PXD029876
PXD029876 is an original dataset announced via ProteomeXchange.
Dataset Summary
| Title | Amyloid fibrils in disease FTLD-TDP are composed of TMEM106B, rather than TDP-43 |
| Description | FTLD is the third most common neurodegenerative condition, following only Alzheimer's and Parkinson's diseases. FTLD typically presents in 45-64-year-olds with behavioral changes or progressive decline of language skills. The subtype FTLD-TDP is characterized by certain clinical symptoms and pathological neuronal inclusions detected by TDP-43 immunoreactivity. Here, we extracted amyloid fibrils from brains of four patients, representing four out of five FTLD-TDP subclasses and determined their near-atomic resolution structures by cryo-EM. Unexpectedly, all amyloid fibrils examined are composed of a 135-residue C-terminal fragment of TMEM106B, a lysosomal membrane protein previously implicated as a genetic risk factor for FTLD-TDP. In addition to TMEM106B fibrils, abundant non-fibrillar aggregated TDP-43 is present, as revealed by immunogold labeling. Our observations confirm that FTLD-TDP is an amyloid-involved disease and suggest that amyloid involvement in FTLD-TDP is of protein TMEM106B, rather than of TDP-43. |
| HostingRepository | MassIVE |
| AnnounceDate | 2022-02-26 |
| AnnouncementXML | Submission_2022-02-26_17:01:10.226.xml |
| DigitalObjectIdentifier | |
| ReviewLevel | Non peer-reviewed dataset |
| DatasetOrigin | Original dataset |
| RepositorySupport | Supported dataset by repository |
| PrimarySubmitter | Janine Fu |
| SpeciesList | scientific name: Homo sapiens; common name: human; NCBI TaxID: 9606; |
| ModificationList | Oxidation; Carbamidomethyl |
| Instrument | Orbitrap Exploris 480 |
Dataset History
| Revision | Datetime | Status | ChangeLog Entry |
|---|---|---|---|
| 0 | 2021-11-22 20:17:19 | ID requested | |
| ⏵ 1 | 2022-02-26 17:01:10 | announced |
Publication List
| no publication |
Keyword List
| submitter keyword: TMEM106B, amyloid, fibril, FTLD-TDP |
Contact List
| Joseph Loo | |
|---|---|
| contact affiliation | University of California Los Angeles |
| contact email | jloo@chem.ucla.edu |
| lab head | |
| Janine Fu | |
| contact affiliation | University of California, Los Angeles |
| contact email | janinefu@chem.ucla.edu |
| 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/MSV000088430/ |
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