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Storage and Excretion of Oligosaccharides and Glycopeptides in the Gangliosidoses

  • Chapter
Current Trends in Sphingolipidoses and Allied Disorders

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 68))

Abstract

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).

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Authors and Affiliations

  1. Donner Laboratory of Experimental Neurochemistry Montreal Neurological Institute, McGill University Montreal, Quebec, H3A 2B4, Canada

    Leonhard S. Wolfe & N. M. K. Ng Ying Kin

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  1. Leonhard S. Wolfe
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Editors and Affiliations

  1. Kingsbrook Jewish Medical Center, Isaac Albert Research Institute, USA

    Bruno W. Volk

  2. Downstate Medical Center, State University of New York, USA

    Bruno W. Volk  & Larry Schneck  & 

  3. Neuroscience Center, Kingsbrook Jewish Medical Center, USA

    Larry Schneck

  4. Birth Defects Center, Isaac Albert Research Institute, USA

    Larry Schneck

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© 1976 Plenum Press, New York

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Wolfe, L.S., Kin, N.M.K.N.Y. (1976). Storage and Excretion of Oligosaccharides and Glycopeptides in the Gangliosidoses. In: Volk, B.W., Schneck, L. (eds) Current Trends in Sphingolipidoses and Allied Disorders. Advances in Experimental Medicine and Biology, vol 68. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-7735-1_2

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  • DOI: https://doi.org/10.1007/978-1-4684-7735-1_2

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4684-7737-5

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