“…Because of unavailability of pre-symptomatic HD brain tissues, the reasons behind selective striatal vulnerability in HD were mostly investigated using animal models. In fact, the basal ganglia and, particularly, the cortico-striatal motor circuitry, appears to be conserved in mouse, minipig, and primates ( Vodicka et al, 2005 ; Stephenson-Jones et al, 2011 ; Balsters et al, 2020 ). Thus, genetically engineered models, bearing normal or pathological CAG repeat lengths, have revealed important pathogenic mechanisms of the HD mutation ( Menalled, 2005 ; Lerner et al, 2012 ; Peng et al, 2016 ; Table 1 and Figures 1A,B ).…”