Human alpha-L-Iduronidase/IDUA Alexa Fluor® 594-conjugated Antibody
R&D Systems, part of Bio-Techne | Catalog # FAB4119T
Conjugate
Catalog #
Key Product Details
Species Reactivity
Human
Applications
Western Blot
Label
Alexa Fluor 594 (Excitation = 590 nm, Emission = 617 nm)
Antibody Source
Monoclonal Mouse IgG1 Clone # 452619
Product Specifications
Immunogen
Mouse myeloma cell line NS0-derived recombinant human α‑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
Applications
Application
Recommended Usage
Western Blot
Optimal dilution of this antibody should be experimentally determined.
Formulation, Preparation, and Storage
Purification
Protein A or G purified
Formulation
Supplied 0.2mg/ml in 1X PBS with RDF1 and 0.09% Sodium Azide
Shipping
The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.
Stability & Storage
Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied
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.
Alternate Names
alphaLIduronidase, IDA, IDUA
Gene Symbol
IDUA
UniProt
Additional alpha-L-Iduronidase/IDUA Products
Product Specific Notices
This product is provided under an agreement between Life Technologies Corporation and R&D Systems, Inc, and the manufacture, use, sale or import of this product is subject to one or more US patents and corresponding non-US equivalents, owned by Life Technologies Corporation and its affiliates. The purchase of this product conveys to the buyer the non-transferable right to use the purchased amount of the product and components of the product only in research conducted by the buyer (whether the buyer is an academic or for-profit entity). The sale of this product is expressly conditioned on the buyer not using the product or its components (1) in manufacturing; (2) to provide a service, information, or data to an unaffiliated third party for payment; (3) for therapeutic, diagnostic or prophylactic purposes; (4) to resell, sell, or otherwise transfer this product or its components to any third party, or for any other commercial purpose. Life Technologies Corporation will not assert a claim against the buyer of the infringement of the above patents based on the manufacture, use or sale of a commercial product developed in research by the buyer in which this product or its components was employed, provided that neither this product nor any of its components was used in the manufacture of such product. For information on purchasing a license to this product for purposes other than research, contact Life Technologies Corporation, Cell Analysis Business Unit, Business Development, 29851 Willow Creek Road, Eugene, OR 97402, Tel: (541) 465-8300. Fax: (541) 335-0354.
For research use only
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