This selective neuronal vulnerability has been associated to the aggregation of misfolded proteins that include: β-amyloid (Aβ) and Tau in Alzheimer’s disease (AD); α-synuclein (α-syn) in Parkinson’s disease (PD), dementia with Lewy bodies and multiple system atrophy; huntingtin in Huntington’s disease; TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis; among others (Dugger and Dickson, 2017; Fu et al., 2018). Here, HTT is linked to Alzheimer disease.