title: Mouse models of frontotemporal dementia: A comparison of phenotypes with clinical symptomatology.
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.
authors.affiliation: Neuroscience Research Australia, Sydney, Australia; ARC Centre of Excellence in Cognition and its Disorders, the University of New South Wales, Sydney, Australia; Sydney Medical School, Brain & Mind Centre, University of Sydney, Australia.
authors.forename: Rebekah M
authors.initials: RM
authors.lastname: Ahmed
authors.affiliation: Neuroscience Research Australia, Sydney, Australia; ARC Centre of Excellence in Cognition and its Disorders, the University of New South Wales, Sydney, Australia; School of Psychology, the University of New South Wales, Sydney, Australia; Brain and Mind Centre, University of Sydney, Australia.
authors.forename: Muireann
authors.initials: M
authors.lastname: Irish
authors.affiliation: Dementia Research Unit, Department of Anatomy, School of Medical Sciences, Faculty of Medicine, University of New South Wales, Sydney, Australia.
authors.forename: Janet
authors.initials: J
authors.lastname: van Eersel
authors.affiliation: Dementia Research Unit, Department of Anatomy, School of Medical Sciences, Faculty of Medicine, University of New South Wales, Sydney, Australia.
authors.forename: Arne
authors.initials: A
authors.lastname: Ittner
authors.affiliation: Dementia Research Unit, Department of Anatomy, School of Medical Sciences, Faculty of Medicine, University of New South Wales, Sydney, Australia.
authors.forename: Yazi D
authors.initials: YD
authors.lastname: Ke
authors.affiliation: Dementia Research Unit, Department of Anatomy, School of Medical Sciences, Faculty of Medicine, University of New South Wales, Sydney, Australia.
authors.forename: Alexander
authors.initials: A
authors.lastname: Volkerling
authors.affiliation: Dementia Research Unit, Department of Anatomy, School of Medical Sciences, Faculty of Medicine, University of New South Wales, Sydney, Australia.
authors.forename: Julia
authors.initials: J
authors.lastname: van der Hoven
authors.affiliation: Dementia Research Unit, Department of Anatomy, School of Medical Sciences, Faculty of Medicine, University of New South Wales, Sydney, Australia.
authors.forename: Kimi
authors.initials: K
authors.lastname: Tanaka
authors.affiliation: Neuroscience Research Australia, Sydney, Australia.
authors.forename: Tim
authors.initials: T
authors.lastname: Karl
authors.affiliation: Faculty of Health Sciences, The University of Sydney, Australia.
authors.forename: Michael
authors.initials: M
authors.lastname: Kassiou
authors.affiliation: Discipline of Pathology, Sydney Medical School, The University of Sydney, Australia.
authors.forename: Jillian J
authors.initials: JJ
authors.lastname: Kril
authors.affiliation: Neuroscience Research Australia, Sydney, Australia; ARC Centre of Excellence in Cognition and its Disorders, the University of New South Wales, Sydney, Australia; School of Medical Sciences, Faculty of Medicine, University of New South Wales, Sydney, Australia; Brain and Mind Centre, University of Sydney, Australia.
authors.forename: Olivier
authors.initials: O
authors.lastname: Piguet
authors.affiliation: Clem Jones Centre for Ageing Dementia Research, Queensland Brain Institute, The University of Queensland, Brisbane, QLD 4072, Australia.
authors.forename: Jürgen
authors.initials: J
authors.lastname: Götz
authors.affiliation: Sydney Medical School, Brain & Mind Centre, University of Sydney, Australia.
authors.forename: Matthew C
authors.initials: MC
authors.lastname: Kiernan
authors.affiliation: Neuroscience Research Australia, Sydney, Australia; School of Medical Sciences, Faculty of Medicine, University of New South Wales, Sydney, Australia; Brain and Mind Centre, University of Sydney, Australia.
authors.forename: Glenda M
authors.initials: GM
authors.lastname: Halliday
authors.affiliation: Neuroscience Research Australia, Sydney, Australia; ARC Centre of Excellence in Cognition and its Disorders, the University of New South Wales, Sydney, Australia; School of Medical Sciences, Faculty of Medicine, University of New South Wales, Sydney, Australia; Brain and Mind Centre, University of Sydney, Australia. Electronic address: john.hodges@sydney.edu.au.
authors.forename: John R
authors.initials: JR
authors.lastname: Hodges
authors.affiliation: Neuroscience Research Australia, Sydney, Australia; Dementia Research Unit, Department of Anatomy, School of Medical Sciences, Faculty of Medicine, University of New South Wales, Sydney, Australia. Electronic address: l.ittner@unsw.edu.au.
authors.forename: Lars M
authors.initials: LM
authors.lastname: Ittner
mesh_terms: D000690:Amyotrophic Lateral Sclerosis; D000818:Animals; D004195:Disease Models, Animal; D057180:Frontotemporal Dementia; D006801:Humans; D051379:Mice; D009154:Mutation; D010641:Phenotype
keywords: Amyotrophic lateral sclerosis; Behavioural neurology; Frontotemporal dementia; Mouse; Transgenic