p.Nitrocatechol Sulfate for Arylsulfatase Assay: Detection of Metachromatic Leukodystrophy Variants

  • Gisèle Dubois
  • Jean-Claude Turpin
  • Nicole Baumann
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 68)


Artificial substrates are being used routinely for the diagnosis of lipidosis. In the case of metachromatic leukodystrophy (MLD), p.nitrocatechol-sulfate (p.NCS) at acidic pH is the substrate for arylsulfatase A (ASA). Complete analogy of elution profile on column chromatography between ASA and cerebroside sulfate sulfatase (1) has secured in the use of the artificial substrate for MLD diagnosis. Although an activator protein is necessary for enzyme action on the natural substrate (2), only the ASA enzyme itself is deficient in MLD variant B (3). Another enzyme arylsulfatase B (ASB) reacts with p.NCS at less acidic pH; together with ASA and other sulfatases, it is deficient in another type of sulfatidosis, Austin’s disease or MLD variant O (3).


Colorimetric Assay Artificial Substrate Nerve Biopsy Identical Monomer Complete Analogy 
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Copyright information

© Plenum Press, New York 1976

Authors and Affiliations

  • Gisèle Dubois
    • 1
    • 2
  • Jean-Claude Turpin
    • 1
    • 2
  • Nicole Baumann
    • 1
    • 2
  1. 1.Laboratoire de NeurochimieINSERM U.134, CNRS ERA 421France
  2. 2.Hôpital de la SalpêtrièreParis Cèdex 13France

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