Storage and Excretion of Oligosaccharides and Glycopeptides in the Gangliosidoses

  • Leonhard S. Wolfe
  • N. M. K. Ng Ying Kin
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 68)


Electron microscopical studies of visceral tissues from the infantile form of GM1-gangliosidosis (Type I or generalized gangliosidosis) show extensive cytoplasmic vacuolation of parenchymal cells, histiocytes, fibrocytes and lymphocytes (9,23–25, 29,33,36,44). Indeed, in the liver, the majority of the hepatocytes are packed with membrane-limited vacuoles filled with highly water-soluble, α-amylase-resistant, PAS-positive material. Similar morphological features are also seen in an affected fetus at 17 weeks of gestation with in addition intense vacuolation of the syncytiotrophoblasts of the placenta (16,28). Vacuolation of hepatocytes is much less marked in the Type II late infantile or juvenile form of of-gangliosidosis (9,11,27). It was recognized early in the descriptions of this disease that the material stored in the vacuoles was unlikely to be GM1-ganglioside since the ul trastructural features were quite different from the neuronal membranous cytoplasmic bodies characteristic of ganglioside storage. Earlier chemical studies of not too rigorous a nature established that the liver from Type I GM1-gangliosidosis contained excessive amounts of polysaccharides rich in galactose and N-acetylglucosamine and fractions containing some mannose were also recognized (5,32–34,44).


Keratan Sulfate Sandhoff Disease Carbohydrate Sequence Trimannosyl Core Ride Fraction 
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Copyright information

© Plenum Press, New York 1976

Authors and Affiliations

  • Leonhard S. Wolfe
    • 1
  • N. M. K. Ng Ying Kin
    • 1
  1. 1.Donner Laboratory of Experimental Neurochemistry Montreal Neurological InstituteMcGill University MontrealQuebecCanada

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