7ZKY

Amyloid fibril from human systemic AA amyloidosis (vascular variant)

Summary for 7ZKY

• Entry DOI: 10.2210/pdb7zky/pdb
• EMDB information: 14771
• Descriptor: Amyloid protein A (1 entity in total)
• Functional Keywords: amyloids, vascular aa, saa, cryo-em, protein fibril
• Biological source: Homo sapiens (human)
• Total number of polymer chains: 12
• Total formula weight: 93306.02

Authors

Banerjee, S.,Schmidt, M.,Faendrich, M. (deposition date: 2022-04-13, release date: 2022-12-07, Last modification date: 2024-07-24)

Primary citation

Banerjee, S.,Baur, J.,Daniel, C.,Pfeiffer, P.B.,Hitzenberger, M.,Kuhn, L.,Wiese, S.,Bijzet, J.,Haupt, C.,Amann, K.U.,Zacharias, M.,Hazenberg, B.P.C.,Westermark, G.T.,Schmidt, M.,Fandrich, M.
Amyloid fibril structure from the vascular variant of systemic AA amyloidosis.
Nat Commun, 13:7261-7261, 2022

PubMed 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.

PubMed: 36433936
DOI: 10.1038/s41467-022-34636-4

Experimental method

ELECTRON MICROSCOPY (2.56 Å)