Zusammenfassung
Amyloid ist eine pathologische fibrilläre Polypeptidaggregation mit einer Cross-β-Struktur, die intra- und/oder extrazellulär auftritt. Amyloidosen sind durch Amyloidablagerungen verursachte Krankheiten, die sich zerebral und extrazerebral manifestieren können. Über 29 verschiedene Proteine können Amyloid bilden. Die polarisationsoptische Auswertung des kongorotgefärbten Schnittpräparats ist der Goldstandard für den Nachweis des Amyloids, das anschließend klassifiziert werden muss. Inzwischen sind auch molekularpathologische Zusatzuntersuchungen ein fester Bestandteil der klinisch-pathologischen Amyloiddiagnostik geworden und haben in Deutschland zur Identifikation zahlreicher hereditärer Amyloidosen geführt. Eine genaue Klassifikation des Amyloids und der Amyloidose ist für die Prognoseabschätzung und Therapieplanung unverzichtbar.
Abstract
Amyloid is a pathologic fibrillar aggregation of polypeptides in a cross-β-sheet conformation. Amyloidoses are caused by the deposition of amyloid and may occur as cerebral and extracerebral disease. More than 29 different amyloid proteins have been identified. Analysis of a Congo red-stained tissue section by polarization microscopy is the gold standard for diagnosing amyloid. Subsequent classification of the amyloid is mandatory and is increasingly supported by molecular biological analyses. In Germany, this recently led to the discovery of several hereditary amyloid diseases. The correct classification of amyloid is of paramount importance. This helps to asses the prognosis and plan patient treatment.
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Danksagung
Die Autoren danken Frau Margrit Möller-Schubotz für die sorgfältige Pflege des Amyloidregisters sowie allen Pathologinnen und Pathologen, die uns Fälle zur Klassifikation zugeschickt haben. Besonderer Dank gilt auch Professor Loy (Berlin) und Professor Herbst (Berlin) für die Zusendung des Falles mit subepithelialer kornealer Amyloidose (Abb. 2 a, b). Die Studien von C.R. und M.E. werden von der Europäischen Union finanziell unterstützt (EU FP6 EURAMY).
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Röcken, C., Eriksson, M. Amyloid und Amyloidosen. Pathologe 30, 182–192 (2009). https://doi.org/10.1007/s00292-009-1128-1
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DOI: https://doi.org/10.1007/s00292-009-1128-1