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Fig. 6 | Orphanet Journal of Rare Diseases

Fig. 6

From: RSPO1-mutated keratinocytes from palmoplantar keratoderma display impaired differentiation, alteration of cell–cell adhesion, EMT-like phenotype and invasiveness properties: implications for squamous cell carcinoma susceptibility in patients with 46XX disorder of sexual development

Fig. 6

Keratinocyte treatment with Rspo1 protein does not revert the EMT-like phenotype. Primary keratinocytes from affected plantar (ANp) skin of RSPO1-mutated XX-sex reversed patient AN and from plantar (Cp) skin of aged-matched unrelated donor were treated with recombinant Rspo1 protein. A, B Immunoblots of β-catenin, E-cadherin, vimentin, keratin 5, involucrin, PCNA, p63, p53, p16, and RAS in ANp or Cp keratinocytes treated with several doses of Rspo1. Densitometric values were normalized to GAPDH levels and expressed as fold change. C Immunoblots of Wnt-5a, Wnt-4 and Wnt-10a in ANp or Cp keratinocytes treated with 200 ng/ml of Rspo1. Densitometric values were normalized to GAPDH levels and expressed as fold change. D ANp and Cp were serially cultivated in presence of 200 ng/ml of Rspo1 and compared to corresponding untreated culture. The cumulative number of cell generations per passage was plotted against the total time in culture. E Representative images of colony forming efficiency (CFE) of Rspo1-treated and untreated ANp cultures. D CFE values and percentage of aborted colonies (paraclones) in Rspo1-treated and untreated ANp cultures (n = 3). Data are shown as mean ± SD. *P < 0.05

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