NFE2L2 and juvenile Huntington disease: However, it is important to note that neuroprotective effects of CDDO-ethyl amide were observed in a transgenic model of Huntington’s disease [348] with an upregulation of ARE-containing genes in the brain (Hmox1, Gts3a, and Nqo1); however, this study did not evaluate nuclear localization of NRF2, so it is not clear whether the effects were mediated by NRF2 activation.