A09 - Regulated cell death and kidney fibrosis as a consequence of mutation of cilia-associated genes
Nephronophthisis (NPH) is an autosomal recessive disorder caused by mutations in single genes relevant for primary cilia, sensory organelles at the surface of tubular epithelial cells. The disease is characterised by progressive loss of renal tubular. However, the role of cell death and the molecular link to cilia signalling are still elusive. The aim of this project is to study the role of regulated cell death (RCD) in the pathogenesis of NPH and to elucidate the role of cilia and cilia-associated NPH proteins in controlling cell death signalling as well as the crosstalk of surrounding cells with dying kidney epithelial cells.