A hypomorphic mouse model of dystrophic epidermolysis bullosa reveals mechanisms of disease and response to fibroblast therapy
J. Clin. Invest. Anja Fritsch, et al. 118:1669 doi:10.1172/JCI34292 [Go to this article.]

Figure 8
Removal of the PKG-Neo cassette reverts the phenotype of Col7a1^flNeo/flNeo mice. (A) Schematic representation of the Col7a1^fl allele. Black boxes denote Col7a1 exons; black triangles denote loxP sites; gray oval denotes Frt site. (B) Col7a1^fl/fl mice were indistinguishable from their littermates, showing the same weight at 25 and 60 days of age (n = 5 [Col7a1^fl/fl]; 8 [Col7a1^fl/WT]; 6 [Col7a1^WT/WT]). (C) Immunoblotting of dermal proteins from Col7a1^WT/WT (lane 1), Col7a1^WT/flNeo (lane 2), and Col7a1^fl/fl (lane 3) mice with the NC2-10 antibody (44) showed no difference in collagen VII expression. Coomassie blue staining of the α1 chain of collagen I is shown as a loading control. (D) Immunofluorescence staining of skin, tongue, and forestomach of Col7a1^fl/fl mice demonstrated normal collagen VII deposition at the DEJZ. Scale bar: 50 μm.