Article
Role of mutations and post-translational modifications in systemic AL amyloidosis studied by cryo-EM.
Nature communications - 5 Nov 2021
Radamaker Lynn, Karimi-Farsijani Sara, Andreotti Giada, Baur Julian, Neumann Matthias, Schreiner Sarah, Berghaus Natalie, Motika Raoul, Haupt Christian, Walther Paul, Schmidt Volker, Huhn Stefanie, Hegenbart Ute, Schönland Stefan O, Wiese Sebastian, Read Clarissa, Schmidt Matthias, Fändrich Marcus
Abstract excerpt
Systemic AL amyloidosis is a rare disease that is caused by the misfolding of immunoglobulin light chains (LCs). Potential drivers of amyloid formation in this disease are post-translational modifications (PTMs) and the mutational changes that are inserted into the LCs by somatic hypermutation. Here we present the cryo electron microscopy (cryo-EM) structure of an ex vivo λ1-AL amyloid fibril whose deposits...
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