Mouse models of frontotemporal dementia

a comparison of phenotypes with clinical symptomatology

Rebekah M. Ahmed, Muireann Irish, Janet van Eersel, Arne Ittner, Yazi D. Ke, Alexander Volkerling, Julia van der Hoven, Kimi Tanaka, Tim Karl, Michael Kassiou, Jillian J. Kril, Olivier Piguet, Jürgen Götz, Matthew C. Kiernan, Glenda M. Halliday, John R. Hodges*, Lars M. Ittner

*Corresponding author for this work

Research output: Contribution to journalReview article

13 Citations (Scopus)

Abstract

Frontotemporal dementia (FTD) is the second most common cause of young onset dementia. It is increasingly recognized that there is a clinical continuum between FTD and amyotrophic lateral sclerosis (ALS). At a clinical, pathological and genetic level there is much heterogeneity in FTD, meaning that our understanding of this condition, pathophysiology and development of treatments has been limited. A number of mouse models focusing predominantly on recapitulating neuropathological and molecular changes of disease have been developed, with most transgenic lines expressing a single specific protein or genetic mutation. Together with the species-typical presentation of functional deficits, this makes the direct translation of results from these models to humans difficult. However, understanding the phenotypical presentations in mice and how they relate to clinical symptomology in humans is essential for advancing translation. Here we review current mouse models in FTD and compare their phenotype to the clinical presentation in patients.

Original languageEnglish
Pages (from-to)126-138
Number of pages13
JournalNeuroscience and Biobehavioral Reviews
Volume74
DOIs
Publication statusPublished - Mar 2017
Externally publishedYes

Keywords

  • frontotemporal dementia
  • transgenic
  • mouse
  • amyotrophic lateral sclerosis
  • behavioural neurology

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