TY - JOUR
T1 - Mouse models of frontotemporal dementia : a comparison of phenotypes with clinical symptomatology
AU - Ahmed, Rebekah M.
AU - Irish, Muireann
AU - van Eersel, Janet
AU - Ittner, Arne
AU - Ke, Yazi D.
AU - Volkerling, Alexander
AU - van der Hoven, Julia
AU - Tanaka, Kimi
AU - Karl, Tim
AU - Kassiou, Michael
AU - Kril, Jillian J.
AU - Piguet, Olivier
AU - Gotz, Jurgen
AU - Kiernan, Matthew C.
AU - Halliday, Glenda M.
AU - Hodges, John R.
AU - Ittner, Lars M.
PY - 2017
Y1 - 2017
N2 - 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.
AB - 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.
UR - https://hdl.handle.net/1959.7/uws:65706
U2 - 10.1016/j.neubiorev.2017.01.004
DO - 10.1016/j.neubiorev.2017.01.004
M3 - Article
SN - 0149-7634
VL - 74
SP - 126
EP - 138
JO - Neuroscience and Biobehavioral Reviews
JF - Neuroscience and Biobehavioral Reviews
IS - Part A
ER -