Dataset Information
Amyloid fibril structure from the vascular variant of systemic AA amyloidosis
Ontology highlight
ABSTRACT:
SUBMITTER: Matthias Schmidt
PROVIDER: EMPIAR-11342 | biostudies-other |
REPOSITORIES: biostudies-other
Publications
Amyloid fibril structure from the vascular variant of systemic AA amyloidosis.
Nature communications 20221125 1
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 ...[more]