PXD057013
PXD057013 is an original dataset announced via ProteomeXchange.
Dataset Summary
| Title | Cryo-EM structure of a lysozyme-derived amyloid fibril from hereditary amyloidosis |
| Description | Systemic ALys amyloidosis is a debilitating protein misfolding diseases that arises from the formation of amyloid fibrils from C-type lysozyme. We here present a 2.8 A cryo-electron microscopy structure of an amyloid fibril, which was isolated from the abdominal fat tissue of a patient who expressed the D87G variant of human lysozyme. We find that the fibril possesses a stable core that is formed by all 130 residues of the fibril precursor protein. There are four disulfide bonds in each fibril protein that connect the same residues as in the globularly folded protein. However, the conformation is fundamentally different in the fibril and in native lysozyme, demonstrating the need of protein unfolding. The position of the mutant residue within the fibril structure implies that the role of this mutation includes the stabilization of the pathogenic fibril structure. |
| HostingRepository | MassIVE |
| AnnounceDate | 2024-10-21 |
| AnnouncementXML | Submission_2024-10-21_06:35:14.787.xml |
| DigitalObjectIdentifier | |
| ReviewLevel | Non peer-reviewed dataset |
| DatasetOrigin | Original dataset |
| RepositorySupport | Unsupported dataset by repository |
| PrimarySubmitter | Sebastian Wiese |
| SpeciesList | scientific name: Homo sapiens; common name: human; NCBI TaxID: 9606; |
| ModificationList | No PTMs are included in the dataset |
| Instrument | LTQ Orbitrap Elite |
Dataset History
| Revision | Datetime | Status | ChangeLog Entry |
|---|---|---|---|
| 0 | 2024-10-21 06:31:43 | ID requested | |
| ⏵ 1 | 2024-10-21 06:35:15 | announced |
Publication List
| no publication |
Keyword List
| submitter keyword: Intakt protein analysis, DatasetType:Other (Intakt Protein) |
Contact List
| Marcus Faendrich | |
|---|---|
| contact affiliation | Institute of protein biochemistry, Ulm University |
| contact email | marcus.faendrich@uni-ulm.de |
| lab head | |
| Sebastian Wiese | |
| contact affiliation | Ulm University |
| contact email | sebastian.wiese@uni-ulm.de |
| dataset submitter | |
Full Dataset Link List
| MassIVE dataset URI |
| Dataset FTP location NOTE: Most web browsers have now discontinued native support for FTP access within the browser window. But you can usually install another FTP app (we recommend FileZilla) and configure your browser to launch the external application when you click on this FTP link. Or otherwise, launch an app that supports FTP (like FileZilla) and use this address: ftp://massive.ucsd.edu/v08/MSV000096142/ |
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