Regarding pathophysiology, TSC inactivation and mTOR activation in renal tubular epithelial cells are associated with tubular dedifferentiation, interstitial fibrosis, and cyst formation.7, 8, 9 As in nephronophthisis, inflammatory mediators secreted by tubular cells can trigger the recruitment of immune cells in response to mTOR activation.S3 We observed a strong expression of phosphorylated S6 ribosomal protein in dystrophic tubular epithelial cells, suggesting an overactivation of the mTOR pathway in this tubular cells. This evidence concerns the gene MTOR and cyst.