Indeed, it has been widely demonstrated that the local production of inflammatory mediators by hematopoietic and non-hematopoietic cells, including Cxcl1, Saa1, Il6 and Mcp1, which we found modulated in cystinotic kidney by Nlrp2 deletion, plays an important role in promoting immune and inflammatory responses which, in turn, may contribute to the exacerbation of kidney damage (29, 30). This evidence concerns the gene CCL2 and Nephropathy.