Fuz controls the morphogenesis and differentiation of hair follicles through the formation of primary cilia.

Planar cell polarity (PCP) signaling is essential in determining the polarity of cells within the plane of an epithelial sheet. Core PCP genes have been recently shown to control the global polarization of hair follicles in mice. Fuz, a homologue of the Drosophila PCP effector gene, fuzzy, is critical in ciliogenesis in vertebrates, and is required for the development of a wide range of organs in mice. Here, we report that disruption of the Fuz gene in mice severely blocked the development of hair follicles in the skin. In contrast to the loss of hair follicle polarization in mice deficient in core PCP genes, hair follicles in mice lacking the Fuz gene retained their typical anterior-posterior orientation. We show that disruption of Fuz impaired the formation of primary cilia and the hedgehog signaling pathway in the skin. In addition, using skin grafts and skin reconstitution assays we demonstrate that the expression of Fuz is required in both epidermal and dermal cells and that the formation of primary cilia is a cell-autonomous process that does not require cross talk between the epithelia and mesenchymal compartments during hair follicle formation.