Article
A polycystin-2 (TRPP2) dimerization domain essential for the function of heteromeric polycystin complexes.
The EMBO journal - 7 Apr 2010
Giamarchi Aurélie, Feng Shuang, Rodat-Despoix Lise, Xu Yaoxian, Bubenshchikova Ekaterina, Newby Linda J, Hao Jizhe, Gaudioso Christelle, Crest Marcel, Lupas Andrei N, Honoré Eric, Williamson Michael P, Obara Tomoko, Ong Albert C M, Delmas Patrick
Abstract excerpt
Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in two genes, PKD1 and PKD2, which encode polycystin-1 (PC1) and polycystin-2 (PC2), respectively. Earlier work has shown that PC1 and PC2 assemble into a polycystin complex implicated in kidney morphogenesis. PC2 also assembles into homomers of uncertain functional significance. However, little is known about the molecular mechanisms...
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