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Pädiatrie pp 304-313 | Cite as

Heteroglykanosen

  • J. Spranger

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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© Springer-Verlag Berlin Heidelberg 2003

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  • J. Spranger

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