Arylsulfatases A and B in Metachromatic Leukodystrophy and Maroteaux-Lamy Syndrome: Studies with 4-Methylumbelliferyl Sulfate

Metachromatic leukodystrophy (MLD), a sphingolipidosis, and Maroteaux-Lamy syndrome (MLS), a mucopolysaccharidosis, are genetically distinct diseases which have in common a markedly impaired ability to cleave the artificial substrate p-nitrocatechol sulfate (NCS) under certain specified conditions. Numerous studies employing this substrate have demonstrated deficiency of arylsulfatase A (AS-A) in MLD (1,6,7) and of arylsulfatase B (AS-B) in MLS(3, 4, 7, 13).However, several problems exist in connection with its use.