产品名称：双-L-天冬氨酸β-罗丹明110

规格：1mg

储存条件：-15℃避光防潮

保质期：24个月

产品物理化学光谱特性

分子量：788.57

溶剂：DMSO

激发波长(nm)：498

发射波长(nm)：520

产品介绍

双-L-天冬氨酸β-罗丹明110是美国AAT Bioquest生产的罗丹明染料 ，Bis-L-天冬氨酸β-若丹明110是一种敏感的荧光底物，用于研究溶酶体糖类天冬酰胺酶（天冬氨酰葡萄糖苷酶）和L-天冬酰胺酶。 它用于溶酶体糖类天冬酰胺酶（天冬氨酰葡糖胺酶）活性的测定和天冬氨酰糖尿症的诊断。 天冬氨酰氨基葡糖苷酶是参与糖蛋白的N-连接寡糖分解代谢的酰胺水解酶。 它从N-乙酰氨基葡萄糖中裂解天冬酰胺，这是糖蛋白溶酶体降解的最后步骤之一。 溶酶体贮积病天冬氨酰糖尿症是由AGA酶缺乏引起的。

参考文献

Aspartylglycosaminuria in a non-Finnish patient caused by a donor splice mutation in the glycoasparaginase gene
Authors: I. Mononen
Journal: J Biol Chem (1992): 3196-9

Chromosomal localization of the human glycoasparaginase gene to 4q32-q33
Authors: C. Morris
Journal: Hum Genet (1992): 295-7

Splicing defect of the glycoasparaginase gene in two Japanese siblings with apartylglucosaminuria
Authors: K. Yoshida
Journal: Hum Genet (1992): 179-80

Substrate specificity and reaction mechanism of human glycoasparaginase. The N-glycosidic linkage of various glycoasparagines is cleaved through a reaction mechanism similar to L-asparaginase
Authors: V. Kaartinen
Journal: J Biol Chem (1992): 6855-8

Aspartylglycosaminuria in the Finnish population: identification of two point mutations in the heavy chain of glycoasparaginase
Authors: I. Mononen
Journal: Proc Natl Acad Sci U S A (1991): 2941-5

Glycoasparaginase in human urine
Authors: V. Kaartinen
Journal: Biochim Biophys Acta (1991): 28-30