History of Moscerdam Substrates
Moscerdam Substrates has been the pioneer for novel substrates for lysosomal enzymes
for the last 15 years. During this period all the conventional, cumbersome assays for
lysosomal enzymes with radio-labelled substrates have been replaced by robust,
fluorogenic assays. The entire range of substrates was developed in a scientific
collaboration between the Institute of Organic Chemistry, Russian Academy of Sciences,
Moscow and the Metabolic Unit of the Erasmus MC, Rotterdam, the Netherlands.

Quality Control
Moscerdam substrates are not only of high chemical purity, but more importantly are
biochemically pure. Enzyme laboratory practice has shown that chemical purity does not
always guarantee optimal performance of enzyme assays. Traces of impurity (< 1%) can
severely jeopardise results. All Moscerdam substrates are tested with fibroblasts from
patients with the corresponding lysosomal storage disease. This biochemical testing is the
only way to ensure constant high-quality substrates.
The tests are performed in the Metabolic Unit of the Department of Clinical Genetics
of the Erasmus University Medical Centre in Rotterdam, the Netherlands.
This unit has been accredited as a diagnostic laboratory in 2005 by the Dutch Laboratory
Accreditation Authority and is one of the leading centres
for the laboratory diagnosis of metabolic disorders.
www.genetischemetaboleziekten.nl

Special features
Certain enzyme assays require additional lysosomal enzymes for their determination.
Enzymes not available from other companies, are provided by Moscerdam Substrates as
ready-for-use kits (e.g. for Hunter’s and Sanfilippo D disease).
All substrates are accompanied by detailed laboratory protocols, which are continuously
refined through the feedback of customers.