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DataSet Summary

  • HostingRepository: PRIDE
  • AnnounceDate: 2018-10-10
  • AnnouncementXML: Submission_2018-11-27_04:18:39.xml
  • DigitalObjectIdentifier:
  • ReviewLevel: Peer-reviewed dataset
  • DatasetOrigin: Original data
  • RepositorySupport: Unsupported dataset by repository
  • PrimarySubmitter: Anna Sokol
  • Title: Loss of the Mia40a oxidoreductase leads to hepato-pancreatic insufficiency in the zebrafish
  • Description: Development 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.
  • SpeciesList: scientific name: Danio rerio (Zebrafish) (Brachydanio rerio); NCBI TaxID: 7955;
  • ModificationList: monohydroxylated residue; iodoacetamide derivatized residue
  • Instrument: Q Exactive

Dataset History

VersionDatetimeStatusChangeLog 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

  1. 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

  1. curator keyword: Biological
  2. submitter keyword: Biological, proteomics, dimethyl labeling (DML), Danio rerio, mitochondria, MIA pathway

Contact List

    Agnieszka Chacinska
    • contact affiliation: Centre of New Technologies, University of Warsaw, S. Banacha 2c, 02-097 Warsaw, Poland
    • contact email: a.chacinska@cent.uw.edu.pl
    • lab head:
    Anna Sokol
    • contact affiliation: Max Planck Institute for Heart and Lung Research
    • contact email: anna.sokol@mpi-bn.mpg.de
    • dataset submitter:

Full Dataset Link List

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