Luijten et al. (2013) show that FLCN localises to primary cilia in HK2 cells and motile cilia in primary HNE cells. Interestingly, both knock down and overexpression of FLCN in HK-2 cells led to fewer cells with functional cilia at 72 hours. However, after 96 hours, the numbers of cilia tended towards wildtype in both the FLCN-depleted and overexpressed experiments, suggesting that FLCN may control the onset of ciliogenesis but is less important thereafter.

These data, when considered with the observation that cystic lesions are a hallmark of ciliopathies, suggest that BHD may also be a ciliopathy.