A Novel Mouse Model Reveals that Polycystin-1 Deficiency in Ependyma and Choroid Plexus Results in Dysfunctional Cilia and Hydrocephalus

Polycystin-1 (PC-1), the product of the PKD1 gene, mutated in the majority of cases of Autosomal Dominant Polycystic Kidney Disease (ADPKD), is a very large (∼520 kDa) plasma membrane receptor localized in several subcellular compartments including cell-cell/matrix junctions as well as cilia. While...

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Main Authors: Wodarczyk, Claas, Rowe, Isaline, Chiaravalli, Marco, Pema, Monika, Qian, Feng, Boletta, Alessandra
格式: Artigo
語言:Inglês
出版: Public Library of Science 2009
主題:
Research Article
在線閱讀:https://ncbi.nlm.nih.gov/pmc/articles/PMC2743994/
https://ncbi.nlm.nih.gov/pubmed/19774080
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0007137
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