| First Author | Doctor RB | Year | 2010 |
| Journal | Nephrol Dial Transplant | Volume | 25 |
| Issue | 11 | Pages | 3496-504 |
| PubMed ID | 20388629 | Mgi Jnum | J:283263 |
| Mgi Id | MGI:6386960 | Doi | 10.1093/ndt/gfq195 |
| Citation | Doctor RB, et al. (2010) Distinct patterns of kidney and liver cyst growth in pkd2(WS25/-) mice. Nephrol Dial Transplant 25(11):3496-504 |
| abstractText | BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is a common genetic disease that results in the development of cystic kidneys and liver. Pkd2(WS25/-) mice are a key genetic mouse model of human ADPKD that recapitulate the 'molecular recessive' nature of human ADPKD. Providing the foundation for future long-term studies, the present work documents distinct patterns of long-term cyst growth in the kidneys and liver of male and female pkd2(WS25/-) mice. METHODS: Gravimetric measurements documented the progression of kidney and liver growth in male and female pkd2(WS25/-) mice over 12 months. A fast imaging with steady-state precision-magnetic resonance imaging (FISP-MRI) technique to measure kidney and liver organ and cyst volumes was optimized and validated. Longitudinal FISP-MRI analyses of changes in cyst volumes were performed in pkd2(WS25/-) mice over 15 months. RESULTS: Male and female pkd2(WS25/-) mice had significant increases in kidney weights after 4 months of age. The progression of kidney growth was minimal after 4 months of age. Liver cyst growth in male pkd2(WS25/-) mice was minimal after 4 months of age but showed an accelerated rate of growth after 8 months of age. Female pkd2(WS25/-) mice also showed accelerated growth but this was delayed in time when compared with male pkd2(WS25/-) mice. CONCLUSIONS: Pkd2(WS25/-) mice are a genetic mouse model that recapitulates the early phenotypic characteristics of human ADPKD kidney cystogenesis. Male pkd2(WS25/-) mice consistently display a late progression in liver growth that is seen in clinically impacted livers of human ADPKD patients. |
