Affiliation: Harvard University
Su X, Wu M, Yao G, El Jouni W, Luo C, Tabari A, et al
. Regulation of polycystin-1 ciliary trafficking by motifs at its C-terminus and polycystin-2 but not by cleavage at the GPS site. J Cell Sci. 2015;128:4063-73 pubmed publisher
..As all pathogenic PC1 mutations tested here are defective in ciliary trafficking, ciliary trafficking might serve as a functional read-out for ADPKD. ..
Yao G, Luo C, Harvey M, Wu M, Schreiber T, Du Y, et al
. Disruption of polycystin-L causes hippocampal and thalamocortical hyperexcitability. Hum Mol Genet. 2016;25:448-58 pubmed publisher
..Our data, thus, indicate for the first time that a ciliary protein complex is required for the control of neuronal excitability in the CNS. ..
Zhou J. Polycystins and primary cilia: primers for cell cycle progression. Annu Rev Physiol. 2009;71:83-113 pubmed publisher
..Their roles in primary cilia, shear stress sensation, alteration of intracellular calcium, and planar cell polarity are examined. The third-hit hypothesis of polycystic kidney disease is discussed. ..
Su X, Driscoll K, Yao G, Raed A, Wu M, Beales P, et al
. Bardet-Biedl syndrome proteins 1 and 3 regulate the ciliary trafficking of polycystic kidney disease 1 protein. Hum Mol Genet. 2014;23:5441-51 pubmed publisher
..Moreover, physical interactions between the BBS and ADPKD proteins may underline the overlapping renal phenotypes in these two diseases...