Study of the functional interaction between integrin β1 and polycistin 1 in the remodelling of the tubular basement membrane and the maintenance of renal architecture - Necker Institute - Paris

 

Autosomal dominant polycystic kidney disease (ADPKD) is a common genetic disorder characterised by the formation of renal cysts, leading to renal hypertrophy and renal failure. Mutations in the Pkd1 and Pkd2 genes, coding for the polycystin-1 and polycystin-2 proteins, are responsible for the disease. Primary cilia, cellular organelles, play a crucial role in maintaining renal architecture, and dysregulation of these cilia contributes to renal ciliopathies.

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The study highlights the importance of β1 integrin in tubular basement membrane remodelling, underlining its role in the development of renal cysts and fibrosis. Experiments show that simultaneous deletion of Pkd1 and Itgb1 reduces the cystic phenotype, suggesting an interrelationship between these proteins. The aim of this study is to explore the functional interaction between β1 integrin and polycystin-1, by characterising the alterations in renal architecture caused by deletion of these genes in adult mice.