NFE2L2 and juvenile Huntington disease: Recently, it has been shown that fumaric acid esters (FAEs) enhance survival of various central nervous system (CNS) cell types in response to oxidative insults in vitro [8–12], improve outcomes in animal models of Huntington’s disease and experimental autoimmune encephalomyelitis [8, 13], and activate the nuclear factor erythroid 2-related factor 2 (Nrf2) pathway and upregulate Nrf2-dependent antioxidant genes and proteins in the brain [8, 9].