Figure 3 | Laboratory Investigation

Figure 3

From: Rapid generation of Col7a1−/− mouse model of recessive dystrophic epidermolysis bullosa and partial rescue via immunosuppressive dermal mesenchymal stem cells

Figure 3

Immunomodulation alleviates recessive dystrophic epidermolysis bullosa (RDEB) pathology. (a) Survival of Col7a1−/− NSG (n=15) and immune-competent C57BL/6 (n=10) mice, created by CRISPR/Cas9 embryo injection (P<0.0001). (b) Survival of Col7a1−/− NSG mice infused with ABCB5+ cells (n=17) versus control (n=8, P=0.01). (c) Immunofluorescence staining for type VII collagen (red) in the mice living past 60 days (long-term survivor, LTS). (d) Immunofluorescence staining of murine CD68+ macrophages within the dermis of Col7a1−/− control (top row), wild-type (middle row), and Col7a1−/− treated with ABCB5+ cells (bottom row). Left column are sections stained for mouse CD45 (green), middle for mouse CD68 (red), and right column are images merged with DAPI nuclear stain. (e) Quantification of intradermal CD68+ macrophages by immunofluorescence staining in neonatal NSG mice (*P<0.05).

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