Methods and compositions for promoting wound healing in a subject suffering from ectodermal dysplasias

The present invention relates to a method for promoting wound healing in a subject suffering from Ectodermal dysplasia in need thereof comprising a step of administering subcutaneously, intradermally or topically to said subject a therapeutically effective amount of a compound which restores the activity of p63. Inventors have performed a primary culture of patient keratinocytes suffering from ectodermal dysplasias with two compounds which restore the activity of p63 (e.g.STIMA-1 and/or PRIMA-1Met). They have shown that there is an important differentiation of the keratinocytes of said patient compared to the cells not treated with these compounds. They observed that the activity of p63 mutated is restored, thus the proliferation and differentiation of keratinocytes from the patient are activated. Moreover, inventors have used PRIMA-1Met by topical application on a young patient suffering from ectodermal dysplasias and shown that said patient presents an improvement on her hand. Typically, severe skin erosions (on hands and feet) are healing when PRIMA-1Met is administered topically on the hand.

Publications:
Cell Death Dis . 2020 Jan 16;11(1):30. doi: 10.1038/s41419-020-2223-8.

Reference:

BIO16331-T1

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Aymeric Empereur
Aymeric Empereur
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Rare disease: Yes

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