Human alpha -L-Iduronidase/IDUA Antibody Summary
Accession # AAA81589
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.
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.
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
- 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.
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).
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.
Citations: Showing 1 - 3
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ZFN-Mediated Inï¿½Vivo 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
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.
Sample Types: Whole Tissue
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):.
Sample Types: Whole Cells
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