Human alpha-L-Iduronidase/IDUA Antibody
R&D Systems, part of Bio-Techne | Catalog # MAB4119
Key Product Details
Species Reactivity
Human
Applications
Western Blot
Label
Unconjugated
Antibody Source
Monoclonal Mouse IgG1 Clone # 452619
Product Specifications
Immunogen
Mouse myeloma cell line NS0-derived recombinant human alpha‑L‑Iduronidase/IDUA
Ala26-Pro653 (Ala26Thr)
Accession # P35475.2
Ala26-Pro653 (Ala26Thr)
Accession # P35475.2
Specificity
Detects human alpha‑L‑Iduronidase/IDUA in direct ELISAs and Western blots.
Clonality
Monoclonal
Host
Mouse
Isotype
IgG1
Scientific Data Images for Human alpha-L-Iduronidase/IDUA Antibody
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
Application
Recommended Usage
Western Blot
2 µg/mL
Sample: Human kidney tissue and IMR‑90 human lung fibroblast cell line
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
Protein A or G purified from hybridoma culture supernatant
Reconstitution
Reconstitute at 0.5 mg/mL in sterile PBS. For liquid material, refer to CoA for concentration.
Formulation
Lyophilized from a 0.2 μm filtered solution in PBS and NaCl with Trehalose. *Small pack size (SP) is supplied either lyophilized or as a 0.2 µm filtered solution in PBS.
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). The sequence of human IDUA shares 79% amino acid identity with mouse IDUA.
References
- Scott, H.S. et al. (1995) Hum. Mutat. 6:288.
- Wraith, J.E. (2005) Expert Opin. Pharmacother. 6:489.
Alternate Names
alphaLIduronidase, IDA, IDUA
Gene Symbol
IDUA
UniProt
Additional alpha-L-Iduronidase/IDUA Products
Product Documents for Human alpha-L-Iduronidase/IDUA Antibody
Product Specific Notices for Human alpha-L-Iduronidase/IDUA Antibody
For research use only
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