Huntington’s disease (HD) is a devastating neurodegenerative disorder caused by a toxic, aggregation-prone expansion of the trinucleotides (cytosine/adenosine/guanine (CAG)) encoding for glutamine (Q) residues in the huntingtin (HTT) gene [5], with an age-dependent progression that leads to behavioral, cognitive and motor symptoms. Here, HTT is linked to juvenile Huntington disease.