Primary cilia as regulators of fibrosis and potential therapeutic targets

Fibrosis complicates numerous rheumatic diseases and accounts for considerable morbidity and mortality. Developing more effective treatments will require a deeper understanding of fibrosis. Discoveries over the past decade implicate the ubiquitous cellular organelle primary cilium in modulating tissue repair in health and disease. In particular, emerging evidence shows that dysregulated function of primary cilia might have a previously unrecognized pathogenic role in fibrosis across multiple organs and conditions. Historically considered vestigial organelles, primary cilia are complex microtubule-based solitary organelles present on most vertebrate cells. They function as signalling hubs for Toll-like receptors and receptors for morphogen and growth factors and thereby regulate physiological and pathological tissue remodelling. Pharmacological modulation of primary cilia represents a potential approach to target cellular function and could offer a future therapeutic strategy for fibrotic diseases.