Metachromatic Leukodystrophy (MLD): X. Immunological Studies of the Abnormal Sulfatase A

David Stumpf, Edward Neuwelt, James Austin, Peter Kohler

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20 Scopus citations

Abstract

A precipitating antibody was produced by immunizing rabbits with normal human sulfatase A. The rabbit antibody precipitated both the normal and the metachromatic leukodystrophy (MLD) sulfatase A from liver supernatant fractions. In the MLD samples, there was negligible sulfatase A activity in the resulting precipitin lines. Thus, the enzyme protein is present in MLD but is functionally abnormal. Ouchterlony double diffusion studies indicated that the sulfatase A protein in MLD was antigenically equivalent to the normal sulfatase A. The antibody does increase the activity of normal sulfatase A. It did not restore any substantial activity to the MLD enzyme, or to the heat inactivated normal enzyme. The existence of an enzyme protein in MLD is of interest. It raises the possibility that the activity of this protein might be increased by a therapeutic agent.

Original languageEnglish (US)
Pages (from-to)427-431
Number of pages5
JournalArchives of Neurology
Volume25
Issue number5
DOIs
StatePublished - Nov 1971

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Keywords

  • Metachromaticleukodystrophy
  • cerebroside sulfatase
  • monospecific antibody to sulfatase A
  • ouchterlony double diffusion studies
  • sulfatase A
  • variant form of metachromatic leukodystrophy

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology

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