Lysosomal storage disease (LSD substrates)

Lysosomal storage disorders are a group of over 70 rare genetically inherited metabolic diseases, the majority of which are due to deficiency in lysosomal enzymes. The deficiency of a single enzyme required for the metabolism of lipids, glycoproteins or mucopolysaccharides cause these substances to accumulate in the lysosome, this has implications on multiple systems in the body. Glycosynth offers a range of fluorogenic substrates for the detection of lysosomal enzymes for diagnosis of lysosomal storage disorders.

4-Methylumbelliferyl alfa-L-iduronide free acid (Product code 44076) is a fluorogenic substrate for the study of Hurler - Scheie Syndrome MPS-I [Blood, 2002, 99, 1857]. Glycosynth has supplied this high chemical and biochemical purity product since 1995, which has been approved in application by our customers and credited in research papers. Our product is the free acid, which offers a considerable weight for weight advantage over the CHA salt (4-Methylumbelliferyl alpha-L-iduronic acid cyclohexylammonium salt, CAS 66895-33-8 in terms of its application dose.