I6034
α-L-Iduronidase human
recombinant, expressed in mouse NSO cells
Synonym(s):
IDUA
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.
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.
Application
α-L-Iduronidase may be used for leukocyte assay in the study of a-L-Iduronidase deficiency in new born.
Biochem/physiol Actions
Catalyzes the hydrolysis of unsulfated α-L-iduronosidic linkages 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 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.
Physical form
Supplied as a solution in 40 mM sodium acetate , 400 mM NaCl and 20% (v/v) glycerol, pH 5.0
Other Notes
One unit will produce 1 picomole of 4-methylumbelliferone from 4-methylumbelliferyl-α-L-iduronide per minute at pH 3.5 at 25 °C.
Storage Class Code
10 - Combustible liquids
WGK
WGK 1
Flash Point(F)
Not applicable
Flash Point(C)
Not applicable
Regulatory Information
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Diagnosis of alpha-L-iduronidase deficiency in dried blood spots on filter paper: the possibility of newborn diagnosis.
N A Chamoles et al.
Clinical chemistry, 47(4), 780-781 (2001-03-29)
Akemi Tanaka et al.
Molecular genetics and metabolism, 107(3), 513-520 (2012-10-02)
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.
Ruben J Boado et al.
Bioconjugate chemistry, 24(1), 97-104 (2012-12-20)
The chronic administration of recombinant fusion proteins in preclinical animal models may generate an immune response and the formation of antidrug antibodies (ADA). Such ADAs could alter the plasma pharmacokinetics of the fusion protein, and mask any underlying toxicity of
Kristin D'Aco et al.
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
Vassili Valayannopoulos et al.
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
Our team of scientists has experience in all areas of research including Life Science, Material Science, Chemical Synthesis, Chromatography, Analytical and many others.
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