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

本项目旨在研究干细胞治疗先天性严重皮肤病隐性营养不良大疱性表皮松解症（EB）的效果，采用蛋白人源化EB模型小鼠。我们将GFP Tg小鼠的骨髓移植到缺乏小鼠VII型胶原（COL7）和表达不完全/完全人COL7的EB模型小鼠。皮肤上皮区显示多达0个GFP+骨髓源性角化细胞。2%。部分小鼠也有COL7 mRNA和COL7蛋白表达，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执行和分析，已知这些基因可以增强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.