Although other mutations within the ZEB1/TCF8 locus and TCF4 trinucleotide repeats are associated with Fuchs’ dystrophy (Riazuddin et al., 2010; Igo et al., 2012; Aldave et al., 2013; Stamler et al., 2013; Nanda et al., 2014; Mootha et al., 2015; Nakano et al., 2015; Afshari et al., 2017; Kuot et al., 2017), only the Col8a2 missense mutant mouse has successfully recapitulated its key features. This evidence concerns the gene COL8A2 and Fuchs endothelial corneal dystrophy.