Development of stem cell therapy for epidermolysis bullosa model mice expressing humanized dermo-epidermal junctional protein

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The aim of the project is to investigate the effect of stem cell therapy for congenital severe skin disorder, recessive dystrophic epidermolysis bullosa(EB), using protein-humanized EB model mice. We performed bone marrow transplantation from GFP Tg mice to EB model mice lacking murine type VII collagen(COL7) and expressing incomplete/complete human COL7. Epithelized skin areas showed GFP+bone marrow-derived keratinocytes as many as 0. 2%. Murine COL7 mRNA and COL7 protein were also expressed in some mice, but the expression levels were low. We are now performing and analyzing another bone marrow transplantation model using HMGB1, CCL27 and CCL19, which are known to enhance transdifferentiation and recruitment from bone marrow cells to skin component cells.