The patient perspective and physician's role in making decisions on instituting dialysis.

ation by the ciliopathy protein Rpgrip1 l is essential for planar cell polarity. J Cell Biol 2012; 198: 927–940 18. Pugacheva EN, Jablonski SA, Hartman TR et al. HEF1-dependent Aurora A activation induces disassembly of the primary cilium. Cell 2007; 129: 1351–1363 19. Kim S, Tsiokas L. Cilia and cell cycle re-entry: more than a coincidence. Cell Cycle 2011; 10: 2683–2690 20. Phillips CL, Miller KJ, Filson AJ et al. Renal cysts of inv/inv mice resemble early infantile nephronophthisis. J Am Soc Nephrol 2004; 15: 1744–1755 21. Shiba D, Manning DK, Koga H et al. Inv acts as a molecular anchor for Nphp3 and Nek8 in the proximal segment of primary cilia. Cytoskeleton 2010; 67: 112–119 22. Morgan D, Eley L, Sayer J et al. Expression analyses and interaction with the anaphase promoting complex protein Apc2 suggest a role for inversin in primary cilia and involvement in the cell cycle. HumMol Genet 2002; 11: 3345–3350 23. Nurnberger J, Bacallao RL, Phillips CL. Inversin forms a complex with catenins and N-cadherin in polarized epithelial cells. Mol Biol Cell 2002; 13: 3096–3106 24. Plotnikova OV, Pugacheva EN, Golemis EA. Aurora A kinase activity influences calcium signaling in kidney cells. J Cell Biol 2011; 193: 1021–1032 25. Streets AJ, Wessely O, Peters DJ et al. Hyperphosphorylation of polycystin-2 at a critical residue in disease reveals an essential role for polycystin-1-regulated dephosphorylation. Hum Mol Genet 2013; 22: 1924–1939 26. Bergmann C, Fliegauf M, Bruchle NO et al. Loss of nephrocystin3 function can cause embryonic lethality, Meckel–Gruber-like syndrome, situs inversus, and renal-hepatic-pancreatic dysplasia. Am J Hum Genet 2008; 82: 959–970 27. Smith LA, Bukanov NO, Husson H et al. Development of polycystic kidney disease in juvenile cystic kidney mice: insights into pathogenesis, ciliary abnormalities, and common features with human disease. J Am Soc Nephrol 2006; 17: 2821–2831 28. Hopp K, Ward CJ, Hommerding CJ et al. Functional polycystin1 dosage governs autosomal dominant polycystic kidney disease severity. J Clin Invest 2012; 122: 4257–4273 29. Verghese E, Ricardo SD, Weidenfeld R et al. Renal primary cilia lengthen after acute tubular necrosis. J Am Soc Nephrol 2009; 20: 2147–2153 30. Bastos AP, Piontek K, Silva AM et al. Pkd1 haploinsufficiency increases renal damage and induces microcyst formation following ischemia/reperfusion. J Am0: 2389–2402 31. Prasad S, McDaid JP, Tam FW et al. Pkd2 dosage influences cellular repair responses following ischemia-reperfusion injury. Am J Pathol 2009; 175: 1493–1503