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Comment
. 2014 Sep;15(9):1113-6.
doi: 10.4161/cbt.29482. Epub 2014 Jun 11.

Kindler syndrome in mice and men

Elizabeth K Duperret  1 , Todd W Ridky  1
Affiliations
Comment

Kindler syndrome in mice and men

Elizabeth K Duperret et al. Cancer Biol Ther. 2014 Sep.

Abstract

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 β-integrin binding protein. Thus far, it has been unclear what specific signaling events occur in KS keratinocytes to promote tumorigenesis, especially since loss of β-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 TGFβ signaling independent of β-integrins. These β1-integrin-independent functions of Kindlin-1 may contribute to the increased SCC risk in KS patients.

Keywords: Kindler syndrome; Kindlin-1; TGFβ; Wnt; integrin αv; integrin β1; skin; squamous cell carcinoma.

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References

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