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

该项目的目的是利用蛋白质人源化的大疱性表皮松解症模型小鼠，研究干细胞治疗先天性严重皮肤病--隐性营养不良性大疱性表皮松解症(EB)的效果。我们进行了从GFP转基因小鼠到EB模型小鼠的骨髓移植，该模型小鼠缺乏鼠VI型胶原(COL7)，并且表达不完全/完全的人COL7。表皮皮肤区显示GFP+骨髓源性角质形成细胞多达0。2%。小鼠COL7mRNA和COL7蛋白在部分小鼠体内也有表达，但表达水平较低。我们现在正在使用HMGB1、CCL27和CCL19进行另一种骨髓移植模型的分析，这些模型已知可以促进骨髓细胞向皮肤成分细胞的转分化和募集。

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.