Abstract

P63 is a major transcription factor regulating skin development and homeostasis : cell proliferation, adhesion, and early differentiation. P63 is mutated in several rare syndromes called p63-related ectodermal dysplasia syndromes (ED). One of the main form, called AEC syndrome, associates ankyloblepharon, clef/lip palate, ED, and severe skin erosions that not always heal. P63 belongs to the p53 gene family. We found that ED-iPSC epidermal commitment can be rescued by a p53-reactivating compounds called PRIMA-1MET currently used in anticancer clinical trials.

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