ATTRV122I amyloid fibrils from hereditary ATTR amloidosis
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ABSTRACT:
SUBMITTER: Matthias Schmidt
PROVIDER: EMPIAR-11704 | biostudies-other |
REPOSITORIES: biostudies-other
Publications
Common transthyretin-derived amyloid fibril structures in patients with hereditary ATTR amyloidosis.
Steinebrei Maximilian M Baur Julian J Pradhan Anaviggha A Kupfer Niklas N Wiese Sebastian S Hegenbart Ute U Schönland Stefan O SO Schmidt Matthias M Fändrich Marcus M
Nature communications 20231122 1
Systemic ATTR amyloidosis is an increasingly important protein misfolding disease that is provoked by the formation of amyloid fibrils from transthyretin protein. The pathological and clinical disease manifestations and the number of pathogenic mutational changes in transthyretin are highly diverse, raising the question whether the different mutations may lead to different fibril morphologies. Using cryo-electron microscopy, however, we show here that the fibril structure is remarkably similar i ...[more]
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