alpha-L-Iduronidase/IDUA Antibody (452619) [Unconjugated]

Images

 
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 alpha ‑L‑Iduronidase/IDUA Monoclonal Antibody ...read more

Product Details

Summary
Reactivity HuSpecies Glossary
Applications WB
Clone
452619
Clonality
Monoclonal
Host
Mouse
Conjugate
Unconjugated
Concentration
LYOPH

Order Details

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Catalog# & Formulation Size Price

alpha-L-Iduronidase/IDUA Antibody (452619) [Unconjugated] Summary

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.

Applications/Dilutions

Dilutions
  • Western Blot 2 ug/mL
Reviewed Applications
Read 1 Review rated 4
using
MAB4119 in the following application:

Packaging, Storage & Formulations

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.
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.

Notes

This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.

Alternate Names for alpha-L-Iduronidase/IDUA Antibody (452619) [Unconjugated]

  • alphaLIduronidase
  • alpha-L-Iduronidase
  • IDA
  • IDUA
  • MPS1
  • MPSI

Background

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.

  1. Scott, H.S. et al. (1995) Hum. Mutat. 6:288.
  2. Wraith, J.E. (2005) Expert Opin. Pharmacother. 6:489.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

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Publications for alpha-L-Iduronidase/IDUA Antibody (MAB4119) (0)

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Review for alpha-L-Iduronidase/IDUA Antibody (MAB4119) (1) 41

Average Rating: 4
(Based on 1 review)
We have 1 review tested in 1 species: Human.

Reviews using MAB4119:
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WB
(1)
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(1)
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Images Ratings Applications Species Date Details
Western Blot alpha-L-Iduronidase/IDUA MAB4119
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4
reviewed by:
NICOLE GREPO
WB Human 07/07/2020
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Summary

ApplicationWestern Blot
Sample Tested293T cell lysate
SpeciesHuman
LotCAAK041910A

Comments

CommentsAntibody worked as expected at manufacturer recommended concentration. Would order again.

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Recent Reviews

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5
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4
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1
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NICOLE GREPO
07/07/2020
Application: WB
Species: Human

Bioinformatics

Gene Symbol IDUA
Uniprot