Astrocyte pathology in a human neural stem cell model of frontotemporal dementia caused by mutant TAU protein

Publikation: Beitrag in FachzeitschriftForschungsartikelBeigetragenBegutachtung

Beitragende

  • Anna Lena Hallmann - , Max Planck Institute for Molecular Biomedicine, Westfälische Wilhelms-Universität Münster (Autor:in)
  • Marcos J. Araúzo-Bravo - , Instituto de Investigación Sanitaria Biodonostia, Ikerbasque Basque Foundation for Science (Autor:in)
  • Lampros Mavrommatis - , Max Planck Institute for Molecular Biomedicine, Ruhr-Universität Bochum (Autor:in)
  • Marc Ehrlich - , Max Planck Institute for Molecular Biomedicine, Westfälische Wilhelms-Universität Münster (Autor:in)
  • Albrecht Röpke - , Westfälische Wilhelms-Universität Münster (Autor:in)
  • Johannes Brockhaus - , Westfälische Wilhelms-Universität Münster (Autor:in)
  • Markus Missler - , Westfälische Wilhelms-Universität Münster (Autor:in)
  • Jared Sterneckert - , IPS Zellen und neurodegenerative Erkrankungen (NFoG) (Autor:in)
  • Hans R. Schöler - , Max Planck Institute for Molecular Biomedicine, Westfälische Wilhelms-Universität Münster (Autor:in)
  • Tanja Kuhlmann - , Westfälische Wilhelms-Universität Münster (Autor:in)
  • Holm Zaehres - , Max Planck Institute for Molecular Biomedicine, Ruhr-Universität Bochum (Autor:in)
  • Gunnar Hargus - , Max Planck Institute for Molecular Biomedicine, Westfälische Wilhelms-Universität Münster, Columbia University (Autor:in)

Abstract

Astroglial pathology is seen in various neurodegenerative diseases including frontotemporal dementia (FTD), which can be caused by mutations in the gene encoding the microtubule-associated protein TAU (MAPT). Here, we applied a stem cell model of FTD to examine if FTD astrocytes carry an intrinsic propensity to degeneration and to determine if they can induce non-cell-autonomous effects in neighboring neurons. We utilized CRISPR/Cas9 genome editing in human induced pluripotent stem (iPS) cell-derived neural progenitor cells (NPCs) to repair the FTD-associated N279K MAPT mutation. While astrocytic differentiation was not impaired in FTD NPCs derived from one patient carrying the N279K MAPT mutation, FTD astrocytes appeared larger, expressed increased levels of 4R-TAU isoforms, demonstrated increased vulnerability to oxidative stress and elevated protein ubiquitination and exhibited disease-associated changes in transcriptome profiles when compared to astrocytes derived from one control individual and to the isogenic control. Interestingly, co-culture experiments with FTD astrocytes revealed increased oxidative stress and robust changes in whole genome expression in previously healthy neurons. Our study highlights the utility of iPS cell-derived NPCs to elucidate the role of astrocytes in the pathogenesis of FTD.

Details

OriginalspracheEnglisch
Aufsatznummer42991
FachzeitschriftScientific reports
Jahrgang7
PublikationsstatusVeröffentlicht - 3 März 2017
Peer-Review-StatusJa

Externe IDs

PubMed 28256506
ORCID /0000-0002-7688-3124/work/142250040

Schlagworte

Ziele für nachhaltige Entwicklung

ASJC Scopus Sachgebiete

Bibliotheksschlagworte