Amyloid fibril structure from the vascular variant of systemic AA amyloidosis

Sambhasan Banerjee, Julian Baur, Christoph Daniel, Peter Benedikt Pfeiffer, Manuel Hitzenberger, Lukas Kuhn, Sebastian Wiese, Johan Bijzet, Christian Haupt, Kerstin U. Amann, Martin Zacharias, Bouke P. C. Hazenberg, Gunilla T. Westermark, Matthias Schmidt and Marcus Fändrich ()
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Sambhasan Banerjee: Ulm University
Julian Baur: Ulm University
Christoph Daniel: Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU)
Peter Benedikt Pfeiffer: Ulm University
Manuel Hitzenberger: Technical University of Munich
Lukas Kuhn: Ulm University
Sebastian Wiese: Ulm University
Johan Bijzet: University Medical Center Groningen, University of Groningen
Christian Haupt: Ulm University
Kerstin U. Amann: Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU)
Martin Zacharias: Technical University of Munich
Bouke P. C. Hazenberg: University Medical Center Groningen, University of Groningen
Gunilla T. Westermark: Uppsala University
Matthias Schmidt: Ulm University
Marcus Fändrich: Ulm University

Nature Communications, 2022, vol. 13, issue 1, 1-8

Abstract: Abstract Systemic AA amyloidosis is a debilitating protein misfolding disease in humans and animals. In humans, it occurs in two variants that are called ‘vascular’ and ‘glomerular’, depending on the main amyloid deposition site in the kidneys. Using cryo electron microscopy, we here show the amyloid fibril structure underlying the vascular disease variant. Fibrils purified from the tissue of such patients are mainly left-hand twisted and contain two non-equal stacks of fibril proteins. They contrast in these properties to the fibrils from the glomerular disease variant which are right-hand twisted and consist of two structurally equal stacks of fibril proteins. Our data demonstrate that the different disease variants in systemic AA amyloidosis are associated with different fibril morphologies.

Date: 2022
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DOI: 10.1038/s41467-022-34636-4

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