Journal Article

Distinct patterns of kidney and liver cyst growth in <i>pkd2(WS25</i><i>/−)</i> mice

R. Brian Doctor, Natalie J. Serkova, Kendra M. Hasebroock, Iram Zafar and Charles L. Edelstein

in Nephrology Dialysis Transplantation

Published on behalf of European Renal Association - European Dialysis and Transplant Assoc

Volume 25, issue 11, pages 3496-3504
Published in print November 2010 | ISSN: 0931-0509
Published online April 2010 | e-ISSN: 1460-2385 | DOI:
Distinct patterns of kidney and liver cyst growth in pkd2(WS25/−) mice

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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.

Keywords: Autosomal dominant polycystic kidney disease (ADPKD); Fast imaging with steady-state precision (FISP); Magnetic resonance imaging (MRI)

Journal Article.  4619 words.  Illustrated.

Subjects: Nephrology

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