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

  • Edwin H. Kolodny
  • Richard A. Mumford
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 68)


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.


Sodium Acetate Lead Acetate Heat Inactivation Metachromatic Leukodystrophy Cellulose Acetate Strip 
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Copyright information

© Plenum Press, New York 1976

Authors and Affiliations

  • Edwin H. Kolodny
    • 1
    • 2
  • Richard A. Mumford
    • 1
    • 2
  1. 1.Eunice Kennedy Shriver Center for Mental Retardation, Inc.Walter E. Fernald State SchoolWalthamUSA
  2. 2.Department of NeurologyMassachusetts General HospitalBostonUSA

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