Human alpha -L-Iduronidase/IDUA Antibody Summary
Ala26-Pro653 (Ala26Thr)
Accession # P35475.2
Applications
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
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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 2 µg/mL of Mouse Anti-Human a-L-Iduronidase/IDUA Monoclonal Antibody (Catalog # MAB4119) followed by HRP-conjugated Anti-Mouse IgG Secondary Antibody (Catalog # HAF018). A specific band was 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.
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.
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). The sequence of human IDUA shares 79% amino acid identity with mouse IDUA.
Product Datasheets
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