Mouse models of InvsC defects (Invs, pcy/Nphp3, jck/Nek8/Nphp9 and Anks6/Nphp16-mutated mice) show enlarged cystic kidneys characteristic of infantile NPH, although pcy/Nphp3 and Anks6/Nphp16 mice have slow disease progression and (late stage) tubulointerstitial fibrosis (Omran et al., 2001; Bakey et al., 2015). The gene discussed is NPHP3; the disease is Renal cyst.