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Kindler syndrome in mice and men #MMPMID24919121
Duperret EK; Ridky TW
Cancer Biol Ther 2014[Sep]; 15 (9): 1113-6 PMID24919121show ga
Kindler syndrome (KS) in humans is a severe skin blistering disease associated with inflammation and increased risk of epidermal squamous cell carcinoma (SCC). This disease is known to be caused by loss-of-function mutations in Kindlin-1, a focal adhesion ?-integrin binding protein. Thus far, it has been unclear what specific signaling events occur in KS keratinocytes to promote tumorigenesis, especially since loss of ?-integrins and focal adhesion complexes has been previously shown to prevent or delay tumor formation. In the April issue of Nature Medicine, Rognoni and colleagues generate a transgenic mouse lacking Kindlin-1 in the epidermis to model the key features of KS, and show that Kindlin-1 regulates Wnt and TGF? signaling independent of ?-integrins. These ?1-integrin-independent functions of Kindlin-1 may contribute to the increased SCC risk in KS patients.