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Glykogenspeicherkrankheiten

Pädiatrie

Part of the book series: Springer Reference Medizin ((SRM))

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Zusammenfassung

Mit der Nahrung zugeführte Kohlenhydrate werden als Monosaccharide resorbiert. Ein großer Teil der ins Blut aufgenommenen Glukose wird nicht sofort metabolisiert, sondern vor allem in der Leber, aber auch in Muskel und Niere in Glykogen eingebaut. In Nüchternphasen wird Glukose, zunächst noch an Phosphat gebunden, durch Glykogenolyse aus dem gespeicherten Glykogen wieder mobilisiert. Glukose-6-Phosphat kann in der Leber auch aus anderen Monosacchariden, aus Eiweißabbauprodukten oder Laktat entstehen (Glukoneogenese). Nur in Leber und Niere kann freie Glukose nach Spaltung durch das Glukose-6-Phosphatase-System an das Blut abgegeben und für andere Organe zur Verfügung gestellt werden. Synthese und Abbau von Glykogen in der Leber sind über Substrate sowie Hormone sehr fein reguliert und von wesentlicher Bedeutung für die Blutzuckerhomöostase.

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Notes

  1. 1.

    Umrechnung: mg/dl × 0,05551 = mmol/l.

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Authors and Affiliations

  1. Klinik und Poliklinik für Kinder- und Jugendmedizin, Universitätsklinikum Hamburg-Eppendorf, Martinistr. 52, 20246, Hamburg, Deutschland

    R. Santer & K. Ullrich

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  1. R. Santer
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  2. K. Ullrich
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Correspondence to R. Santer .

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Editors and Affiliations

  1. Universität Heidelberg, Zentrum für Kinder- und Jugendmedizin, Heidelberg, Germany

    Georg F. Hoffmann

  2. Zentrum für Kinderheilkunde, Bonn, Germany

    Michael J. Lentze

  3. Sinzheim, Germany

    Jürgen Spranger

  4. Universitätsmedizin Mainz Zentrum für Kinder- und Jugendmediz, Mainz, Germany

    Fred Zepp

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Santer, R., Ullrich, K. (2015). Glykogenspeicherkrankheiten. In: Hoffmann, G., Lentze, M., Spranger, J., Zepp, F. (eds) Pädiatrie. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-54671-6_78-1

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  • DOI: https://doi.org/10.1007/978-3-642-54671-6_78-1

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  • Online ISBN: 978-3-642-54671-6

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  1. Latest

    Glykogenspeicherkrankheiten
    Published:
    02 May 2019

    DOI: https://doi.org/10.1007/978-3-642-54671-6_78-2

  2. Original

    Glykogenspeicherkrankheiten
    Published:
    21 September 2015

    DOI: https://doi.org/10.1007/978-3-642-54671-6_78-1

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