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 (3)
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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 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.

Data Examples

Western Blot Detection  of Human  alpha ‑L‑Iduronidase/IDUA 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 alpha ‑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 alpha ‑L‑Iduronidase/IDUA at approximately 74 kDa (as indicated). This experiment was conducted under reducing conditions and using Immunoblot Buffer Group 1.

Immunocytochemistry alpha ‑L‑Iduronidase/IDUA in HepG2 Human Cell Line. View Larger

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.

Reconstitution Calculator

Reconstitution Calculator

The reconstitution calculator allows you to quickly calculate the volume of a reagent to reconstitute your vial. Simply enter the mass of reagent and the target concentration and the calculator will determine the rest.

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Preparation and Storage

Reconstitution
Reconstitute at 0.2 mg/mL in sterile PBS.
Reconstitution Buffer Available
Reconstitution Buffer 1 (PBS)
Catalog #
Availability
Size / Price
Qty
RB01
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

Product Datasheets

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.

3 Citations: Showing 1 - 3
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  1. 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;0(0):.
    Species: Transgenic Mouse
    Sample Types: Tissue Homogenates
    Applications: Western Blot
  2. 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;10(0):79-88.
    Species: Human
    Sample Types: Whole Tissue
    Applications: IHC-P
  3. Mutation in VPS33A affects metabolism of glycosaminoglycans: a new type of mucopolysaccharidosis with severe systemic symptoms
    Authors: Hidehito Kondo
    Hum. Mol. Genet, 2016;0(0):.
    Species: Human
    Sample Types: Whole Cells
    Applications: ICC

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Reconstitution Buffers

Secondary Antibodies

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