跳转至内容
Merck
CN

I6034

α- L -艾杜糖醛酸酶 人

recombinant, expressed in mouse NSO cells

别名:

IDUA

登录 查看组织和合同定价。

选择尺寸


关于此项目

UNSPSC Code:
12352204
NACRES:
NA.54
Specific activity:
≥7,500 units/μg protein
Recombinant:
expressed in mouse NSO cells
技术服务
需要帮助?我们经验丰富的科学家团队随时乐意为您服务。
让我们为您提供帮助
技术服务
需要帮助?我们经验丰富的科学家团队随时乐意为您服务。
让我们为您提供帮助

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

历史批次信息供参考:

分析证书(COA)

Lot/Batch Number

没有发现合适的版本?

如果您需要特殊版本,可通过批号或批次号查找具体证书。

已有该产品?

在文件库中查找您最近购买产品的文档。

访问文档库

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

我们的科学家团队拥有各种研究领域经验,包括生命科学、材料科学、化学合成、色谱、分析及许多其他领域.

联系客户支持