Since mice null for Elp1 are embryonic lethal (Chen et al., 2009a; Dietrich et al., 2011), FD has been modeled in mice using conditional knockout of Elp1 in neural crest cells (Elp1fl/fl; Wnt1-Cre+, abbreviated ‘Elp1 CKO’), which recapitulates several aspects of the human disease, including significant loss of sensory and autonomic neurons (George et al., 2013; Jackson et al., 2014). Here, ELP1 is linked to Fabry disease.