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Publication : Novel roles of Pkd2 in male reproductive system development.

First Author  Nie X Year  2014
Journal  Differentiation Volume  87
Issue  3-4 Pages  161-71
PubMed ID  24951251 Mgi Jnum  J:214978
Mgi Id  MGI:5604316 Doi  10.1016/j.diff.2014.04.001
Citation  Nie X, et al. (2014) Novel roles of Pkd2 in male reproductive system development. Differentiation 87(3-4):161-71
abstractText  Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common inherited genetic diseases, caused by mutations in PKD1 and/ or PKD2. Infertility and reproductive tract abnormalities in male ADPKD patients are very common and have higher incidence than in the general population. In this work, we reveal novel roles of Pkd2 for male reproductive system development. Disruption of Pkd2 caused dilation of mesonephric tubules/efferent ducts, failure of epididymal coiling, and defective testicular development. Deletion of Pkd2 in the epithelia alone was sufficient to cause reproductive tract defects seen in Pkd2(-/-) mice, suggesting that epithelial Pkd2 plays a pivotal role for development and maintenance of the male reproductive tract. In the testis, Pkd2 also plays a role in interstitial tissue and testicular cord development. In-depth analysis of epithelial-specific knockout mice revealed that Pkd2 is critical to maintain cellular phenotype and developmental signaling in the male reproductive system. Taken together, our data for the first time reveal novel roles for Pkd2 in male reproductive system development and provide new insights in male reproductive system abnormality and infertility in ADPKD patients.
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