Nephrologie aktuell 2024; 28(06): 257-263
DOI: 10.1055/a-2276-1494
Schwerpunkt
Nephrologie

Renale Amyloidosen

Etablierte Therapiestrategien und experimentelle Ansätze
Jens Gaedeke
1   Klinik für Nephrologie/Dialyse, Evangelisches Krankenhaus Königin Elisabeth Herzberge, Berlin
› Author Affiliations

ZUSAMMENFASSUNG

Die Amyloidosen sind eine Gruppe von seltenen Erkrankungen, die sich durch die Bildung und Ablagerung von aberrant gefalteten Proteinen definieren. Grundsätzlich können sowohl Mutationen als auch die Überproduktion von Proteinen den komplexen Prozess der Amyloidogenese starten. Krankheitswert bekommen diese Ablagerungen teilweise durch direkte zytotoxische Effekte, größtenteils jedoch durch eine mechanische Störung der Organarchitektur (z. B. Ablagerung in der glomerulären Basalmembran). Die Diagnose wird oft erst in späteren Stadien über einen manifesten Organschaden (z. B. Proteinurie) gestellt. Die Klinik wird wesentlich von der Art des Vorläuferproteins beziehungsweise der daraus folgenden Organlokalisation bestimmt. Das Überleben hängt bei vielen Amyloidoseerkrankungen vom Grad der kardialen Beteiligung ab. Etablierte Therapiestrategien zielen vor allem auf die Produktionshemmung der Vorläuferproteine ab; experimentelle Ansätze verfolgen das Ziel, bereits abgelagertes Amyloid aufzulösen.



Publication History

Article published online:
24 July 2024

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