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J Am Soc Nephrol. 2014 Jan;25(1):18-32. doi: 10.1681/ASN.2013040398. Epub 2013 Dec 12.

Strategies targeting cAMP signaling in the treatment of polycystic kidney disease.

Author information

1
Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota.

Abstract

Polycystic kidney disease (PKD) is a leading cause of ESRD worldwide. In PKD, excessive cell proliferation and fluid secretion, pathogenic interactions of mutated epithelial cells with an abnormal extracellular matrix and alternatively activated interstitial macrophages, and the disruption of mechanisms controlling tubular diameter contribute to cyst formation. Studies with animal models suggest that several diverse pathophysiologic mechanisms, including dysregulation of intracellular calcium levels and cAMP signaling, mediate these cystogenic mechanisms. This article reviews the evidence implicating calcium and cAMP as central players in a network of signaling pathways underlying the pathogenesis of PKD and considers the therapeutic relevance of treatment strategies targeting cAMP signaling.

PMID:
24335972
PMCID:
PMC3871779
DOI:
10.1681/ASN.2013040398
[Indexed for MEDLINE]
Free PMC Article

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