In the most common FTD subtype,
behavioral variant FTD, patients
present with social dysfunction, apathy, disinhibition, repetitive behavior, and other
behavioral abnormalities (Rascovsky et al., 2011; Karageorgiou and Miller, 2014).
It is known that motor
abnormalities in CAG 94 KI and CAG 140 KI mouse models are
present from an early age, with a delayed onset observed in the CAG 94 KI model as compared to CAG 140 KI; in addition, overt
behavioral deficits were observed after 2 years of age in the CAG 140 KI mouse model (Menalled et al., 2003, Hickey et al., 2008).