In this work, we have studied the role of Sig-1R and its agonist 2-(4-morpholinoethyl)-1-phenylcyclohexane-1-carboxylate hydrochloride (PRE084) in a cellular model of HD with overexpression of wild-type and mutant huntingtin proteins in neuronal PC6.3 cells. Here, HTT is linked to Huntington disease.