7zky

Publication Abstract from PubMed

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.

Amyloid fibril structure from the vascular variant of systemic AA amyloidosis.,Banerjee S, Baur J, Daniel C, Pfeiffer PB, Hitzenberger M, Kuhn L, Wiese S, Bijzet J, Haupt C, Amann KU, Zacharias M, Hazenberg BPC, Westermark GT, Schmidt M, Fandrich M Nat Commun. 2022 Nov 25;13(1):7261. doi: 10.1038/s41467-022-34636-4. PMID:36433936^[1]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.