PXD057013-1

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

no publication

Keyword List

submitter keyword: Intakt protein analysis, DatasetType:Other (Intakt Protein)

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
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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/