| Reactivity | HuSpecies Glossary |
| Applications | WB |
| Clone | 452619 |
| Clonality | Monoclonal |
| Host | Mouse |
| Conjugate | Unconjugated |
| Concentration | LYOPH |
| Immunogen | Mouse myeloma cell line NS0-derived recombinant human alpha ‑L‑Iduronidase/IDUA Ala26-Pro653 (Ala26Thr) Accession # P35475.2 |
| Specificity | Detects human alpha ‑L‑Iduronidase/IDUA in direct ELISAs and Western blots. |
| Source | N/A |
| Isotype | IgG1 |
| Clonality | Monoclonal |
| Host | Mouse |
| Gene | IDUA |
| Purity Statement | Protein A or G purified from hybridoma culture supernatant |
| Innovator's Reward | Test in a species/application not listed above to receive a full credit towards a future purchase. |
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| Storage | Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
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| Buffer | 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. |
| Preservative | No Preservative |
| Concentration | LYOPH |
| Reconstitution Instructions | Reconstitute at 0.5 mg/mL in sterile PBS. |
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.
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| Images | Ratings | Applications | Species | Date | Details | ||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Enlarge |
reviewed by:
Verified Customer |
WB | Human | 07/07/2020 |
Summary
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Secondary Antibodies |
Isotype Controls |
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