NFE2L2 and Netherton syndrome: To assess the consequences of Nrf2 activation in a murine model of Netherton syndrome, Spink5 knockout mice (Spink5ko) (Yang et al., 2004) and transgenic mice expressing caNrf2 in keratinocytes (K5cre-CMVcaNrf2) (Schäfer et al., 2012) were used for the generation of mice lacking Spink5 and expressing caNrf2 in keratinocytes using a three-step breeding scheme (Fig. 1A,B).