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Heterogeneity and Special Features of the Storage Process in Niemann-Pick Disease

  • Conference paper
Lipid Storage Disorders

Part of the book series: NATO ASI Series ((NSSA,volume 150))

Abstract

The main topic discussed during the Prague Symposium on Niemann- Pick disease in 1982 was the prominent heterogeneity of the chemical pathology of the complex urgently calling for revision of the original Croker’s scheme (Croker, 1961) in which all the types were considered as sphingomyelinoses differing only in the degree of storage of the critical lipid (Elleder and Jirasek, 1983). Recent biochemical investigations on Niemann- Pick disease explained its chemical heterogeneity in terms of enzymology, i.e. succeeded in finding out the metabolic error of the second part of the Niemann-Pick disease complex (Butler et al., 1987)

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© 1988 Plenum Press, New York

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Elleder, M. (1988). Heterogeneity and Special Features of the Storage Process in Niemann-Pick Disease. In: Salvayre, R., Douste-Blazy, L., Gatt, S. (eds) Lipid Storage Disorders. NATO ASI Series, vol 150. Springer, Boston, MA. https://doi.org/10.1007/978-1-4613-1029-7_17

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  • DOI: https://doi.org/10.1007/978-1-4613-1029-7_17

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4612-8300-3

  • Online ISBN: 978-1-4613-1029-7

  • eBook Packages: Springer Book Archive

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