Folliculin Interacts With P0071 (Plakophilin-4) and Deficiency Is Associated With Disordered RhoA Signalling, Epithelial Polarization, and Cytokinesis

    September 2012 in “ Human molecular genetics online/Human molecular genetics ”
    Michael S. Nahorski, Laurence Seabra, Ania Straatman-Iwanowska, Aileen Wingenfeld, Anne Reiman, Xiaohong Lü, Jeffrey A. Klomp, Bin Tean Teh, Meçhthild Hatzfeld, Paul Gissen, Eamonn R. Maher
    TLDR Folliculin deficiency causes problems with cell division and positioning due to disrupted RhoA signaling and interaction with p0071.
    The study investigated the interaction between folliculin (FLCN) and p0071 (plakophilin-4), revealing that FLCN deficiency disrupted RhoA signaling, epithelial polarization, and cytokinesis. This disruption led to increased cell migration, tight junction defects, and mislocalization of junctional proteins like E-cadherin. The findings highlighted the crucial role of the FLCN/p0071 complex in maintaining cell structure and function, suggesting potential therapeutic targets for diseases related to FLCN deficiency, such as Birt–Hogg–Dubé syndrome.
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