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 6
Mitten deformities of the extremities in the Col7a1^flNeo/flNeo mouse are not primarily caused by bone abnormalities. (A–D) High-resolution digital radiography in 2 planes of the extremities of Col7a1^WT/WT (A) and Col7a1^flNeo/flNeo mice (B and C) as well as the right hand of a RDEB patient, demonstrating severe mitten deformity (D). Col7a1^flNeo/flNeo mice are shown both mildly (B) and strongly (C) affected. Top panels, anterior-posterior; bottom panels, lateral. Note the reduced mineral content of the long bones in the extremities of the mice demonstrated by increased X-ray transparency. (E–G) Corresponding high-resolution MRI in sagittal (top panels), transversal (middle panels), and coronal (bottom panels) orientation in mildly (F) and strongly affected (G) Col7a1^flNeo/flNeo mice as well as Col7a1^WT/WT mice (E) revealed the extent of deformities and thickening of the soft tissues. Scale bars: 1 mm (A–C and E–G); 20 mm (D).