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Mitochondriopathien

Pädiatrie

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

Zusammenfassung

Mitochondrien sind die zentralen Organellen der Zelle für die Energiegewinnung. Hier befindet sich die Endstrecke der Verbrennung von Eiweiß, Zucker und Fett. Dazu wird Sauerstoff verbraucht, CO2 und Wasser gebildet. Unter Ausnutzung von 3 Protonengradienten in der Atmungskette entstehen energiereiche Phosphate (Adenosintriphosphat, ATP) über eine turbinenartig funktionierende ATP-Synthase („Lebensmotor“). Zusätzlich fällt Zellwärme an. Jede Zelle enthält abhängig von der Gewebsart wenige bis tausende Mitochondrien. Es ist bemerkenswert, dass dieses lebenswichtige Zelloxidationssystem störanfällig ist. So werden in der inneren mitochondrialen Membran freie Radikale gebildet, die nur teilweise durch das Antioxidanziensystem aufgefangen werden können. Die Imbalance zwischen Radikalproduktion und antioxidativem Schutz wird als oxidativer Stress bezeichnet.

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Authors and Affiliations

  1. Universitätsklinik und Poliklinik für Kinder- und Jugendmedizin, Paracelsus Medizinische Privatuniversität, Müllner Hauptstr. 46, 5020, Salzburg, Österreich

    W. Sperl & P. Freisinger

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  1. W. Sperl
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  2. P. Freisinger
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Correspondence to W. Sperl .

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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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Sperl, W., Freisinger, P. (2015). Mitochondriopathien. 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_76-1

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  • DOI: https://doi.org/10.1007/978-3-642-54671-6_76-1

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  • Online ISBN: 978-3-642-54671-6

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Chapter history

  1. Latest

    Mitochondriopathien
    Published:
    02 May 2019

    DOI: https://doi.org/10.1007/978-3-642-54671-6_76-2

  2. Original

    Mitochondriopathien
    Published:
    24 July 2015

    DOI: https://doi.org/10.1007/978-3-642-54671-6_76-1

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