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.
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
10 - Combustible liquids
wgk
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
Regulatory Information
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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
Human kidney alpha-L-iduronidase: purification and characterization.
L H Rome et al.
Archives of biochemistry and biophysics, 189(2), 344-353 (1978-08-01)
Francesca Gatto et al.
Stem cells and development, 21(9), 1466-1477 (2012-01-28)
Mucopolysaccharidosis type I (MPS IH; Hurler syndrome) is a rare genetic disorder that is caused by mutations in the α-L-iduronidase (IDUA) gene, resulting in the deficiency of IDUA enzyme activity and intra-cellular accumulation of glycosaminoglycans. A characteristic skeletal phenotype is
Global Trade Item Number
| SKU | GTIN |
|---|---|
| I6034-10UG | 04061833857663 |