| |  Online-Ressource |
|---|
| Verfasst von: | Radamaker, Lynn [VerfasserIn]  |
|---|
| | Baur, Julian [VerfasserIn] |
|---|
| | Huhn, Stefanie [VerfasserIn] |
|---|
| | Haupt, Christian [VerfasserIn] |
|---|
| | Hegenbart, Ute [VerfasserIn] |
|---|
| | Schönland, Stefan [VerfasserIn] |
|---|
| | Bansal, Akanksha [VerfasserIn] |
|---|
| | Schmidt, Matthias [VerfasserIn] |
|---|
| | Fändrich, Marcus [VerfasserIn] |
|---|
| Titel: | Cryo-EM reveals structural breaks in a patient-derived amyloid fibril from systemic AL amyloidosis |
|---|
| Verf.angabe: | Lynn Radamaker, Julian Baur, Stefanie Huhn, Christian Haupt, Ute Hegenbart, Stefan Schönland, Akanksha Bansal, Matthias Schmidt & Marcus Fändrich |
|---|
| E-Jahr: | 2021 |
|---|
| Jahr: | 08 February 2021 |
|---|
| Umfang: | 10 S. |
|---|
| Fussnoten: | Gesehen am 06.08.2021 |
|---|
| Titel Quelle: | Enthalten in: Nature Communications |
|---|
| Ort Quelle: | [London] : Nature Publishing Group UK, 2010 |
|---|
| Jahr Quelle: | 2021 |
|---|
| Band/Heft Quelle: | 12(2021), Artikel-ID 875, Seite 1-10 |
|---|
| ISSN Quelle: | 2041-1723 |
|---|
| Abstract: | Systemic AL amyloidosis is a debilitating and potentially fatal disease that arises from the misfolding and fibrillation of immunoglobulin light chains (LCs). The disease is patient-specific with essentially each patient possessing a unique LC sequence. In this study, we present two ex vivo fibril structures of a λ3 LC. The fibrils were extracted from the explanted heart of a patient (FOR005) and consist of 115-residue fibril proteins, mainly from the LC variable domain. The fibril structures imply that a 180° rotation around the disulfide bond and a major unfolding step are necessary for fibrils to form. The two fibril structures show highly similar fibril protein folds, differing in only a 12-residue segment. Remarkably, the two structures do not represent separate fibril morphologies, as they can co-exist at different z-axial positions within the same fibril. Our data imply the presence of structural breaks at the interface of the two structural forms. |
|---|
| DOI: | doi:10.1038/s41467-021-21126-2 |
|---|
| URL: | Bitte beachten Sie: Dies ist ein Bibliographieeintrag. Ein Volltextzugriff für Mitglieder der Universität besteht hier nur, falls für die entsprechende Zeitschrift/den entsprechenden Sammelband ein Abonnement besteht oder es sich um einen OpenAccess-Titel handelt.
Volltext: https://doi.org/10.1038/s41467-021-21126-2 |
|---|
| | Volltext: https://www.nature.com/articles/s41467-021-21126-2 |
|---|
| | DOI: https://doi.org/10.1038/s41467-021-21126-2 |
|---|
| Datenträger: | Online-Ressource |
|---|
| Sprache: | eng |
|---|
| K10plus-PPN: | 1755770723 |
|---|
| Verknüpfungen: | → Zeitschrift |
|---|
Cryo-EM reveals structural breaks in a patient-derived amyloid fibril from systemic AL amyloidosis / Radamaker, Lynn [VerfasserIn]; 08 February 2021 (Online-Ressource)
