l General Information |
Product Name | α-L-Iduronidase human |
General deion | α-L-Iduronidase is an enzyme encoded by IDUA gene; participate in lysosomal degradation of glycosaminoglycans.In human, mutation of gene encoding α-L-Iduronidase causes lysosomal storage diseases (mucopolysaccharidosis type I). |
Assay | recombinant, expressed in mouse NSO cells; ≥7,500 units/μg protein |
Impurities | ≤1.0 EU/μg Endotoxin |
Suitability | BioReagent |
l Physical and Chemical Information |
Physical form | Supplied as a solution in 40 mM sodium acetate , 400 mM NaCl and 20% (v/v) glycerol, pH 5.0 |
l Biological Information |
Biochem/Physiol Actions | Catalyzes the hydrolysis of unsulfated α-L-iduronosidic ages in dermatan sulfate In lysosomal degradation process α-L-Iduronidase plays a crucial role. It hydrolyzes the non-reducing terminal α-L-iduronic acid residues in GAGs, including dermatan sulfate and heparan sulfate. |
Application | α-L-Iduronidase may be used for leukocyte assay in the study of a-L-Iduronidase deficiency in new born. |
Unit Definition | One unit will produce 1 picomole of 4-methylumbelliferone from 4-methylumbelliferyl-α-L-iduronide per minute at pH 3.5 at 25 °C. |
l Storage |
Storage temp. | -20°C |
l Precautions and Disclaimer |
This product is for R&D use only, not for drug, household, or other uses. |
l References |
1. http://www.drugbank.ca 2. https://ncit.nci.nih.gov 3. https://www.ncbi.nlm.nih.gov |
