Specifically, we have shown that cystine crystals activate the inflammasome in monocytes (15); and that NLRP2 is markedly overexpressed in human cystinotic proximal tubular epithelial cells (PTEC) in a kidney biopsy obtained from a patient with cystinosis, in primary cultured PTEC and in immortalized PTEC cell lines derived from cystinotic patients (19). This evidence concerns the gene NLRP2 and cystinosis.