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Maturation and phenotype of pathophysiological neuronal excitability of human cells in tau-related dementia

Kopach, Olga, Esteras, Noemí, Wray, Selina, Rusakov, Dmitri A., Abramov, Andrey Y.·2020·Journal of Cell Science
DOI 10.1242/jcs.241687
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Abstract

Frontotemporal dementia and parkinsonism (FTDP-17) caused by the 10+16 splice-site mutation in the gene encoding microtubule-associated protein tau (MAPT) provides an established platform to model tau-related dementia in vitro. Neurons derived from human induced pluripotent stem cells (iPSCs) have been shown to recapitulate the neurodevelopmental profile of tau pathology during in vitro corticogenesis, as in the adult human brain. However, the neurophysiological phenotype of these cells has remained unknown, leaving unanswered questions regarding the functional relevance and the gnostic power of this disease model. In this study, we used electrophysiology to explore the membrane properties and intrinsic excitability of the generated neurons and found that human cells mature by ∼150 days of neurogenesis to become compatible with matured cortical neurons. In earlier FTDP-17, however, neurons exhibited a depolarized resting membrane potential associated with increased resistance and reduced voltage-gated Na+- and K+-channel-mediated conductance. Expression of the Nav1.6 protein was reduced in FTDP-17. These effects led to reduced cell capability of induced firing and changed the action potential waveform in FTDP-17. The revealed neuropathology might thus contribute to the clinicopathological profile of the disease. This sheds new light on the significance of human in vitro models of dementia.

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Metadata

Authors
Kopach, O., Esteras, N., Wray, S., Rusakov, D. A., & Abramov, A. Y.
Affiliations
  • UCL Queen Square Institute of Neurology 1 Department of Clinical and Experimental Epilepsy , , London , WC1N 3BG, UK
  • UCL Queen Square Institute of Neurology 2 Department of Clinical and Movement Neurosciences , , London , WC1N 3BG, UK
  • UCL Queen Square Institute of Neurology 3 Department of Neurodegenerative Disease , , London , WC1N 1PJ, UK
ORCID
Kopach, Olga
Venue
Journal of Cell Science
Publisher
The Company of Biologists
Published
2020-05-15
Volume
133
Issue
10
ISSN
  • 1477-9137
  • 0021-9533
DOI
10.1242/jcs.241687
Cited by
29
References
3
Language
en
Licence
www.biologists.com/user-licence-1-1/ (vor)
Funders
  • Engineering and Physical Sciences Research Council (EP/R024898/1)
  • Wellcome Trust (212251_Z_18_Z)
  • European Commission (857562)

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