Zusammenfassung
Mukopolysaccharidosen (MPS) sind erbliche Speicherkrankheiten, hervorgerufen durch eine intrazelluläre Anhäufung von Glykosaminoglykanen (sauren Mukopolysacchariden). Glykosaminoglykane sind komplexe Kohlenhydratketten aus Uronsäuren, Aminozuk-kern und Neutralzuckern. Im Gewebe sind sie mit Proteinen zu großmolekularen Proteoglykanen verbunden.
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Spranger, J. (2003). Heteroglykanosen. In: Lentze, M.J., Schulte, F.J., Schaub, J., Spranger, J. (eds) Pädiatrie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-09176-0_34
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DOI: https://doi.org/10.1007/978-3-662-09176-0_34
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