Cilium, centrosome and cell cycle regulation in polycystic kidney disease

Polycystic kidney disease is the defining condition of a group of common life-threatening genetic disorders characterized by the bilateral formation and progressive expansion of renal cysts that lead to end stage kidney disease. Although a large body of information has been acquired in the past years about the cellular functions that characterize the cystic cells, the mechanisms triggering the cystogenic conversion are just starting to emerge. Recent findings link defects in ciliary functions, planar cell polarity pathway, and centrosome integrity in early cystic development. Many of the signals dysregulated during cystogenesis may converge on the centrosome for its central function as a structural support for cilia formation and a coordinator of protein trafficking, polarity, and cell division. Here, we will discuss the contribution of proliferation, cilium and planar cell polarity to the cystic signal and will analyze in particular the possible role that the basal bodies/centrosome may play in the cystogenetic mechanisms. This article is part of a Special Issue entitled: Polycystic Kidney Disease.

Research Highlights
► The sequence of early molecular events that trigger renal cystogenesis is unclear.
► Cell growth is required but not sufficient to trigger cystogenesis.
► Cilia and planar cell polarity defects during development lead to cystic kidneys.
► Dysregulation of polycystic proteins causes centrosome aberrations.
► Centrosome controls cilium position, cell cycle, and spindle organization.