Moscerdam

Order Code	Disease	Substrate
INCL    »»»»»»»»»	INCL; CLN1 newly available	4MU-6-thiopalmitoyl-beta-D-glucoside     now available from Carbosynth (click INCL)
KRB	Krabbe	6HMU-beta-D-galactoside
M2    »»»»»»»»»	MPS II discontinued	4MU-alpha-L-iduronide-2-sulphate    no longer available, click M2 for alternative
M3A    »»»»»»»»»	MPS III A newly available	4MU-alpha-N-sulpho-D-glucosaminide    now available from Carbosynth (click M3A)
M3C	MPS III C	4MU-beta-D-glucosaminide
M3D    »»»»»»»»»	MPS III D  discontinued	4MU-alpha-N-acetyl-D-glucosaminide-6-sulphate        no longer available, click M3D for alternative
M4A    »»»»»»»»»	MPS IV A discontinued	4MU-beta-D-galactoside-6-sulphate        no longer available, click M4A for alternative
NPAB	Niemann-Pick A/B	6HMU-phosphorylcholine
ANAGA	Schindler	4MU-alpha-N-acetyl-D-galactosaminide

HMU

6-HMU-standard

6-hexadecanoylamino-4-methylumbelliferone

Fluorogenic substrates for the laboratory diagnosis of lysosomal storage diseases

• Each lot is biochemically tested with fibroblasts from patients, 
  in an academic, lysosomal laboratory
• Enzymes for "two-step" assays are included
• Laboratory protocols are continuously upgraded through customer feedback

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 During this period all the conventional, cumbersome assays for lysosomal enzymes with radio-labelled substrates have been replaced by robust, fluorogenic assays.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 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 Department of Medical Biochemistry, headed by Prof. Hans Aerts at the Academic Medical Centre in Amsterdam in the Netherlands. This department has a long standing experience with lysosomal storage disorders

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.