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Exp Cell Res. 2009 Apr 15;315(7):1157-70. doi: 10.1016/j.yexcr.2009.01.027. Epub 2009 Feb 10.

A short carboxy-terminal domain of polycystin-1 reorganizes the microtubular network and the endoplasmic reticulum.

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Renal Division, University Hospital Freiburg, Freiburg, Germany.


Mutations of PKD1 cause autosomal dominant polycystic kidney disease (ADPKD), a syndrome characterized by kidney cysts and progressive renal failure. Polycystin-1, the protein encoded by PKD1, is a large integral membrane protein with a short carboxy-terminal cytoplasmic domain that appears to initiate multiple cellular programs. We report now that this polycystin-1 domain contains a novel motif responsible for rearrangements of intermediate filaments, microtubules and the endoplasmic reticulum (ER). This motif reveals homology to CLIMP-63, a microtubule-binding protein that rearranges the ER. Our findings suggest that polycystin-1 influences the shape and localization of both the microtubular network and the ER.

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