7ZKY

Amyloid fibril from human systemic AA amyloidosis (vascular variant)

7ZKY の概要

• エントリーDOI: 10.2210/pdb7zky/pdb
• EMDBエントリー: 14771
• 分子名称: Amyloid protein A (1 entity in total)
• 機能のキーワード: amyloids, vascular aa, saa, cryo-em, protein fibril
• 由来する生物種: Homo sapiens (human)
• タンパク質・核酸の鎖数: 12
• 化学式量合計: 93306.02

構造登録者

Banerjee, S.,Schmidt, M.,Faendrich, M. (登録日: 2022-04-13, 公開日: 2022-12-07, 最終更新日: 2024-07-24)

主引用文献

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

実験手法

ELECTRON MICROSCOPY (2.56 Å)