PXD045329
PXD045329 is an original dataset announced via ProteomeXchange.
Dataset Summary
| Title | Ribosome Profiling and Mass Spectrometry Reveal Widespread Mitochondrial Translation Defects in a Striatal Cell Model of Huntington Disease |
| Description | Huntington disease (HD) is caused by an expanded polyglutamine mutation in huntingtin (mHTT) that promotes prominent atrophy in the striatum and subsequent psychiatric, cognitive, and choreiform movements. Multiple lines of evidence point to an association between HD and aberrant striatal mitochondrial functions; however, the present knowledge about whether (or how) mitochondrial mRNA translation is differentially regulated in HD remains unclear. We found that protein synthesis is diminished in HD mitochondria compared to healthy control striatal cell models. We utilized ribosome profiling (Ribo Seq) to analyze detailed snapshots of ribosome occupancy of the mitochondrial mRNA transcripts in control and HD striatal cell models. The Ribo-Seq data revealed almost unaltered ribosome occupancy on the nuclear encoded mitochondrial transcripts involved in oxidative phosphorylation (OXPHOS) (SDHA, Ndufv1, Timm23, Tomm5, Mrps22) in HD cells. By contrast, ribosome occupancy was dramatically increased for mitochondrially encoded OXPHOS mRNAs (mtNd-1, mtNd-2, mtNd-4, mtNd-4l, mtNd-5, mtNd-6, mt-Co1, mtCyt b, and mt-ATP8). We also applied tandem mass tag based mass spectrometry identification of mitochondrial proteins to derive correlations between ribosome occupancy and actual mature mitochondrial protein products. We found many mitochondrial transcripts with comparable or higher ribosome occupancy, but diminished mitochondrial protein products, in HD. Thus, our study provides the first evidence of a widespread dichotomous effect on ribosome occupancy and protein turnover of mitochondria related genes in HD. |
| HostingRepository | MassIVE |
| AnnounceDate | 2024-04-01 |
| AnnouncementXML | Submission_2024-04-01_11:04:55.170.xml |
| DigitalObjectIdentifier | |
| ReviewLevel | Non peer-reviewed dataset |
| DatasetOrigin | Original dataset |
| RepositorySupport | Supported dataset by repository |
| PrimarySubmitter | Gogce Ceren Crynen |
| SpeciesList | scientific name: Mus musculus; common name: house mouse; NCBI TaxID: 10090; |
| ModificationList | Deamidated; TMTpro; L-cysteine methyl disulfide |
| Instrument | Orbitrap Fusion |
Dataset History
| Revision | Datetime | Status | ChangeLog Entry |
|---|---|---|---|
| 0 | 2023-09-12 14:36:45 | ID requested | |
| ⏵ 1 | 2024-04-01 11:04:55 | announced |
Publication List
| no publication |
Keyword List
| submitter keyword: Dichotomy, energy metabolism, TMT, brain disease, vulnerability, oxidative stress, mitoribosome, cytoribosome, mRNA translation |
Contact List
| Srinivasa Subramaniam | |
|---|---|
| contact affiliation | The Herbert Wertheim UF Scripps Institute for Biomedical Innovation & Technology |
| contact email | subramaniams@ufl.edu |
| lab head | |
| Gogce Ceren Crynen | |
| contact affiliation | UF Scripps Biomedical Research |
| contact email | gogce@ufl.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/v06/MSV000092843/ |
to receive all new ProteomeXchange dataset release announcements!
