Table 1.
Murine models used in this study
| Model | Name | Mutation/Deletion | Age/Disease Stage | Provider |
|---|---|---|---|---|
| Jck | juvenile cystic kidney | Missense mutation on Nek8 gene, G448V, in the highly conserved RCC1 domain of NEK8. | 26–64 days (Fig. 7); 64 days in all other experiments/high level of cystogenesis | N. Bukanov and O. Ibraghimov-Beskrovnay. |
| Bpk | BALB/c polycystic kidney | Abnormal 3′ coding region of the bicaudal C homolog 1 gene, Bicc1 | Postnatal day 21/very advanced disease | T. Weimbs |
| Pkd1flox/−:Ksp-Cre | Polycystic kidney disease 1 (polycystin 1) | Pkd1 gene inactivated in kidneys | P7/severe development of cystogenesis (mice die at P13) | A. Boletta |
| Pkd1 extra 11 | Polycystic kidney disease 1 (polycystin 1) | Deletion of a fragment of the Pkd1 gene. Expresses only extracellular domain of PKD1 | 15–24 mo | M. Trudel |
| Pkd1TAG26 | Polycystic kidney disease 1 (polycystin 1) | Mutation on Pkd1 gene in renal and extra renal tissues | 5–7 mo | M. Trudel |
| Pkd1-cKO | Polycystic kidney disease 1 (polycystin 1) | Conditional knockout of Pkd1 gene | 52–54 days/moderate level of cystogenesis | N. Bukanov and O. Ibraghimov-Beskrovnaya |
| Pcy | Polycystic | Missense mutation (T1841G) in the mouse ortholog of the human Nphp3 gene (encoding Nephrocystin-3) | 30 wk/mild level of cystogenesis | N. Bukanov and O. Ibraghimov-Beskrovnaya |
| Pkd2-cKO | Polycystic kidney disease 2 (polycystin 2) | Pkd2 gene inactivation in reneal collecting ducts | 4 wk/severe cystogenesis | M. Grosch and R. Witzgall |
| Cpk | Congenital polycystic kidney | Mutation in the Cys1 gene | 10 or 21 days | M. Mrug |
| HNF1β | Hepatocyte nuclear factor 1β | Knockout of Hnf1β gene in kidneys | 21–28 days/mild to severe cystogenesis | E. Fischer and M. Pontoglio |
| Thm1-cKO | Tetratricopeptide repeat-containing hedgehog modulator-1 | Conditional knockout of Thm1 gene (ROSA26-CreERT) |
Gene deletion at P0/analysis at P42 | P. V. Tran |
| Nphp4 | Nephronophthisis 4 | Knockout of Nphp4 gene | 4 or 8 mo/no cysts | S. Saunier |