Nrf2 deficient mice exacerbate pathology in models of AD, PD, Huntington's disease, vascular impairment/stroke and multiple sclerosis, while genetic or pharmacological activation of Nrf2 has shown beneficial effects in these models through its capacity to attenuate various pathological processes such as neuroinflammation, mitochondrial dysfunction and oxidative stress [[6], [7], [8]]. Here, NFE2L2 is linked to juvenile Huntington disease.