Fig. 3.

Model of ciliary signaling disruption. a Signaling proteins are enriched in the cilium where they coordinate cilia-specific signaling that is transmitted to the cell. Two examples of ciliary signaling pathways are illustrated; PC1- and PC2-mediated Ca²⁺ signaling and GPCR-mediated cAMP-signaling. b Disruption in trafficking of signaling proteins (indicated by X) into the ciliary compartment leads to loss of a cilia-specific signal. Ciliary receptors that are not trafficked to the cilium may accumulate in intracellular vesicles or mislocalize on the cell membrane (indicated by ?), possibly resulting in a gain of signal. c Defects in cilia structure prevents proper localization of ciliary signaling proteins (indicated by X) and leads to loss of a cilia-specific signal. Ciliary receptors that are not trafficked to the cilium may accumulate in intracellular vesicles or mislocalize on the cell membrane (indicated by ?), possibly resulting in a gain of signal