Canine GM2-Gangliosidosis: Chemical and Enzymatic Features

  • Yoshikatsu Eto
  • Lucila Autilio-Gambetti
  • John T. McGrath
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 174)


GM2-gangliosidosis in animals has been observed in cat, dog and swine.1–4 In 1968, McGrath et al. found a new strain of GM2-gangliosidosis in male German short hair pointer dog which had shown signs of progressive neural dysfunction. Onset of the disease was 10–11 months. Clinical signs initiated by a deterioration of his previously keen response to a whistle, included in sequence: incoordination and postual difficulties, progressive impairment of hearing and vision, psychic changes simulating idiocy, terminal convulsions and prostration. Histological observations showed typical ballooning of the cell bodies of many neurons throughout the nervous system. The intracyto-plasmic materials was Sudan black B and PAS positive. Electron microscopic examination revealed typical MCB as found in human Tay-Sachs disease.4 Clinical and pathological features in dog GM2-gangliosidosis appear to be similar to human Tay-Sachs disease. Brief biochemical features of canine GM2-gangliosidosis were previously reported by Autilio-Gambetti. The present report concerns more detail on chemical and enzymatic analysis in tissues of canine GM2-gangliosidosis.


Enzymatic Feature Sandhoff Disease Brain Ganglioside Neutral Glycolipid Total Ganglioside 
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Copyright information

© Plenum Press, New York 1984

Authors and Affiliations

  • Yoshikatsu Eto
    • 1
  • Lucila Autilio-Gambetti
    • 2
  • John T. McGrath
    • 3
  1. 1.Department of PediatricsThe Tokyo Jikei University School of MedicineTokyoJapan
  2. 2.Institute of PathologyCase Western Reserve UniversityClevelandUSA
  3. 3.Veterinary SchoolUniversity of PennsylvaniaPhiladelphiaUSA

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