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Fettstoffwechsel pp 613–643Cite as

Gangliosidosen

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Part of the book series: Handbuch der inneren Medizin ((INNEREN 7,volume 7 / 4))

Zusammenfassung

Gangliosidosen sind durch genetischen Enzymdefekt bedingte seltene Lipidstoffwech-selstörungen, bei denen Ganglioside, also spezielle Glykosphingolipide (s. Abschnitt E) pathologisch angehäuft werden. Der fatale Speicherprozeß läuft vor allem im zentralen Nervensystem ab; der Tod tritt im allgemeinen im frühen oder späteren Kindesalter ein.

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Authors
  1. K. Harzer
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  2. H. U. Benz
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Editor information

Editors and Affiliations

  1. Abteilung für Klinische Chemie der Universitätskliniken, Joseph-Stelzmann-Straße 9, D-5000, Köln 41, Deutschland

    G. Assmann

  2. Klinisches Institut für Herzinfarktforschung, Medizinische Universitätsklinik, Bergheimer Straße 58, D-6900, Heidelberg, Deutschland

    J. Augustin , H. Greten , G. Middelhoff  & G. Schlierf , ,  & 

  3. Section and Laboratory of Liver Diseases, Nutrition and Alcoholism, Veterans Administration Hospital, 130 West Kingsbridge Road, Bronx, N.Y., 10468, USA

    E. Baraona  & C. S. Lieber  & 

  4. Mt. Sinai School of Medicine(CUNY), New York, N.Y., USA

    E. Baraona  & C. S. Lieber  & 

  5. Institut für Hirnforschung der Universität, Belthlestraße 15, D-7400, Tübingen, Deutschland

    H. U. Benz  & K. Harzer  & 

  6. Internistische Germeinschaftspraxis, Dialyse-Institut, Werastraße 33, D-7990, Friedrichshafen, Deutschland

    W. Brech

  7. Division of Metabolic Disease, Department of Medicine, School of Medicine, University of California, La Jolla, California, 92037, USA

    W. V. Brown

  8. Departments of Pediatrics, Genetics and Cell Biology and The Dight Institute of Human Genetics, University of Minnesota, Box 231, Mayo Memorial Building, Minneapolis, Minnesota, 55455, USA

    R. J. Desnick

  9. Department of Internal Medicine, College of Medicine, Lipid Research Clinic, University of Cincinnati Medical Center, 234 Goodman Street, Cincinnati, Ohio, 45229, USA

    W. R. Fallat

  10. Lipid Research Center, University of Cincinnati, Cincinnati, Ohio, 45229, USA

    S. Ford  & Ch. J. Glueck  & 

  11. General Clinical Research Center, University of Cincinnati, Cincinnati, Ohio, 45229, USA

    S. Ford  & Ch. J. Glueck  & 

  12. National Institutes of Health, Bethesda, Maryland, 20014, USA

    D. Fredrickson

  13. Molecular Disease Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland, 20014, USA

    P. Herbert  & R. I. Levy  & 

  14. Institut für Medizinische Biochemie der Universitä, Harrachgasse 21, A-8010, Graz, Öterreich

    G. M. Kostner

  15. Department of Pediatrics, University of Minnesota, Box 231, Mayo Memorial Building, Minneaplis, Minnesota, 55455, USA

    W. Krivit

  16. Medizinische Universitätsklinik, Bergheimer Straße 58, D-6900, Heidelberg, Deutschland

    M. Liersch , G. Schettler , E. Stange  & A. Weizel , ,  & 

  17. Courtauld Institute of Biochemistry, Middlesex Hospital Medical School, London W 1, England

    G. L. Mills

  18. Medical Research Council Lipid Metabolism Unit, Hammersmith Hospital, Ducane Road, London, W12 OHS, England

    N. B. Myant  & J. Slack  & 

  19. Departments of Pediatrics, University of Minnesota Medical School, Minneapolis, Minnesota, 55455, USA

    R. F. O’Dea

  20. Research Associate in the Pharmacology, Laboratory of Clinical Science, National Institute of Mental Health, Bethesda, Maryland, 20014, USA

    R. F. O’Dea

  21. Innere Abteilung, Knappschafts-Krankenhaus, Am Deimelsberg 34a, 4300, Essen-Steele, Deutschland

    J. Papenberg

  22. Neurologische Klinik und Poliklinik der Universität, v. Siebold-Straße 5, D-3400, Göttingen, Deutschland

    H. Pilz

  23. Schierghoferstraße 24a, D-8220, Traunstein/Obb, Deutschland

    H. M. Rauen

  24. Medizinische Universitätsklinik, Anich-Straße 35, A-6020, Innsbruck, Austria

    S. Sailer

  25. Abteilung für Experimentlle Zellforschung, Physiologisch-Chemisches Institute der Universität, Waldeyerstraße 15, D-4400, Münster/Westf, Deutschland

    H. Schriewer

  26. Klinisch-Chemisches Laboratorium, Medizinische Universitätsklinik, Bergheimer Straße 58, D-6900, Heidelberg, Deutschland

    D. Seidel  & H. Wieland  & 

  27. Department of Experimental Medicine and Cancer Research, Hebrew University Hadassah Medical School, Jerusalem, Israel

    O. Stein

  28. Lipid Research Laboratory, Department of Medicine B, Hadassah University Hospital, Jerusalem, Israel

    Y. Stein

  29. Division of Metabolic Disease, Department of Medicine, School of Medicine, University of California, San Diego, La Jolla, California, 92093, USA

    D. Steinberg

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Harzer, K., Benz, H.U. (1976). Gangliosidosen. In: Assmann, G., et al. Fettstoffwechsel. Handbuch der inneren Medizin, vol 7 / 4. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-66302-4_28

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  • DOI: https://doi.org/10.1007/978-3-642-66302-4_28

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