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Nephrol Dial Transplant. 2010 Nov;25(11):3496-504. doi: 10.1093/ndt/gfq195. Epub 2010 Apr 13.

Distinct patterns of kidney and liver cyst growth in pkd2(WS25/-) mice.

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  • 1Department of Medicine, University of Colorado Denver, Aurora, CO, USA. brian.doctor@ucdenver.edu

Abstract

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.

PMID:
20388629
[PubMed - indexed for MEDLINE]
PMCID:
PMC2980992
Free PMC Article
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