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PXD009594

PXD009594 is an original dataset announced via ProteomeXchange.

Dataset Summary
TitleLoss of the Mia40a oxidoreductase leads to hepato-pancreatic insufficiency in the zebrafish
DescriptionDevelopment and function of tissues and organs are powered by the activity of mitochondria. In humans, inherited genetic mutations that lead to progressive mitochondrial pathology often manifest during infancy and can lead to death, reflecting the indispensable nature of mitochondrial function and biogenesis. Here, we describe a zebrafish mutant for the gene mia40a, the life-essential homologue of the evolutionarily conserved Mia40 oxidoreductase which drives the biogenesis of cysteine-rich mitochondrial proteins. We report that mia40a mutant animals undergo progressive cellular respiration defects and develop enlarged mitochondria in skeletal muscles before their ultimate at the larval stage. We generated a rich transcriptomic and proteomic resource that allowed us to identify abnormalities in the development of endodermal organs, in particular the liver and pancreas. We identify the acinar cells of the exocrine pancreas to be severely affected by mutations in the MIA pathway. Our data contribute to a better understanding of the molecular, cellular and organismal effects of mitochondrial deficiency, important for the accurate diagnosis and future treatment strategies of these diseases.
HostingRepositoryPRIDE
AnnounceDate2018-10-10
AnnouncementXMLSubmission_2018-11-27_04:18:39.xml
DigitalObjectIdentifier
ReviewLevelPeer-reviewed dataset
DatasetOriginOriginal dataset
RepositorySupportUnsupported dataset by repository
PrimarySubmitterAnna Sokol
SpeciesList scientific name: Danio rerio (Zebrafish) (Brachydanio rerio); NCBI TaxID: 7955;
ModificationListmonohydroxylated residue; iodoacetamide derivatized residue
InstrumentQ Exactive
Dataset History
RevisionDatetimeStatusChangeLog Entry
02018-04-24 23:54:24ID requested
12018-10-10 07:17:50announced
22018-11-27 04:18:40announcedUpdated publication reference for PubMed record(s): 30457989.
Publication List
Sokol AM, Uszczynska-Ratajczak B, Collins MM, Bazala M, Topf U, Lundegaard PR, Sugunan S, Guenther S, Kuenne C, Graumann J, Chan SSL, Stainier DYR, Chacinska A, Loss of the Mia40a oxidoreductase leads to hepato-pancreatic insufficiency in zebrafish. PLoS Genet, 14(11):e1007743(2018) [pubmed]
Keyword List
curator keyword: Biological
submitter keyword: Biological, proteomics, dimethyl labeling (DML), Danio rerio, mitochondria, MIA pathway
Contact List
Agnieszka Chacinska
contact affiliationCentre of New Technologies, University of Warsaw, S. Banacha 2c, 02-097 Warsaw, Poland
contact emaila.chacinska@cent.uw.edu.pl
lab head
Anna Sokol
contact affiliationMax Planck Institute for Heart and Lung Research
contact emailanna.sokol@mpi-bn.mpg.de
dataset submitter
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Dataset FTP location
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