Figure 2 | Laboratory Investigation

Figure 2

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

Figure 2

Phenotypic manifestations of recessive dystrophic epidermolysis bullosa (RDEB) in CRISPR/Cas9 knockout NSG mice. (a) Phenotypic manifestations of RDEB in Col7a1−/− NSG mice. Neonatal mice exhibit blistered paws shortly after birth, followed by formation of the more severe blisters and open wounds characteristic of skin fragility. (b–e) Immunofluorescence staining of type VII collagen expression in Col7a1−/− NSG mice. Cross-sections of skin and esophagus in wild-type (b, d) and knockout (c, e) neonates showing the absence of type VII (red) in the esophageal membrane and at the dermal–epidermal junction in skin. (f) Representative patterns of Col7a1 mutations produced by CRISPR/Cas9 nuclease activity after embryo injection. Indels are observed at both guide RNA (gRNA) target sites independently and simultaneously. gRNA-spanning deletions are also observed.

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