Interestingly, receptors for dopamine and somatostatin are enriched in primary cilia in these cells, suggesting an important sensory role in hormone secretion [14, 15, 82, 83] Given the essential function of SHH signaling and ciliary function for midline specification, it is more likely that variants in ciliary proteins will present with CH in association with syndromes such as Joubert and Bardet-Biedl; however, isolated CH cannot be ruled out as a patient with a nonsense variant in GLI2 presented with CH, but without the polydactyly usually observed in Culler-Jones syndrome [84]. The gene discussed is GLI2; the disease is cyclic hematopoiesis.