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DataSet Summary

  • HostingRepository: PRIDE
  • AnnounceDate: 2019-01-11
  • AnnouncementXML: Submission_2019-01-11_05:02:37.xml
  • DigitalObjectIdentifier:
  • ReviewLevel: Peer-reviewed dataset
  • DatasetOrigin: Original data
  • RepositorySupport: Unsupported dataset by repository
  • PrimarySubmitter: Niels Henning Skotte
  • Title: Integrative Characterization of the R6/2 Mouse Brain Model of Huntington Disease Reveals Dysfunctional Neurotransmitter Metabolism
  • Description: Huntington disease (HD) is a fatal neurodegenerative disease, where dysfunction and loss of striatal and cortical neurons are central to the pathogenesis of the disease. Here, we integrated quantitative studies to investigate the underlying mechanisms behind HD pathology in a systems-wide manner. To this end, we used state-of-the-art mass spectrometry (MS) to establish a spatial brain proteome from late-stage R6/2 mice and compared this to wild-type (WT) counterparts. From the quantitative analysis, we observed altered expression of proteins in pathways related to energy metabolism, synapse function, and neurotransmitter homeostasis. To support these findings, metabolic 13C labelling studies confirmed a compromised astrocytic regulation of glutamate-GABA-glutamine cycling resulting in impaired release of glutamine and GABA synthesis. In recent years increasing attention has been focused on the role of astrocytes in HD, and our data supports that therapeutic strategies to improve astrocytic glutamine metabolism may help ameliorate symptoms in HD.
  • SpeciesList: scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
  • ModificationList: No PTMs are included in the dataset
  • Instrument: Q Exactive

Dataset History

VersionDatetimeStatusChangeLog Entry
02017-11-01 02:27:27ID requested
12019-01-11 05:02:38announced

Publication List

  1. Skotte NH, Andersen JV, Santos A, Aldana BI, Willert CW, Nørremølle A, Waagepetersen HS, Nielsen ML, Integrative Characterization of the R6/2 Mouse Model of Huntington's Disease Reveals Dysfunctional Astrocyte Metabolism. Cell Rep, 23(7):2211-2224(2018) [pubmed]

Keyword List

  1. curator keyword: Biomedical
  2. submitter keyword: High resolution LC-MS, GS-MS, Proteome, Huntington disease, glutamine and glutamate metabolism, Glutamatergic and GABAergic neurons

Contact List

    Michael L. Nielsen
    • contact affiliation: Faculty of Health Sciences, Department of Proteomics, The Novo Nordisk Foundation Centre for Protein Research, University of Copenhagen, DK-2200 Copenhagen, Denmark
    • contact email: michael.lund.nielsen@cpr.ku.dk
    • lab head:
    Niels Henning Skotte
    • contact affiliation: University of Copenhagen
    • contact email: niels.skotte@cpr.ku.dk
    • dataset submitter:

Full Dataset Link List

  1. Dataset FTP location
  2. PRIDE project URI
Repository Record List

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