Virchows Archiv B

, Volume 11, Issue 1, pp 224–233 | Cite as

Histochemical demonstration of acid mucopolysaccharides in Sanfilippo’s disease: An ultrastructural study of the liver

  • E. Konyar
  • M. Tondeur
  • A. Resibois


In the liver of three children with Sanfilippo’s disease, electron microscopic studies show that the content of the clear inclusions, specific for this kind of disease, presents an affinity for silver nitrate after periodic oxidation, for phosphotungstic acid at pH 1.5 and for colloidal iron pH 1.8. It is resistant to hyaluronidase digestion. These results demonstrate the presence of mucopolysaccharide and more precisely of heparan-sulfate.


Heparan Sulfate Kupffer Cell Silver Nitrate Phosphotungstic Acid Mucopolysaccharide 
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Copyright information

© Springer-Verlag 1972

Authors and Affiliations

  • E. Konyar
    • 1
    • 2
    • 4
  • M. Tondeur
    • 1
    • 2
  • A. Resibois
    • 1
    • 2
    • 3
  1. 1.Laboratory of Pathology and Electron MicroscopyUniversity Hospital St. PierreBrusselsBelgium
  2. 2.Department of PediatricsUniversity Hospital St. PierreBrusselsBelgium
  3. 3.Department of Electron MicroscopySchool of MedicineBrusselsBelgium
  4. 4.2nd Department of PathologyMedical University of BudapestHungary

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