Mouse models of frontotemporal dementia: a comparison of phenotypes with clinical symptomatology

Ahmed, Rebekah M., Irish, Muireann, van Eersel, Janet, Ittner, Arne, Ke, Yazi D., Volkerling, Alexander, van der Hoven, Julia, Tanaka, Kimi, Karl, Tim, Kassiou, Michael, Kril, Jillian J., Piguet, Olivier, Goetz, Juergen, Kiernan, Matthew C., Halliday, Glenda M., Hodges, John R. and Ittner, Lars M. (2017) Mouse models of frontotemporal dementia: a comparison of phenotypes with clinical symptomatology. Neuroscience and Biobehavioral Reviews, 74 A: 126-138. doi:10.1016/j.neubiorev.2017.01.004


Author Ahmed, Rebekah M.
Irish, Muireann
van Eersel, Janet
Ittner, Arne
Ke, Yazi D.
Volkerling, Alexander
van der Hoven, Julia
Tanaka, Kimi
Karl, Tim
Kassiou, Michael
Kril, Jillian J.
Piguet, Olivier
Goetz, Juergen
Kiernan, Matthew C.
Halliday, Glenda M.
Hodges, John R.
Ittner, Lars M.
Title Mouse models of frontotemporal dementia: a comparison of phenotypes with clinical symptomatology
Journal name Neuroscience and Biobehavioral Reviews   Check publisher's open access policy
ISSN 1873-7528
0149-7634
Publication date 2017-03-01
Year available 2017
Sub-type Critical review of research, literature review, critical commentary
DOI 10.1016/j.neubiorev.2017.01.004
Open Access Status Not yet assessed
Volume 74
Issue A
Start page 126
End page 138
Total pages 13
Place of publication Kidlington, Oxford, United Kingdom
Publisher Pergamon Press
Language eng
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.
Keyword Amyotrophic lateral sclerosis
Behavioural neurology
Frontotemporal dementia
Mouse
Transgenic
Q-Index Code C1
Q-Index Status Provisional Code
Grant ID 1037746
CE110001021
1003139
Institutional Status UQ

Document type: Journal Article
Sub-type: Critical review of research, literature review, critical commentary
Collections: HERDC Pre-Audit
Queensland Brain Institute Publications
 
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