Bis-L-Aspartic acid Beta-rhodamine 110

Bis-L-Aspartic acid beta-rhodamine 110 is a sensitive fluorogenic substrate for studying lysosomal glycoasparaginases (aspartylglucosaminidases) and L-asparaginases. It is used for the assays for lysosomal glycoasparaginase (aspartylglucosaminidase) activity and the diagnosis of aspartylglucosaminuria. Aspartylglucosaminidase is an amidohydrolase enzyme involved in the catabolism of N-linked oligosaccharides of glycoproteins. It cleaves asparagine from N-acetylglucosamines as one of the final steps in the lysosomal breakdown of glycoproteins. The lysosomal storage disease aspartylglycosaminuria is caused by a deficiency in the AGA enzyme.