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 [
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Figure 5Mitten deformities in the extremities of the
Col7a1flNeo/flNeo mice closely resemble severe human RDEB.
(
A–
C) Hands in human RDEB patients at 2.5 years (
A), 6.5 years (
B), and 7.5 years of age (
C). (
D–
G) Fore paws of
Col7a1flNeo/flNeo mice were documented at 17 days (
D), 60 days (
E), and 80 days of age (
F and
G). The clinical phenotypes in RDEB patients and in
Col7a1flNeo/flNeo mice developed in a similar manner.