PXD025595 is an
original dataset announced via ProteomeXchange.
Dataset Summary
Title | The biochemical basis of mitochondrial dysfunctionin Zellweger Spectrum Disorder |
Description | Peroxisomal Biogenesis Disorders (PBDs) are genetic disorders of peroxisome biogenesis and metabolism that are characterized by profound developmental and neurological phenotypes. The most severe class of PBDs—Zellweger Spectrum Disorder(ZSD)—is caused by mutations in peroxin genes that result in both non-functional peroxisomes and mitochondrial dysfunction. It is unclear, however, how defective peroxisomes contribute to mitochondrial impairment. In order to understand the molecular basis of this inter-organellar relationship, we investigated the fate of peroxisomal mRNAs and proteins in ZSD model systems. We found that peroxins were still expressed and a subset of them accumulated on the mitochondrial membrane, which resulted in gross mitochondrial abnormalities and impaired mitochondrial metabolic function. We showed that overexpression of ATAD1, a mitochondrial quality control factor, was sufficient to rescue several aspects of mitochondrial function in human ZSD fibroblasts. Together, these data suggest that aberrant peroxisomal protein localization is necessary and sufficient for the devastating mitochondrial morphological and metabolic phenotypes in ZSDs. |
HostingRepository | PRIDE |
AnnounceDate | 2021-05-11 |
AnnouncementXML | Submission_2021-05-10_21:50:45.409.xml |
DigitalObjectIdentifier | |
ReviewLevel | Peer-reviewed dataset |
DatasetOrigin | Original dataset |
RepositorySupport | Unsupported dataset by repository |
PrimarySubmitter | Joao Paulo |
SpeciesList | scientific name: Saccharomyces cerevisiae (Baker's yeast); NCBI TaxID: 4932; |
ModificationList | TMT6plex-126 reporter+balance reagent acylated residue; monohydroxylated residue; iodoacetamide derivatized residue |
Instrument | Orbitrap Fusion Lumos |
Dataset History
Revision | Datetime | Status | ChangeLog Entry |
0 | 2021-04-23 06:21:00 | ID requested | |
⏵ 1 | 2021-05-10 21:50:45 | announced | |
Publication List
Dataset with its publication pending |
Keyword List
submitter keyword: Peroxisomal Biogenesis Disorders, Zellweger Spectrum Disorder, mitochondria, LC-MS/Ms |
Contact List
Jared Rutter |
contact affiliation | Jared Rutter University of Utah Salt Lake City, UT, 84112 |
contact email | rutter@biochem.utah.edu |
lab head | |
Joao Paulo |
contact affiliation | Harvard Medical School |
contact email | joao_paulo@post.harvard.edu |
dataset submitter | |
Full Dataset Link List
Dataset FTP location
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PRIDE project URI |
Repository Record List
[ + ]
[ - ]
- PRIDE
- PXD025595
- Label: PRIDE project
- Name: The biochemical basis of mitochondrial dysfunctionin Zellweger Spectrum Disorder