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PXD025595

PXD025595 is an original dataset announced via ProteomeXchange.

Dataset Summary
TitleThe biochemical basis of mitochondrial dysfunctionin Zellweger Spectrum Disorder
DescriptionPeroxisomal 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.
HostingRepositoryPRIDE
AnnounceDate2021-05-11
AnnouncementXMLSubmission_2021-05-10_21:50:45.409.xml
DigitalObjectIdentifier
ReviewLevelPeer-reviewed dataset
DatasetOriginOriginal dataset
RepositorySupportUnsupported dataset by repository
PrimarySubmitterJoao Paulo
SpeciesList scientific name: Saccharomyces cerevisiae (Baker's yeast); NCBI TaxID: 4932;
ModificationListTMT6plex-126 reporter+balance reagent acylated residue; monohydroxylated residue; iodoacetamide derivatized residue
InstrumentOrbitrap Fusion Lumos
Dataset History
RevisionDatetimeStatusChangeLog Entry
02021-04-23 06:21:00ID requested
12021-05-10 21:50:45announced
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 affiliationJared Rutter University of Utah Salt Lake City, UT, 84112
contact emailrutter@biochem.utah.edu
lab head
Joao Paulo
contact affiliationHarvard Medical School
contact emailjoao_paulo@post.harvard.edu
dataset submitter
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Dataset FTP location
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