Although the profiles of Nrf2 in HD patients are still unknown, striatal cells expressing mutant Htt with 111 glutamines (STHdh Q111/Q111) cells show reduced Nrf2 activity, accompanied by the increased expression of DRP1 and decreased expression of OPA1 [95,96,97] (Figure 5 and Table 3). Here, OPA1 is linked to Huntington disease.