X-linked nephrogenic diabetes insipidus (XNDI) is a severe congenital kidney disease caused by mutations in the V2R gene. Currently, there is no effective drug to specifically treat XNDI, mainly because there are no good animal models of the disease.
However, Jόrgen Wess and colleagues, at the National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, have now developed a viable mouse model of XNDI that recapitulates the major manifestations of the human disease. The research appears in the Sept. 1, 2009 issue of the Journal of Clinical Investigation.
As analysis of the mouse model indicated that disease was associated with increased expression in the kidney of the protein EP4, the authors tested selective EP4 receptor agonists as potential therapeutics, and found that they alleviated all the symptoms of disease.
The authors therefore suggest that selective EP4 receptor agonists could be of benefit to individuals with XNDI and hope that their mouse model can be used to test additional new therapeutic strategies.
- Jian Hua Li et al. A selective EP4 PGE2 receptor agonist alleviates disease in a new mouse model of X-linked nephrogenic diabetes insipidus. Journal of Clinical Investigation, 2009; DOI: 10.1172/JCI39680
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