Dr. Kenji Kabashima and colleagues at the Kyoto University Graduate School of Medicine have discovered that Wnt signaling is involved in the development of pachydermoperiostosis. They present these findings in the February 2010 issue of The American Journal of Pathology.
Pachydermoperiostosis is a rare skin disease characterized by pachydermia (skin thickening), digital clubbing, and periostosis (abnormal bone deposits) of long bones. Fibroblasts help to form the structural framework for various tissues, including the skin and bones, and therefore likely play a role in this process.
As the Wnt signaling pathway plays a developmental role in both bone and skin, Kabashima et al hypothesized that Wnt signaling was involved in the development of pachydermia. They found that DKK1, a Wnt-signaling antagonist, was expressed at lower levels in pachydermoperiostosis than in control fibroblasts, whereas the Wnt-signaling molecule β-catenin was expressed at higher levels. In addition, expression of DKK1 inhibited fibroblast proliferation, and DKK1-inhibition increased skin thickening in mouse ears. Therefore, enhanced Wnt signaling may contribute to pachydermia by increasing levels of fibroblast proliferation.
Dr. Kabashima and colleagues therefore conclude that "enhanced Wnt signaling is related to the development of pachydermia."
- Kabashima K, Sakabe J-i, Yoshiki R, Tabata Y, Kohno K, Tokura Y. Involvement of Wnt Signaling in Dermal Fibroblasts. American Journal Of Pathology, 2009; DOI: 10.2353/ajpath.2010.090454
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