema, C Yallop, M Havenga, WAM Bakker
    Protein Expression and Purification, 2021-05-25;0(0):105920.
    Species: Human
    Sample Types: Cell Culture Supernates
    Applications: ELISA Capture
  2. ZFN-Mediated In�Vivo Genome Editing Corrects Murine Hurler Syndrome
    Authors: L Ou, RC DeKelver, M Rohde, S Tom, R Radeke, SJ St Martin, Y Santiago, S Sproul, MJ Przybilla, BL Koniar, KM Podetz-Ped, K Laoharawee, RD Cooksley, KE Meyer, MC Holmes, RS McIvor, T Wechsler, CB Whitley
    Mol. Ther., 2018-11-01;0(0):.
    Species: Transgenic Mouse
    Sample Types: Tissue Homogenates
    Applications: Western Blot
  3. Toxicology Study of Intra-Cisterna Magna Adeno-Associated Virus 9 Expressing Human Alpha-L-Iduronidase in Rhesus Macaques
    Authors: J Hordeaux, C Hinderer, T Goode, N Katz, EL Buza, P Bell, R Calcedo, LK Richman, JM Wilson
    Mol Ther Methods Clin Dev, 2018-07-14;10(0):79-88.
    Species: Human
    Sample Types: Whole Tissue
    Applications: IHC-P
  4. Mutation in VPS33A affects metabolism of glycosaminoglycans: a new type of mucopolysaccharidosis with severe systemic symptoms
    Authors: Hidehito Kondo
    Hum. Mol. Genet, 2017-01-01;0(0):.
    Species: Human
    Sample Types: Whole Cells
    Applications: ICC

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