产品名称

α-L-Iduronidase human

一般描述

α-L-Iduronidase is an enzyme encoded by IDUA gene; participate in lysosomal degradation of glycosaminoglycans.In human, mutation of gene encoding α-L-Iduronidase causes lysosomal storage diseases (mucopolysaccharidosis type I).

含 量

recombinant, expressed in mouse NSO cells; ≥7,500units/μg protein; ≥95% by SDS-PAGE

杂 质

≤1.0EU/μg Endotoxin

适用范围

生物试剂

l  理化信息

物理形态

Supplied as a solution in 40mM sodium  acetate ,400 mM NaCl and 20% (v/v) glycerol, pH5.0

l  生物学信息

生物活性/药理作用

Catalyzes the hydrolysis of unsulfated α-L-iduronosidic ages 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 GAGs, including dermatan sulfate and heparan sulfate.

应 用

α-L-Iduronidase may be used for leukocyte assay in the study of a-L-Iduronidase deficiency in new born.

有效单位定义

One unit will produce 1 picomole of 4-methylumbelliferone from 4-methylumbelliferyl-α-L-iduronide per minute at pH3.5 at 25°C.

l  存储

存储温度

-20°C

l  注意事项及免责声明

本产品仅用于实验研究，不得作为药物使用，不得用于家用或其它用途。

l  参考文献

1.    http://www.drugbank.ca

2.    https://ncit.nci.nih.gov

3.    https://www.ncbi.nlm.nih.gov