Cilia in Kidney Development and Disease

The kidney is a uniquely shaped organ that our body requires to maintain fluid homeostasis. Its formation begins with a mesenchymal to epithelial transition, where epithelial branching will give rise to the distinct kidney sub-structures. During kidney development, the primary cilium, a sensory organelle serving as the cell’s antenna contributes to correct renal formation and function. One of the signalling pathways that are transduced via the primary cilium in the kidney is Wnt signalling. Wnt signalling consists of three branches: canonical Wnt signalling through  catenin, non-canonical Wnt/Planar Cell Polarity (PCP) signalling and non-canonical Wnt/Ca⁺² mediated signalling. Defects in either of the three pathways have been shown to affect kidney morphogenesis and function and have been associated with ciliary abnormalities that could also result in chronic, inherited kidney disease. This chapter examines the role of primary cilia in kidney development and function, dissects the significance of Wnt signalling in the context of the kidney and relates ciliary and Wnt signalling perturbations with renal development and disease.