I6034
α-L-Iduronidase human
recombinant, expressed in mouse NSO cells
Synonym(s):
IDUA
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recombinant
expressed in mouse NSO cells
Quality Level
form
solution
specific activity
≥7,500 units/μg protein
mol wt
83 kDa
impurities
≤1.0 EU/μg Endotoxin
shipped in
wet ice
storage temp.
−20°C
General description
α-L-Iduronidase (IDUA) is mapped to human chromosome 4p16.3. The mature IDUA protein is glycosylated and comprises triosephosphate isomerase (TIM) barrel domain, β-sandwich, helix-loop-helix region and an immunoglobin-like domain. α-L-Iduronidase is classified under glycoside hydrolase (GH) family 39.
Application
α-L-Iduronidase may be used for leukocyte assay in the study of a-L-Iduronidase deficiency in new born.
Biochem/physiol Actions
In lysosomal degradation process α-L-Iduronidase plays a crucial role. It hydrolyzes the non-reducing terminal α-L-iduronic acid residues in glycosaminoglycans (GAGs), including dermatan sulfate and heparan sulfate.
Mutation in the α-L-Iduronidase is implicated in Mucopolysaccharidosis I (MPS I) . This enzyme defect leades to accumulation of dermatan and heparan sulfate . MPS I pathophysiology is accompanied with deformation of the skull, mental retardation and hernias.
Catalyzes the hydrolysis of unsulfated α-L-iduronosidic linkages in dermatan sulfate
Physical properties
Expressed as C-terminal histine-tagged protein (residues 1-653) with a caluclated molecular mass of 71 kDa migrating at ~83 kDa under SDS-PAGE reducing conditions.
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.
Physical form
Supplied as a solution in 40 mM sodium acetate , 400 mM NaCl and 20% (v/v) glycerol, pH 5.0
Storage Class
10 - Combustible liquids
wgk_germany
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
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Mei Dai et al.
Proceedings of the National Academy of Sciences of the United States of America, 111(7), 2680-2685 (2014-02-20)
Use of megakaryocytes/platelets for transgene expression may take advantage of their rapid turnover and protective storage in platelets and reduce the risk of activating oncogenes in hematopoietic stem and progenitor cells (HSCs). Here, we show that human megakaryocytic cells could
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European journal of pediatrics, 171(6), 911-919 (2012-01-12)
Our objective was to assess how the diagnosis and treatment of mucopolysaccharidosis I (MPS I) have changed over time. We used data from 891 patients in the MPS I Registry, an international observational database, to analyze ages at symptom onset
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Hematopoietic stem cell transplantation (HSCT) has not been indicated for patients with mucopolysaccharidosis II (MPS II, Hunter syndrome), while it is indicated for mucopolysaccharidosis I (MPS I) patients <2 years of age and an intelligence quotient (IQ) of ≥ 70.
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N A Chamoles et al.
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Rheumatology (Oxford, England), 50 Suppl 5, v49-v59 (2012-01-11)
Better understanding of disease pathophysiology, improved supportive care and availability of disease-specific treatments for some of the mucopolysaccharidosis (MPS) disorders have greatly improved the outlook for patients with MPS disorders. Optimal management of these multisystemic disorders involves a multidisciplinary team
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