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  • 1
    Electronic Resource
    Electronic Resource
    The Biochemistry of Sphingolipid Storage Diseases (1977)
    Weinheim : Wiley-Blackwell
    Angewandte Chemie International Edition in English 16 (1977), S. 273-285 
    Details
    ISSN: 0570-0833
    Keywords: Sphingolipids ; Hereditary diseases ; Neurochemistry ; Chemistry ; General Chemistry
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology
    Notes: Sphingolipids are components of animal membranes that are made up of a hydrophobic ceramide and a hydrophilic moiety (phosphorylcholine, oligosaccharides or their derivatives). Several enzymes are required for the step-by-step degradation of the sphingolipids. If one of these enzymes is deficient or totally lacking, then its substrates accumulate in the body and cause severe damage, especially when storage occurs in the nervous system. The enzyme defects are inherited in a recessive manner.
    Additional Material: 8 Ill.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/anie.197702733
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