recombinant
expressed in mouse NSO cells
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
Quality Level
General description
α-L-艾杜糖苷酸酶 (IDUA) 定位于人染色体4p16.3。成熟的IDUA蛋白被糖基化，并包含三糖磷酸异构酶（TIM）桶状结构域、β夹心式螺旋-环-螺旋区域和免疫球蛋白样结构域。α-L-艾杜糖苷酸酶被归类为糖苷水解酶（GH）家族39。
在 SDS-PAGE 还原条件下，表达为 C-末端组氨酸标记蛋白（残基 1-653），其含钙核分子量为 71 kDa，在约 83 kDa 处迁移。
Application
α-L-艾杜糖醛酸酶可用于新生儿 a-L-艾杜糖醛酸酶缺乏症的白细胞测定。
Biochem/physiol Actions
α-L-艾杜糖苷酸酶突变与粘多糖贮积病I型（MPS I）有关。该酶的缺陷导致皮肤素和硫酸乙酰肝素的积累。MPS I的病理生理学伴随着颅骨变形、智力低下和疝气。
催化硫酸皮肤素中未硫酸化的 L -艾杜糖苷键的水解
在溶酶体降解过程中，α-L-艾杜糖苷酸酶（Iduronidase ）起着至关重要的作用。它水解糖胺聚糖（GAG）（包括硫酸皮肤素和硫酸乙酰肝素） 中非还原末端的α L-艾杜糖醛酸残基。
Physical form
40 mM 醋酸钠、400 mM NaCl 和 20% (v/v) 甘油溶液 (pH 5.0)
Other Notes
一个单元将在-25°C pH 3.5 下每分钟从 4-甲基伞形酮-α-L-艾杜糖苷酸生成 1 皮摩尔的 4-甲基伞形酮。
存储类别
10 - Combustible liquids
wgk
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
法规信息
常规特殊物品
此项目有
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
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
Xu He et al.
Plant molecular biology, 79(1-2), 157-169 (2012-03-24)
Processes associated with late events of N-glycosylation within the plant Golgi complex are a major limitation to the use of plant-based systems to produce recombinant pharmaceutical proteins for parenteral administration. Specifically, sugars added to the N-glycans of a recombinant protein
Haiying Bie et al.
Nature chemical biology, 9(11), 739-745 (2013-09-17)
Mucopolysaccharidosis type I (MPS I), caused by mutations in the gene encoding α-L-iduronidase (IDUA), is one of approximately 70 genetic disorders collectively known as the lysosomal storage diseases. To gain insight into the basis for MPS I, we crystallized human
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