Human alpha -L-Iduronidase/IDUA Alexa Fluor® 647-conjugated Antibody

Catalog #: FAB4119R Datasheet
Catalog # Availability Size / Price Qty
FAB4119R-100UG
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Human alpha -L-Iduronidase/IDUA Alexa Fluor® 647-conjugated Antibody Summary

Species Reactivity
Human
Specificity
Detects human alpha ‑L‑Iduronidase/IDUA in direct ELISAs and Western blots.
Source
Monoclonal Mouse IgG1 Clone # 452619
Purification
Protein A or G purified
Immunogen
Mouse myeloma cell line NS0-derived recombinant human α‑L‑Iduronidase/IDUA
Ala26-Pro653 (Ala26Thr)
Accession # P35475.2
Formulation
Supplied 0.2mg/ml in 1X PBS with RDF1 and 0.09% Sodium Azide
Label
Alexa Fluor 647 (Excitation= 650 nm, Emission= 668 nm)

Applications

Recommended Concentration
Sample
Western Blot
Optimal dilution of this antibody should be experimentally determined.
 

Please Note: Optimal dilutions should be determined by each laboratory for each application. General Protocols are available in the Technical Information section on our website.

Preparation and Storage

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.

Entrez Gene IDs
3425 (Human); 15932 (Mouse); 360904 (Rat); 102116889 (Cynomolgus Monkey)
Alternate Names
alphaLIduronidase; alpha-L-Iduronidase; IDA; IDUA; MPS1; MPSI

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