EIF2B5 and ovarioleukodystrophy: In human and rodent models, mutations that lead to persistent activation of the ISR are associated with neurological disturbances (Harding et al., 2009; Borck et al., 2012; Abdulkarim et al., 2015; Kernohan et al., 2015; Skopkova et al., 2017) and those that affect the eIF2B subunits cause a rare leukodystrophy called vanishing white matter disease (VWMD) (van der Knaap et al., 2006).