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KMID : 0578320100290010063
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Molecules and Cells
2010 Volume.29 No. 1 p.63 ~ p.69
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Downregulation of Wnt-Mediated ROS Generation Is Causally Implicated in Leprechaunism
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Park Ji-Won
Kuehn Hye-Sun
Kim So-Youn
Chung Kyung-Min
Choi Hyun
Kim Mira
Kim Jaesang
Lee Soo-Young
Bae Duk-Soo
Jin Dong-Kyu
Bae Yun-Soo
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Abstract
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Although mutations in the insulin receptor have been causally implicated with leprechaunsim, the full pathophysiology of the syndrome cannot be accounted for by malfunction of this gene alone. We sought to characterize a connection between Wnt-mediated cell signaling and the production of reactive oxygen species (ROS) which revealed a novel mechanistic basis for understanding the pathogenesis of leprechaunism. To identify candidate genes involved in this process, a PCR-based subtractive hybridization was performed. Candidate genes were examined for interaction with the Wnt signaling pathway and ROS generation. We found that Dickkopf 1 (Dkk1), a Wnt inhibitor, is overexpressed in skin fibroblast cells derived from three leprechaunism patients and that the cells showed an impaired response to Wnt2 in terms of
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KEYWORD
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dickkopf, leprechaunism, NADPH oxidase, NF-AT, Wnt
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