Cryo-EM reveals structural breaks in a patient-derived amyloid fibril from systemic AL amyloidosis
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 s...
Gespeichert in:
| Hauptverfasser: | , , , , , , , , |
|---|---|
| Dokumenttyp: | Article (Journal) |
| Sprache: | Englisch |
| Veröffentlicht: |
08 February 2021
|
| In: |
Nature Communications
Year: 2021, Jahrgang: 12, Pages: 1-10 |
| ISSN: | 2041-1723 |
| DOI: | 10.1038/s41467-021-21126-2 |
| Online-Zugang: | Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1038/s41467-021-21126-2 Verlag, lizenzpflichtig, Volltext: https://www.nature.com/articles/s41467-021-21126-2 
|
| Verfasserangaben: | Lynn Radamaker, Julian Baur, Stefanie Huhn, Christian Haupt, Ute Hegenbart, Stefan Schönland, Akanksha Bansal, Matthias Schmidt & Marcus Fändrich |
MARC
| LEADER | 00000caa a2200000 c 4500 | ||
|---|---|---|---|
| 001 | 1755770723 | ||
| 003 | DE-627 | ||
| 005 | 20240413193329.0 | ||
| 007 | cr uuu---uuuuu | ||
| 008 | 210423s2021 xx |||||o 00| ||eng c | ||
| 024 | 7 | |a 10.1038/s41467-021-21126-2 |2 doi | |
| 035 | |a (DE-627)1755770723 | ||
| 035 | |a (DE-599)KXP1755770723 | ||
| 035 | |a (OCoLC)1341405762 | ||
| 040 | |a DE-627 |b ger |c DE-627 |e rda | ||
| 041 | |a eng | ||
| 084 | |a 33 |2 sdnb | ||
| 100 | 1 | |a Radamaker, Lynn |e VerfasserIn |0 (DE-588)1188484915 |0 (DE-627)1667454447 |4 aut | |
| 245 | 1 | 0 | |a Cryo-EM reveals structural breaks in a patient-derived amyloid fibril from systemic AL amyloidosis |c Lynn Radamaker, Julian Baur, Stefanie Huhn, Christian Haupt, Ute Hegenbart, Stefan Schönland, Akanksha Bansal, Matthias Schmidt & Marcus Fändrich |
| 264 | 1 | |c 08 February 2021 | |
| 300 | |a 10 | ||
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a Computermedien |b c |2 rdamedia | ||
| 338 | |a Online-Ressource |b cr |2 rdacarrier | ||
| 500 | |a Gesehen am 06.08.2021 | ||
| 520 | |a 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. | ||
| 700 | 1 | |a Baur, Julian |e VerfasserIn |4 aut | |
| 700 | 1 | |a Huhn, Stefanie |d 1982- |e VerfasserIn |0 (DE-588)1014984424 |0 (DE-627)705313190 |0 (DE-576)349876436 |4 aut | |
| 700 | 1 | |a Haupt, Christian |e VerfasserIn |4 aut | |
| 700 | 1 | |a Hegenbart, Ute |e VerfasserIn |0 (DE-588)1028373708 |0 (DE-627)73062157X |0 (DE-576)35901903X |4 aut | |
| 700 | 1 | |a Schönland, Stefan |d 1969- |e VerfasserIn |0 (DE-588)122405226 |0 (DE-627)705896137 |0 (DE-576)293255792 |4 aut | |
| 700 | 1 | |a Bansal, Akanksha |e VerfasserIn |4 aut | |
| 700 | 1 | |a Schmidt, Matthias |e VerfasserIn |4 aut | |
| 700 | 1 | |a Fändrich, Marcus |e VerfasserIn |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |t Nature Communications |d [London] : Springer Nature, 2010 |g 12(2021), Artikel-ID 875, Seite 1-10 |h Online-Ressource |w (DE-627)626457688 |w (DE-600)2553671-0 |w (DE-576)331555905 |x 2041-1723 |7 nnas |a Cryo-EM reveals structural breaks in a patient-derived amyloid fibril from systemic AL amyloidosis |
| 773 | 1 | 8 | |g volume:12 |g year:2021 |g elocationid:875 |g pages:1-10 |g extent:10 |a Cryo-EM reveals structural breaks in a patient-derived amyloid fibril from systemic AL amyloidosis |
| 856 | 4 | 0 | |u https://doi.org/10.1038/s41467-021-21126-2 |x Verlag |x Resolving-System |z lizenzpflichtig |3 Volltext |
| 856 | 4 | 0 | |u https://www.nature.com/articles/s41467-021-21126-2 |x Verlag |z lizenzpflichtig |3 Volltext |
| 951 | |a AR | ||
| 992 | |a 20210423 | ||
| 993 | |a Article | ||
| 994 | |a 2021 | ||
| 998 | |g 122405226 |a Schönland, Stefan |m 122405226:Schönland, Stefan |d 910000 |d 910100 |d 50000 |e 910000PS122405226 |e 910100PS122405226 |e 50000PS122405226 |k 0/910000/ |k 1/910000/910100/ |k 0/50000/ |p 6 | ||
| 998 | |g 1028373708 |a Hegenbart, Ute |m 1028373708:Hegenbart, Ute |d 910000 |d 910100 |d 50000 |e 910000PH1028373708 |e 910100PH1028373708 |e 50000PH1028373708 |k 0/910000/ |k 1/910000/910100/ |k 0/50000/ |p 5 | ||
| 998 | |g 1014984424 |a Huhn, Stefanie |m 1014984424:Huhn, Stefanie |d 910000 |d 910100 |e 910000PH1014984424 |e 910100PH1014984424 |k 0/910000/ |k 1/910000/910100/ |p 3 | ||
| 999 | |a KXP-PPN1755770723 |e 3914519061 | ||
| BIB | |a Y | ||
| SER | |a journal | ||
| JSO | |a {"id":{"doi":["10.1038/s41467-021-21126-2"],"eki":["1755770723"]},"relHost":[{"part":{"pages":"1-10","year":"2021","volume":"12","text":"12(2021), Artikel-ID 875, Seite 1-10","extent":"10"},"language":["eng"],"id":{"zdb":["2553671-0"],"issn":["2041-1723"],"eki":["626457688"]},"physDesc":[{"extent":"Online-Ressource"}],"recId":"626457688","type":{"media":"Online-Ressource","bibl":"periodical"},"disp":"Cryo-EM reveals structural breaks in a patient-derived amyloid fibril from systemic AL amyloidosisNature Communications","title":[{"title":"Nature Communications","title_sort":"Nature Communications"}],"origin":[{"publisherPlace":"[London] ; [London]","publisher":"Springer Nature ; Nature Publishing Group UK","dateIssuedDisp":"[2010]-"}],"note":["Gesehen am 13.06.24"],"pubHistory":["2010-"]}],"recId":"1755770723","name":{"displayForm":["Lynn Radamaker, Julian Baur, Stefanie Huhn, Christian Haupt, Ute Hegenbart, Stefan Schönland, Akanksha Bansal, Matthias Schmidt & Marcus Fändrich"]},"physDesc":[{"extent":"10 S."}],"type":{"media":"Online-Ressource","bibl":"article-journal"},"origin":[{"dateIssuedKey":"2021","dateIssuedDisp":"08 February 2021"}],"note":["Gesehen am 06.08.2021"],"title":[{"title":"Cryo-EM reveals structural breaks in a patient-derived amyloid fibril from systemic AL amyloidosis","title_sort":"Cryo-EM reveals structural breaks in a patient-derived amyloid fibril from systemic AL amyloidosis"}],"language":["eng"],"person":[{"role":"aut","given":"Lynn","family":"Radamaker","display":"Radamaker, Lynn"},{"display":"Baur, Julian","family":"Baur","given":"Julian","role":"aut"},{"display":"Huhn, Stefanie","family":"Huhn","given":"Stefanie","role":"aut"},{"family":"Haupt","display":"Haupt, Christian","given":"Christian","role":"aut"},{"display":"Hegenbart, Ute","family":"Hegenbart","role":"aut","given":"Ute"},{"role":"aut","given":"Stefan","display":"Schönland, Stefan","family":"Schönland"},{"role":"aut","given":"Akanksha","family":"Bansal","display":"Bansal, Akanksha"},{"display":"Schmidt, Matthias","family":"Schmidt","given":"Matthias","role":"aut"},{"family":"Fändrich","display":"Fändrich, Marcus","given":"Marcus","role":"aut"}]} | ||
| SRT | |a RADAMAKERLCRYOEMREVE0820 | ||