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.
Human alpha-L-Iduronidase/IDUA Antibody (452619)
R&D Systems | Catalog # MAB4119
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Key Product Details
Species Reactivity
Validated:
Cited:
Applications
Validated:
Cited:
Label
Antibody Source
Product Specifications
Immunogen
Ala26-Pro653 (Ala26Thr)
Accession # P35475.2
Specificity
Clonality
Host
Isotype
Scientific Data Images for Human alpha-L-Iduronidase/IDUA Antibody (452619)
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.Applications for Human alpha-L-Iduronidase/IDUA Antibody (452619)
Western Blot
Sample: Human kidney tissue and IMR‑90 human lung fibroblast cell line
Reviewed Applications
Read 2 reviews rated 4 using MAB4119 in the following applications:
Formulation, Preparation, and Storage
Purification
Reconstitution
Reconstitute at 0.5 mg/mL in sterile PBS. For liquid material, refer to CoA for concentration.
Formulation
Shipping
Stability & 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.
Calculators
Background: alpha-L-Iduronidase/IDUA
References
- Scott, H.S. et al. (1995) Hum. Mutat. 6:288.
- Wraith, J.E. (2005) Expert Opin. Pharmacother. 6:489.
Alternate Names
Gene Symbol
UniProt
Additional alpha-L-Iduronidase/IDUA Products
Product Documents for Human alpha-L-Iduronidase/IDUA Antibody (452619)
Certificate of Analysis
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Note: Certificate of Analysis not available for kit components.
Product Specific Notices for Human alpha-L-Iduronidase/IDUA Antibody (452619)
For research use only
Related Research Areas
Citations for Human alpha-L-Iduronidase/IDUA Antibody (452619)
Customer Reviews for Human alpha-L-Iduronidase/IDUA Antibody (452619) (2)
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Customer Images
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Application: Western BlotSample Tested: 293T cell lysateSpecies: HumanVerified Customer | Posted 07/07/2020Western blot of HEK wild type versus HEK over expressing IDUA.Antibody worked as expected at manufacturer recommended concentration. Would order again.
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Application: ELISASample Tested: Monoclonal Mouse IgG1 CloneSpecies: MouseVerified Customer | Posted 09/01/2017We used this antibody for an ELISA in order to detect human Iduronidase as a positive control for our tests. It worked perfectly at a working concentration of 2ug/mL.
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Protocols
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- Troubleshooting Guide: Western Blot Figures
- Western Blot Conditions
- Western Blot Protocol
- Western Blot Protocol for Cell Lysates
- Western Blot Troubleshooting
- Western Blot Troubleshooting Guide
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