TGFB1 and recessive dystrophic epidermolysis bullosa: While animal models recapitulated the phenotype in RDEB and identified increased transforming growth factor-beta (TGFβ) signaling [18,21], studies in primary human cells clearly demonstrated that absent expression of wild-type C7 in RDEB dermal fibroblasts or knockdown of C7 in normal dermal fibroblasts leads to increased TGFβ signaling and a disruption to ECM protein organization and composition that is tumor-promoting [15,17].