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Search: (WFRF:(Bauer Michael)) srt2:(2015-2019) > (2018) > Early postnatal beh...

  • Siebzehnrübl, Florian A.Cardiff University,University of Florida,Charité - University Medicine Berlin (author)

Early postnatal behavioral, cellular, and molecular changes in models of Huntington disease are reversible by HDAC inhibition

  • Article/chapterEnglish2018

Publisher, publication year, extent ...

  • 2018-08-27
  • Proceedings of the National Academy of Sciences,2018

Numbers

  • LIBRIS-ID:oai:lup.lub.lu.se:eaa7d714-14f9-42c1-b170-7b508884ec13
  • https://lup.lub.lu.se/record/eaa7d714-14f9-42c1-b170-7b508884ec13URI
  • https://doi.org/10.1073/pnas.1807962115DOI
  • http://kipublications.ki.se/Default.aspx?queryparsed=id:139133169URI

Supplementary language notes

  • Language:English
  • Summary in:English

Part of subdatabase

Classification

  • Subject category:art swepub-publicationtype
  • Subject category:ref swepub-contenttype

Notes

  • Huntington disease (HD) is an autosomal dominant neurodegenerative disorder caused by expanded CAG repeats in the huntingtin gene (HTT). Although mutant HTT is expressed during embryonic development and throughout life, clinical HD usually manifests later in adulthood. A number of studies document neurodevelopmental changes associated with mutant HTT, but whether these are reversible under therapy remains unclear. Here, we identify very early behavioral, molecular, and cellular changes in preweaning transgenic HD rats and mice. Reduced ultrasonic vocalization, loss of prepulse inhibition, and increased risk taking are accompanied by disturbances of dopaminergic regulation in vivo, reduced neuronal differentiation capacity in subventricular zone stem/progenitor cells, and impaired neuronal and oligodendrocyte differentiation of mouse embryo-derived neural stem cells in vitro. Interventional treatment of this early phenotype with the histone deacetylase inhibitor (HDACi) LBH589 led to significant improvement in behavioral changes and markers of dopaminergic neurotransmission and complete reversal of aberrant neuronal differentiation in vitro and in vivo. Our data support the notion that neurodevelopmental changes contribute to the prodromal phase of HD and that early, presymptomatic intervention using HDACi may represent a promising novel treatment approach for HD.

Subject headings and genre

Added entries (persons, corporate bodies, meetings, titles ...)

  • Raber, Kerstin A.Friedrich-Alexander University Erlangen-Nürnberg,Jülich Research Centre (author)
  • Urbach, Yvonne K.Friedrich-Alexander University Erlangen-Nürnberg (author)
  • Schulze-Krebs, AnjaFriedrich-Alexander University Erlangen-Nürnberg (author)
  • Canneva, FabioFriedrich-Alexander University Erlangen-Nürnberg (author)
  • Moceri, SandraFriedrich-Alexander University Erlangen-Nürnberg (author)
  • Habermeyer, JohannaFriedrich-Alexander University Erlangen-Nürnberg (author)
  • Achoui, DalilaUniversity of Florida (author)
  • Gupta, BhavanaCardiff University (author)
  • Steindler, Dennis A.Tufts University,University of Florida (author)
  • Stephan, MichaelHannover Medical School (author)
  • Nguyen, Huu PhucUniversity of Tübingen (author)
  • Bonin, MichaelUniversity of Tübingen (author)
  • Riess, OlafUniversity of Tübingen (author)
  • Bauer, AndreasJülich Research Centre (author)
  • Aigner, LudwigParacelsus Private Medical University of Salzburg (author)
  • Couillard-Despres, SebastienParacelsus Private Medical University of Salzburg (author)
  • Paucar, Martin ArceKarolinska Institute (author)
  • Svenningsson, PerKarolinska Institutet,Karolinska Institute (author)
  • Osmand, AlexanderUniversity of Tennessee (author)
  • Andreew, AlexanderCharité - University Medicine Berlin (author)
  • Zabel, ClausCharité - University Medicine Berlin (author)
  • Weiss, AndreasNovartis Pharma AG (author)
  • Kuhn, RainerNovartis Pharma AG (author)
  • Moussaoui, SalihaNovartis Pharma AG (author)
  • Blockx, InesUniversity of Antwerp (author)
  • Van der Linden, AnnemieUniversity of Antwerp (author)
  • Cheong, Rachel Y.Lund University,Lunds universitet,Translationell neuroendokrinologi,Forskargrupper vid Lunds universitet,Translational Neuroendocrinology,Lund University Research Groups(Swepub:lu)med-rco (author)
  • Roybon, LaurentLund University,Lunds universitet,Stamcellslaboratoriet för sjukdomsmodellering i det centrala nervsystemet,Forskargrupper vid Lunds universitet,IPSC Laboratory for CNS Disease Modeling,Lund University Research Groups(Swepub:lu)mphy-lra (author)
  • Petersén, ÅsaLund University,Lunds universitet,Translationell neuroendokrinologi,Forskargrupper vid Lunds universitet,Translational Neuroendocrinology,Lund University Research Groups(Swepub:lu)mphy-apn (author)
  • Von Hörsten, StephanFriedrich-Alexander University Erlangen-Nürnberg (author)
  • Cardiff UniversityUniversity of Florida (creator_code:org_t)

Related titles

  • In:Proceedings of the National Academy of Sciences of the United States of America: Proceedings of the National Academy of Sciences115:37, s. 8765-87740027-84241091-6490
  • In:Proceedings of the National Academy of Sciences: Proceedings of the National Academy of Sciences115:37, s. 8765-87741091-6490

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