As shown in Fig. 3a, the expression and localization of marker proteins representing endoderm cells (α-fetoprotein; AFP), mesoderm cells (Desmin, Brachyury: BRY), and ectoderm cells (βIII-tubulin, Nestin) could be clearly detected, which demonstrated the pluripotency of ADPKD-iPSCs. Here, DES is linked to autosomal dominant polycystic kidney disease.