Mukopolysaccharidosen

Living reference work entry
Part of the Springer Reference Medizin book series (SRM)

Zusammenfassung

Mukopolysaccharidosen (MPS) sind erbliche, progrediente Speicherkrankheiten, hervorgerufen durch die intrazelluläre Anhäufung von Glykosaminoglykanen (sauren Mukopolysacchariden). Glykosaminoglykane sind komplexe Kohlenhydratketten aus Uronsäuren, Aminozuckern und Neutralzuckern. Die wichtigsten sind Chondroitin-4-sulfat, Chondroitin-6-sulfat, Heparansulfat, Dermatansulfat, Keratansulfat und Hyaluronan. Sie werden intrazellulär synthetisiert, überwiegend in den Extrazellulärraum ausgeschieden und teilweise in Lysosomen wieder abgebaut. Im Gewebe sind sie mit Proteinen zu großmolekularen Proteoglykanen verbunden.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.SinzheimDeutschland

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