No.	Year	Category	Country	Citation	Journal	Type	PMID	Article
1	2012	antidiuretic hormone receptor antagonists	U.S	471	N Engl J Med	Clinical Trial, Phase III	23121377	Torres VE, Chapman AB, Devuyst O, Gansevoort RT, Grantham JJ, Higashihara E, Perrone RD, Krasa HB, Ouyang J, Czerwiec FS. Tolvaptan in patients with autosomal dominant polycystic kidney disease. N Engl J Med. 2012 Dec 20;367(25):2407-18. doi: 10.1056/NEJMoa1205511. Epub 2012 Nov 3
2	2010	antagonists & inhibitors	Germany	222	N Engl J Med	Journal Article	20581392	Walz G, Budde K, Mannaa M, Nürnberger J, Wanner C, Sommerer C, Kunzendorf U, Banas B, Hörl WH, Obermüller N, Arns W, Pavenstädt H, Gaedeke J, Büchert M, May C, Gschaidmeier H, Kramer S, Eckardt KU. Everolimus in patients with autosomal dominant polycystic kidney disease. N Engl J Med. 2010 Aug 26;363(9):830-40. doi: 10.1056/NEJMoa1003491. Epub 2010 Jun 26
3	2010	antagonists & inhibitors	Switzerland	211	N Engl J Med	Journal Article	20581391	Serra AL, Poster D, Kistler AD, Krauer F, Raina S, Young J, Rentsch KM, Spanaus KS, Senn O, Kristanto P, Scheffel H, Weishaupt D, Wüthrich RP. Sirolimus and kidney growth in autosomal dominant polycystic kidney disease. N Engl J Med. 2010 Aug 26;363(9):820-9. doi: 10.1056/NEJMoa0907419. Epub 2010 Jun 26
4	2013	genetics	France	164	J Am Soc Nephrol	Journal Article	23431072	Cornec-Le Gall E, Audrézet MP, Chen JM, Hourmant M, Morin MP, Perrichot R, Charasse C, Whebe B, Renaudineau E, Jousset P, Guillodo MP, Grall-Jezequel A, Saliou P, Férec C, Le Meur Y. Type of PKD1 mutation influences renal outcome in ADPKD. J Am Soc Nephrol. 2013 May;24(6):1006-13. doi: 10.1681/ASN.2012070650. Epub 2013 Feb 21
5	2013	metabolism	Italy	161	Nat Med	Journal Article	23524344	Rowe I, Chiaravalli M, Mannella V, Ulisse V, Quilici G, Pema M, Song XW, Xu H, Mari S, Qian F, Pei Y, Musco G, Boletta A. Defective glucose metabolism in polycystic kidney disease identifies a new therapeutic strategy. Nat Med. 2013 Apr;19(4):488-93. doi: 10.1038/nm.3092. Epub 2013 Mar 24.
6	2015	diagnosis	U.S	147	J Am Soc Nephrol	Journal Article	24904092	Irazabal MV, Rangel LJ, Bergstralh EJ, Osborn SL, Harmon AJ, Sundsbak JL, Bae KT, Chapman AB, Grantham JJ, Mrug M, Hogan MC, El-Zoghby ZM, Harris PC, Erickson BJ, King BF, Torres VE. Imaging classification of autosomal dominant polycystic kidney disease: a simple model for selecting patients for clinical trials. J Am Soc Nephrol. 2015 Jan;26(1):160-72. doi: 10.1681/ASN.2013101138. Epub 2014 Jun 5
7	2012	glomerular filtration rate	U.S	146	Clin J Am Soc Nephrol	Journal Article	22344503	Chapman AB, Bost JE, Torres VE, Guay-Woodford L, Bae KT, Landsittel D, Li J, King BF, Martin D, Wetzel LH, Lockhart ME, Harris PC, Moxey-Mims M, Flessner M, Bennett WM, Grantham JJ. Kidney volume and functional outcomes in autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 2012 Mar;7(3):479-86. doi: 10.2215/CJN.09500911. Epub 2012 Feb 16.
8	2015	therapy	U.S	144	Kidney Int	Congress	25786098	Chapman AB, Devuyst O, Eckardt KU, Gansevoort RT, Harris T, Horie S, Kasiske BL, Odland D, Pei Y, Perrone RD, Pirson Y, Schrier RW, Torra R, Torres VE, Watnick T, Wheeler DC. Autosomal-dominant polycystic kidney disease (ADPKD): executive summary from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney Int. 2015 Jul;88(1):17-27. doi: 10.1038/ki.2015.59. Epub 2015 Mar 18.
9	2013	genetics	U.S	143	Nat Genet	Journal Article	23892607	Ma M, Tian X, Igarashi P, Pazour GJ, Somlo S. Loss of cilia suppresses cyst growth in genetic models of autosomal dominant polycystic kidney disease. Nat Genet. 2013 Sep;45(9):1004-12. doi: 10.1038/ng.2715. Epub 2013 Jul 28.
10	2014	therapeutic use	U.S	141	N Engl J Med	Journal Article	25399733	Schrier RW, Abebe KZ, Perrone RD, Torres VE, Braun WE, Steinman TI, Winklhofer FT, Brosnahan G, Czarnecki PG, Hogan MC, Miskulin DC, Rahbari-Oskoui FF, Grantham JJ, Harris PC, Flessner MF, Bae KT, Moore CG, Chapman AB. Blood pressure in early autosomal dominant polycystic kidney disease. N Engl J Med. 2014 Dec 11;371(24):2255-66. doi: 10.1056/NEJMoa1402685. Epub 2014 Nov 15
11	2012	genetic loci	U.S	134	J Clin Invest	Journal Article	23064367	Hopp K, Ward CJ, Hommerding CJ, Nasr SH, Tuan HF, Gainullin VG, Rossetti S, Torres VE, Harris PC. Functional polycystin-1 dosage governs autosomal dominant polycystic kidney disease severity. J Clin Invest. 2012 Nov;122(11):4257-73. doi: 10.1172/JCI64313. Epub 2012 Oct 15.
12	2017	therapeutic use	Netherlands	129	N Engl J Med	Clinical Trial, Phase III	29105594	Torres VE, Chapman AB, Devuyst O, Gansevoort RT, Perrone RD, Koch G, Ouyang J, McQuade RD, Blais JD, Czerwiec FS, Sergeyeva O. Tolvaptan in Later-Stage Autosomal Dominant Polycystic Kidney Disease. N Engl J Med. 2017 Nov 16;377(20):1930-1942. doi: 10.1056/NEJMoa1710030. Epub 2017 Nov 4.
13	2010	drug therapy	U.S	124	J Am Soc Nephrol	Journal Article	20431041	Hogan MC, Masyuk TV, Page LJ, Kubly VJ, Bergstralh EJ, Li X, Kim B, King BF, Glockner J, Holmes DR 3rd, Rossetti S, Harris PC, LaRusso NF, Torres VE. Randomized clinical trial of long-acting somatostatin for autosomal dominant polycystic kidney and liver disease. J Am Soc Nephrol. 2010 Jun;21(6):1052-61. doi: 10.1681/ASN.2009121291. Epub 2010 Apr 29
14	2016	genetics	U.S	120	Am J Hum Genet	Journal Article	27259053	Porath B, Gainullin VG, Cornec-Le Gall E, Dillinger EK, Heyer CM, Hopp K, Edwards ME, Madsen CD, Mauritz SR, Banks CJ, Baheti S, Reddy B, Herrero JI, Bañales JM, Hogan MC, Tasic V, Watnick TJ, Chapman AB, Vigneau C, Lavainne F, Audrézet MP, Ferec C, Le Meur Y, Torres VE, Harris PC. Mutations in GANAB, Encoding the Glucosidase IIα Subunit, Cause Autosomal-Dominant Polycystic Kidney and Liver Disease. Am J Hum Genet. 2016 Jun 2;98(6):1193-1207. doi: 10.1016/j.ajhg.2016.05.004.
15	2014	genetics	U.K	117	J Clin Invest	Journal Article	24892705	Harris PC, Torres VE. Genetic mechanisms and signaling pathways in autosomal dominant polycystic kidney disease. J Clin Invest. 2014 Jun;124(6):2315-24. doi: 10.1172/JCI72272. Epub 2014 Jun 2.
16	2010	genetics	U.S	111	J Cell Biol	Journal Article	21079243	Chapin HC, Caplan MJ. The cell biology of polycystic kidney disease. J Cell Biol. 2010 Nov 15;191(4):701-10. doi: 10.1083/jcb.201006173.
17	2011	pathology	U.S	106	Nat Genet	Journal Article	21685914	Fedeles SV, Tian X, Gallagher AR, Mitobe M, Nishio S, Lee SH, Cai Y, Geng L, Crews CM, Somlo S. A genetic interaction network of five genes for human polycystic kidney and liver diseases defines polycystin-1 as the central determinant of cyst formation. Nat Genet. 2011 Jun 19;43(7):639-47. doi: 10.1038/ng.860.
18	2010	genetics	U.S	106	J Am Soc Nephrol	Journal Article	20075061	Shillingford JM, Piontek KB, Germino GG, Weimbs T. Rapamycin ameliorates PKD resulting from conditional inactivation of Pkd1. J Am Soc Nephrol. 2010 Mar;21(3):489-97. doi: 10.1681/ASN.2009040421. Epub 2010 Jan 14
19	2015	therapy	U.K	105	Lancet	Journal Article	26090645	Ong AC, Devuyst O, Knebelmann B, Walz G. Autosomal dominant polycystic kidney disease: the changing face of clinical management. Lancet. 2015 May 16;385(9981):1993-2002. doi: 10.1016/S0140-6736(15)60907-2.
20	2011	cilia	Norway	105	Am J Hum Genet	Case Reports	22019273	Bredrup C, Saunier S, Oud MM, Fiskerstrand T, Hoischen A, Brackman D, Leh SM, Midtbø M, Filhol E, Bole-Feysot C, Nitschké P, Gilissen C, Haugen OH, Sanders JS, Stolte-Dijkstra I, Mans DA, Steenbergen EJ, Hamel BC, Matignon M, Pfundt R, Jeanpierre C, Boman H, Rødahl E, Veltman JA, Knappskog PM, Knoers NV, Roepman R, Arts HH. Ciliopathies with skeletal anomalies and renal insufficiency due to mutations in the IFT-A gene WDR19. Am J Hum Genet. 2011 Nov 11;89(5):634-43. doi: 10.1016/j.ajhg.2011.10.001. Epub 2011 Oct 20.
21	2011	physiopathology	U.S	105	Nat Rev Nephrol	Journal Article	21862990	Grantham JJ, Mulamalla S, Swenson-Fields KI. Why kidneys fail in autosomal dominant polycystic kidney disease. Nat Rev Nephrol. 2011 Aug 23;7(10):556-66. doi: 10.1038/nrneph.2011.109.
22	2014	metabolism	U.S	104	J Am Soc Nephrol	Journal Article	24335972	Torres VE, Harris PC. Strategies targeting cAMP signaling in the treatment of polycystic kidney disease. J Am Soc Nephrol. 2014 Jan;25(1):18-32. doi: 10.1681/ASN.2013040398. Epub 2013 Dec 12
23	2013	therapeutic use	Italy	102	Lancet	Journal Article	23972263	Caroli A, Perico N, Perna A, Antiga L, Brambilla P, Pisani A, Visciano B, Imbriaco M, Messa P, Cerutti R, Dugo M, Cancian L, Buongiorno E, De Pascalis A, Gaspari F, Carrara F, Rubis N, Prandini S, Remuzzi A, Remuzzi G, Ruggenenti P. Effect of longacting somatostatin analogue on kidney and cyst growth in autosomal dominant polycystic kidney disease (ALADIN): a randomised, placebo-controlled, multicentre trial. Lancet. 2013 Nov 2;382(9903):1485-95. doi: 10.1016/S0140-6736(13)61407-5. Epub 2013 Aug 21.
24	2011	metabolism	U.S	102	Proc Natl Acad Sci U S A	Journal Article	21262823	Takiar V, Nishio S, Seo-Mayer P, King JD Jr, Li H, Zhang L, Karihaloo A, Hallows KR, Somlo S, Caplan MJ. Activating AMP-activated protein kinase (AMPK) slows renal cystogenesis. Proc Natl Acad Sci U S A. 2011 Feb 8;108(6):2462-7. doi: 10.1073/pnas.1011498108. Epub 2011 Jan 24.
25	2013	genetics	U.S	96	Nat Genet	Journal Article	23396133	Kirby A, Gnirke A, Jaffe DB, Barešová V, Pochet N, Blumenstiel B, Ye C, Aird D, Stevens C, Robinson JT, Cabili MN, Gat-Viks I, Kelliher E, Daza R, DeFelice M, Hůlková H, Sovová J, Vylet'al P, Antignac C, Guttman M, Handsaker RE, Perrin D, Steelman S, Sigurdsson S, Scheinman SJ, Sougnez C, Cibulskis K, Parkin M, Green T, Rossin E, Zody MC, Xavier RJ, Pollak MR, Alper SL, Lindblad-Toh K, Gabriel S, Hart PS, Regev A, Nusbaum C, Kmoch S, Bleyer AJ, Lander ES, Daly MJ. Mutations causing medullary cystic kidney disease type 1 lie in a large VNTR in MUC1 missed by massively parallel sequencing. Nat Genet. 2013 Mar;45(3):299-303. doi: 10.1038/ng.2543. Epub 2013 Feb 10.
26	2015	gout	Germany	94	Kidney Int	Consensus Development Conference	25738250	Eckardt KU, Alper SL, Antignac C, Bleyer AJ, Chauveau D, Dahan K, Deltas C, Hosking A, Kmoch S, Rampoldi L, Wiesener M, Wolf MT, Devuyst O. Autosomal dominant tubulointerstitial kidney disease: diagnosis, classification, and management--A KDIGO consensus report. Kidney Int. 2015 Oct;88(4):676-83. doi: 10.1038/ki.2015.28. Epub 2015 Mar 4.
27	2014	therapeutic use	Colorado	92	N Engl J Med	Journal Article	25399731	Torres VE, Abebe KZ, Chapman AB, Schrier RW, Braun WE, Steinman TI, Winklhofer FT, Brosnahan G, Czarnecki PG, Hogan MC, Miskulin DC, Rahbari-Oskoui FF, Grantham JJ, Harris PC, Flessner MF, Moore CG, Perrone RD. Angiotensin blockade in late autosomal dominant polycystic kidney disease. N Engl J Med. 2014 Dec 11;371(24):2267-76. doi: 10.1056/NEJMoa1402686. Epub 2014 Nov 15
28	2012	genetics	U.S	90	Nat Genet	Journal Article	22246503	Lee JE, Silhavy JL, Zaki MS, Schroth J, Bielas SL, Marsh SE, Olvera J, Brancati F, Iannicelli M, Ikegami K, Schlossman AM, Merriman B, Attié-Bitach T, Logan CV, Glass IA, Cluckey A, Louie CM, Lee JH, Raynes HR, Rapin I, Castroviejo IP, Setou M, Barbot C, Boltshauser E, Nelson SF, Hildebrandt F, Johnson CA, Doherty DA, Valente EM, Gleeson JG. CEP41 is mutated in Joubert syndrome and is required for tubulin glutamylation at the cilium. Nat Genet. 2012 Jan 15;44(2):193-9. doi: 10.1038/ng.1078.
29	2011	genetics	Germany	89	J Am Soc Nephrol	Journal Article	22034641	Bergmann C, von Bothmer J, Ortiz Brüchle N, Venghaus A, Frank V, Fehrenbach H, Hampel T, Pape L, Buske A, Jonsson J, Sarioglu N, Santos A, Ferreira JC, Becker JU, Cremer R, Hoefele J, Benz MR, Weber LT, Buettner R, Zerres K. Mutations in multiple PKD genes may explain early and severe polycystic kidney disease. J Am Soc Nephrol. 2011 Nov;22(11):2047-56. doi: 10.1681/ASN.2010101080. Epub 2011 Oct 27.
30	2016	metabolism	U.S	85	Cell	Journal Article	27768895	Shen PS, Yang X, DeCaen PG, Liu X, Bulkley D, Clapham DE, Cao E. The Structure of the Polycystic Kidney Disease Channel PKD2 in Lipid Nanodiscs. Cell. 2016 Oct 20;167(3):763-773.e11. doi: 10.1016/j.cell.2016.09.048.
31	2016	algorithms	France	80	J Am Soc Nephrol	Journal Article	26150605	Cornec-Le Gall E, Audrézet MP, Rousseau A, Hourmant M, Renaudineau E, Charasse C, Morin MP, Moal MC, Dantal J, Wehbe B, Perrichot R, Frouget T, Vigneau C, Potier J, Jousset P, Guillodo MP, Siohan P, Terki N, Sawadogo T, Legrand D, Menoyo-Calonge V, Benarbia S, Besnier D, Longuet H, Férec C, Le Meur Y. The PROPKD Score: A New Algorithm to Predict Renal Survival in Autosomal Dominant Polycystic Kidney Disease. J Am Soc Nephrol. 2016 Mar;27(3):942-51. doi: 10.1681/ASN.2015010016. Epub 2015 Jul 6
32	2016	therapeutic use	Netherlands	79	Nephrol Dial Transplant	Journal Article	26908832	Gansevoort RT, Arici M, Benzing T, Birn H, Capasso G, Covic A, Devuyst O, Drechsler C, Eckardt KU, Emma F, Knebelmann B, Le Meur Y, Massy ZA, Ong AC, Ortiz A, Schaefer F, Torra R, Vanholder R, Wi?cek A, Zoccali C, Van Biesen W. Recommendations for the use of tolvaptan in autosomal dominant polycystic kidney disease: a position statement on behalf of the ERA-EDTA Working Groups on Inherited Kidney Disorders and European Renal Best Practice. Nephrol Dial Transplant. 2016 Mar;31(3):337-48. doi: 10.1093/ndt/gfv456. Epub 2016 Jan 29.
33	2011	cell movement	U.S	77	J Am Soc Nephrol	Journal Article	21921140	Karihaloo A, Koraishy F, Huen SC, Lee Y, Merrick D, Caplan MJ, Somlo S, Cantley LG. Macrophages promote cyst growth in polycystic kidney disease. J Am Soc Nephrol. 2011 Oct;22(10):1809-14. doi: 10.1681/ASN.2011010084. Epub 2011 Sep 15.
34	2010	physiology	U.S	73	J Am Soc Nephrol	Journal Article	19959710	Nishio S, Tian X, Gallagher AR, Yu Z, Patel V, Igarashi P, Somlo S. Loss of oriented cell division does not initiate cyst formation. J Am Soc Nephrol. 2010 Feb;21(2):295-302. doi: 10.1681/ASN.2009060603. Epub 2009 Dec 3
35	2014	mortality	Netherlands	72	Nephrol Dial Transplant	Journal Article	25165182	2014
36	2011	genetics	U.S	72	Am J Hum Genet	Journal Article	21763481	Dowdle WE, Robinson JF, Kneist A, Sirerol-Piquer MS, Frints SG, Corbit KC, Zaghloul NA, van Lijnschoten G, Mulders L, Verver DE, Zerres K, Reed RR, Attié-Bitach T, Johnson CA, García-Verdugo JM, Katsanis N, Bergmann C, Reiter JF. Disruption of a ciliary B9 protein complex causes Meckel syndrome. Am J Hum Genet. 2011 Jul 15;89(1):94-110. doi: 10.1016/j.ajhg.2011.06.003.
37	2010	polycystic kidney, autosomal dominant	U.S	72	Adv Chronic Kidney Dis	Journal Article	20219615	Gallagher AR, Germino GG, Somlo S. Molecular advances in autosomal dominant polycystic kidney disease. Adv Chronic Kidney Dis. 2010 Mar;17(2):118-30. doi: 10.1053/j.ackd.2010.01.002.
38	2015	adverse effects	U.S	71	Drug Saf	Case Reports	26188764	Watkins PB, Lewis JH, Kaplowitz N, Alpers DH, Blais JD, Smotzer DM, Krasa H, Ouyang J, Torres VE, Czerwiec FS, Zimmer CA. Clinical Pattern of Tolvaptan-Associated Liver Injury in Subjects with Autosomal Dominant Polycystic Kidney Disease: Analysis of Clinical Trials Database. Drug Saf. 2015 Nov;38(11):1103-13. doi: 10.1007/s40264-015-0327-3.
39	2013	genetics	U.S	71	Proc Natl Acad Sci U S A	Journal Article	23759744	Patel V, Williams D, Hajarnis S, Hunter R, Pontoglio M, Somlo S, Igarashi P. miR-17~92 miRNA cluster promotes kidney cyst growth in polycystic kidney disease. Proc Natl Acad Sci U S A. 2013 Jun 25;110(26):10765-70. doi: 10.1073/pnas.1301693110. Epub 2013 Jun 12.
40	2012	mutation	U.S	70	J Am Soc Nephrol	Journal Article	22383692	Rossetti S, Hopp K, Sikkink RA, Sundsbak JL, Lee YK, Kubly V, Eckloff BW, Ward CJ, Winearls CG, Torres VE, Harris PC. Identification of gene mutations in autosomal dominant polycystic kidney disease through targeted resequencing. J Am Soc Nephrol. 2012 May;23(5):915-33. doi: 10.1681/ASN.2011101032. Epub 2012 Mar 1
41	2011	antidiuretic hormone receptor antagonists	U.S	68	Am J Physiol Renal Physiol	Journal Article	21816754	Reif GA, Yamaguchi T, Nivens E, Fujiki H, Pinto CS, Wallace DP. Tolvaptan inhibits ERK-dependent cell proliferation, Cl??secretion, and in vitro cyst growth of human ADPKD cells stimulated by vasopressin. Am J Physiol Renal Physiol. 2011 Nov;301(5):F1005-13. doi: 10.1152/ajprenal.00243.2011. Epub 2011 Aug 3.
42	2016	mutation	U.S	67	J Am Soc Nephrol	Journal Article	26823553	Heyer CM, Sundsbak JL, Abebe KZ, Chapman AB, Torres VE, Grantham JJ, Bae KT, Schrier RW, Perrone RD, Braun WE, Steinman TI, Mrug M, Yu AS, Brosnahan G, Hopp K, Irazabal MV, Bennett WM, Flessner MF, Moore CG, Landsittel D, Harris PC. Predicted Mutation Strength of Nontruncating PKD1 Mutations Aids Genotype-Phenotype Correlations in Autosomal Dominant Polycystic Kidney Disease. J Am Soc Nephrol. 2016 Sep;27(9):2872-84. doi: 10.1681/ASN.2015050583. Epub 2016 Jan 28
43	2017	cellular microenvironment	U.S	65	Nat Mater	Journal Article	28967916	Cruz NM, Song X, Czerniecki SM, Gulieva RE, Churchill AJ, Kim YK, Winston K, Tran LM, Diaz MA, Fu H, Finn LS, Pei Y, Himmelfarb J, Freedman BS. Organoid cystogenesis reveals a critical role of microenvironment in human polycystic kidney?disease. Nat Mater. 2017 Nov;16(11):1112-1119. doi: 10.1038/nmat4994. Epub 2017 Oct 2.
44	2012	methods	France	65	Hum Mutat	Journal Article	22508176	Audrézet MP, Cornec-Le Gall E, Chen JM, Redon S, Quéré I, Creff J, Bénech C, Maestri S, Le Meur Y, Férec C. Autosomal dominant polycystic kidney disease: comprehensive mutation analysis of PKD1 and PKD2 in 700 unrelated patients. Hum Mutat. 2012 Aug;33(8):1239-50. doi: 10.1002/humu.22103. Epub 2012 May 24.
45	2010	physiology	France	65	Nat Med	Journal Article	19966811	Verdeguer F, Le Corre S, Fischer E, Callens C, Garbay S, Doyen A, Igarashi P, Terzi F, Pontoglio M. A mitotic transcriptional switch in polycystic kidney disease. Nat Med. 2010 Jan;16(1):106-10. doi: 10.1038/nm.2068. Epub 2009 Dec 6.
46	2018	diagnosis	Germany	64	Nat Rev Dis Primers	Journal Article	30523303	Bergmann C, Guay-Woodford LM, Harris PC, Horie S, Peters DJM, Torres VE. Polycystic kidney disease. Nat Rev Dis Primers. 2018 Dec 6;4(1):50. doi: 10.1038/s41572-018-0047-y.
47	2013	metabolism	U.S	64	Mol Cell	Journal Article	23973373	Choi HJ, Lin JR, Vannier JB, Slaats GG, Kile AC, Paulsen RD, Manning DK, Beier DR, Giles RH, Boulton SJ, Cimprich KA. NEK8 links the ATR-regulated replication stress response and S phase CDK activity to renal ciliopathies. Mol Cell. 2013 Aug 22;51(4):423-39. doi: 10.1016/j.molcel.2013.08.006.
48	2011	prevention & control	U.S	64	Hum Mol Genet	Journal Article	21821671	Takakura A, Nelson EA, Haque N, Humphreys BD, Zandi-Nejad K, Frank DA, Zhou J. Pyrimethamine inhibits adult polycystic kidney disease by modulating STAT signaling pathways. Hum Mol Genet. 2011 Nov 1;20(21):4143-54. doi: 10.1093/hmg/ddr338. Epub 2011 Aug 5.
49	2011	physiology	U.S	64	Biochim Biophys Acta	Journal Article	21118718	Wallace DP. Cyclic AMP-mediated cyst expansion. Biochim Biophys Acta. 2011 Oct;1812(10):1291-300. doi: 10.1016/j.bbadis.2010.11.005. Epub 2010 Nov 28.
50	2010	disease progression	Italy	64	J Am Soc Nephrol	Journal Article	20466742	Perico N, Antiga L, Caroli A, Ruggenenti P, Fasolini G, Cafaro M, Ondei P, Rubis N, Diadei O, Gherardi G, Prandini S, Panozo A, Bravo RF, Carminati S, De Leon FR, Gaspari F, Cortinovis M, Motterlini N, Ene-Iordache B, Remuzzi A, Remuzzi G. Sirolimus therapy to halt the progression of ADPKD. J Am Soc Nephrol. 2010 Jun;21(6):1031-40. doi: 10.1681/ASN.2009121302. Epub 2010 May 13
51	2010	alleles	Sweden	63	J Am Soc Nephrol	Case Reports	20558538	Vujic M, Heyer CM, Ars E, Hopp K, Markoff A, Orndal C, Rudenhed B, Nasr SH, Torres VE, Torra R, Bogdanova N, Harris PC. Incompletely penetrant PKD1 alleles mimic the renal manifestations of ARPKD. J Am Soc Nephrol. 2010 Jul;21(7):1097-102. doi: 10.1681/ASN.2009101070. Epub 2010 Jun 17.
52	2017	statistics & numerical data	U.S	62	Nephrol Dial Transplant	Journal Article	27325254	Willey CJ, Blais JD, Hall AK, Krasa HB, Makin AJ, Czerwiec FS. Prevalence of autosomal dominant polycystic kidney disease in the European Union. Nephrol Dial Transplant. 2017 Aug 1;32(8):1356-1363. doi: 10.1093/ndt/gfw240.
53	2015	metabolism	U.K	61	Kidney Int	Journal Article	26200945	Ong AC, Harris PC. A polycystin-centric view of cyst formation and disease: the polycystins revisited. Kidney Int. 2015 Oct;88(4):699-710. doi: 10.1038/ki.2015.207. Epub 2015 Jul 22.
54	2014	metabolism	U.S	61	J Clin Invest	Journal Article	25365220	Cai Y, Fedeles SV, Dong K, Anyatonwu G, Onoe T, Mitobe M, Gao JD, Okuhara D, Tian X, Gallagher AR, Tang Z, Xie X, Lalioti MD, Lee AH, Ehrlich BE, Somlo S. Altered trafficking and stability of polycystins underlie polycystic kidney disease. J Clin Invest. 2014 Dec;124(12):5129-44. doi: 10.1172/JCI67273. Epub 2014 Nov 3.
55	2013	pharmacology	U.S	61	J Clin Invest	Journal Article	23778143	Zhou X, Fan LX, Sweeney WE Jr, Denu JM, Avner ED, Li X. Sirtuin 1 inhibition delays cyst formation in autosomal-dominant polycystic kidney disease. J Clin Invest. 2013 Jul;123(7):3084-98. doi: 10.1172/JCI64401. Epub 2013 Jun 17.
56	2013	immunology	U.S	61	Kidney Int	Journal Article	23423256	Swenson-Fields KI, Vivian CJ, Salah SM, Peda JD, Davis BM, van Rooijen N, Wallace DP, Fields TA. Macrophages promote polycystic kidney disease progression. Kidney Int. 2013 May;83(5):855-64. doi: 10.1038/ki.2012.446. Epub 2013 Feb 20.
57	2010	pharmacology	U.S	61	Nat Med	Comparative Study	20562878	Natoli TA, Smith LA, Rogers KA, Wang B, Komarnitsky S, Budman Y, Belenky A, Bukanov NO, Dackowski WR, Husson H, Russo RJ, Shayman JA, Ledbetter SR, Leonard JP, Ibraghimov-Beskrovnaya O. Inhibition of glucosylceramide accumulation results in effective blockade of polycystic kidney disease in mouse models. Nat Med. 2010 Jul;16(7):788-92. doi: 10.1038/nm.2171. Epub 2010 Jun 20.
58	2016	therapeutic use	U.S	60	Am J Kidney Dis	Journal Article	26530876	Chebib FT, Torres VE. Autosomal Dominant Polycystic Kidney Disease: Core Curriculum 2016. Am J Kidney Dis. 2016 May;67(5):792-810. doi: 10.1053/j.ajkd.2015.07.037. Epub 2015 Oct 31.
59	2011	pharmacology	Netherlands	60	Am J Physiol Renal Physiol	Journal Article	21345977	Leonhard WN, van der Wal A, Novalic Z, Kunnen SJ, Gansevoort RT, Breuning MH, de Heer E, Peters DJ. Curcumin inhibits cystogenesis by simultaneous interference of multiple signaling pathways: in vivo evidence from a Pkd1-deletion model. Am J Physiol Renal Physiol. 2011 May;300(5):F1193-202. doi: 10.1152/ajprenal.00419.2010. Epub 2011 Feb 23.
60	2010	angiotensin receptor antagonists	U.S	60	Clin J Am Soc Nephrol	Journal Article	20089507	Chapman AB, Torres VE, Perrone RD, Steinman TI, Bae KT, Miller JP, Miskulin DC, Rahbari Oskoui F, Masoumi A, Hogan MC, Winklhofer FT, Braun W, Thompson PA, Meyers CM, Kelleher C, Schrier RW. The HALT polycystic kidney disease trials: design and implementation. Clin J Am Soc Nephrol. 2010 Jan;5(1):102-9. doi: 10.2215/CJN.04310709.
61	2010	metabolism	U.S	59	Dis Model Mech	Journal Article	20335443	Mangos S, Lam PY, Zhao A, Liu Y, Mudumana S, Vasilyev A, Liu A, Drummond IA. The ADPKD genes pkd1a/b and pkd2 regulate extracellular matrix formation. Dis Model Mech. 2010 May-Jun;3(5-6):354-65. doi: 10.1242/dmm.003194. Epub 2010 Mar 24
62	2014	genetics	U.S	58	Mod Pathol	Case Reports	24481001	Doros LA, Rossi CT, Yang J, Field A, Williams GM, Messinger Y, Cajaiba MM, Perlman EJ, A Schultz K, Cathro HP, Legallo RD, LaFortune KA, Chikwava KR, Faria P, Geller JI, Dome JS, Mullen EA, Gratias EJ, Dehner LP, Hill DA. DICER1 mutations in childhood cystic nephroma and its relationship to DICER1-renal sarcoma. Mod Pathol. 2014 Sep;27(9):1267-80. doi: 10.1038/modpathol.2013.242. Epub 2014 Jan 31
63	2011	metabolism	Netherlands	58	J Pathol	Journal Article	21381034	Happé H, van der Wal AM, Leonhard WN, Kunnen SJ, Breuning MH, de Heer E, Peters DJ. Altered Hippo signalling in polycystic kidney disease. J Pathol. 2011 May;224(1):133-42. doi: 10.1002/path.2856. Epub 2011 Mar 7.
64	2010	diagnosis	U.S	58	Nat Rev Nephrol	Journal Article	20177400	Harris PC, Rossetti S. Molecular diagnostics for autosomal dominant polycystic kidney disease. Nat Rev Nephrol. 2010 Apr;6(4):197-206. doi: 10.1038/nrneph.2010.18. Epub 2010 Feb 23
65	2018	diagnosis	U.S	57	J Am Soc Nephrol	Journal Article	29038287	Cornec-Le Gall E, Torres VE, Harris PC. Genetic Complexity of Autosomal Dominant Polycystic Kidney and Liver Diseases. J Am Soc Nephrol. 2018 Jan;29(1):13-23. doi: 10.1681/ASN.2017050483. Epub 2017 Oct 16
66	2018	alleles	U.S	56	Am J Hum Genet	Journal Article	29706351	Cornec-Le Gall E, Olson RJ, Besse W, Heyer CM, Gainullin VG, Smith JM, Audrézet MP, Hopp K, Porath B, Shi B, Baheti S, Senum SR, Arroyo J, Madsen CD, Férec C, Joly D, Jouret F, Fikri-Benbrahim O, Charasse C, Coulibaly JM, Yu AS, Khalili K, Pei Y, Somlo S, Le Meur Y, Torres VE, Harris PC. Monoallelic Mutations to DNAJB11 Cause Atypical Autosomal-Dominant Polycystic Kidney Disease. Am J Hum Genet. 2018 May 3;102(5):832-844. doi: 10.1016/j.ajhg.2018.03.013. Epub 2018 Apr 26.
67	2014	pathology	U.S	55	J Am Soc Nephrol	Journal Article	24925719	Schrier RW, Brosnahan G, Cadnapaphornchai MA, Chonchol M, Friend K, Gitomer B, Rossetti S. Predictors of autosomal dominant polycystic kidney disease progression. J Am Soc Nephrol. 2014 Nov;25(11):2399-418. doi: 10.1681/ASN.2013111184. Epub 2014 Jun 12.
68	2011	etiology	U.K	55	Biochim Biophys Acta	Journal Article	21745567	Norman J. Fibrosis and progression of autosomal dominant polycystic kidney disease (ADPKD). Biochim Biophys Acta. 2011 Oct;1812(10):1327-36. doi: 10.1016/j.bbadis.2011.06.012. Epub 2011 Jul 1.
69	2015	metabolism	U.S	54	J Cell Biol	Journal Article	25869670	Roberson EC, Dowdle WE, Ozanturk A, Garcia-Gonzalo FR, Li C, Halbritter J, Elkhartoufi N, Porath JD, Cope H, Ashley-Koch A, Gregory S, Thomas S, Sayer JA, Saunier S, Otto EA, Katsanis N, Davis EE, Attié-Bitach T, Hildebrandt F, Leroux MR, Reiter JF. TMEM231, mutated in orofaciodigital and Meckel syndromes, organizes the ciliary transition zone. J Cell Biol. 2015 Apr 13;209(1):129-42. doi: 10.1083/jcb.201411087.
70	2014	drug effects	U.S	54	Clin J Am Soc Nephrol	Clinical Trial, Phase III	24721893	Cadnapaphornchai MA, George DM, McFann K, Wang W, Gitomer B, Strain JD, Schrier RW. Effect of pravastatin on total kidney volume, left ventricular mass index, and microalbuminuria in pediatric autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 2014 May;9(5):889-96. doi: 10.2215/CJN.08350813. Epub 2014 Apr 10.
71	2018	chemistry	China	53	Science	Journal Article	30093605	Su Q, Hu F, Ge X, Lei J, Yu S, Wang T, Zhou Q, Mei C, Shi Y. Structure of the human PKD1-PKD2 complex. Science. 2018 Sep 7;361(6406):eaat9819. doi: 10.1126/science.aat9819. Epub 2018 Aug 9
72	2017	metabolism	U.S	53	Nat Commun	Journal Article	28205547	Hajarnis S, Lakhia R, Yheskel M, Williams D, Sorourian M, Liu X, Aboudehen K, Zhang S, Kersjes K, Galasso R, Li J, Kaimal V, Lockton S, Davis S, Flaten A, Johnson JA, Holland WL, Kusminski CM, Scherer PE, Harris PC, Trudel M, Wallace DP, Igarashi P, Lee EC, Androsavich JR, Patel V. microRNA-17 family promotes polycystic kidney disease progression through modulation of mitochondrial metabolism. Nat Commun. 2017 Feb 16;8:14395. doi: 10.1038/ncomms14395.
73	2011	phenotype	U.S	53	PLoS Genet	Journal Article	21490950	Fogelgren B, Lin SY, Zuo X, Jaffe KM, Park KM, Reichert RJ, Bell PD, Burdine RD, Lipschutz JH. The exocyst protein Sec10 interacts with Polycystin-2 and knockdown causes PKD-phenotypes. PLoS Genet. 2011 Apr;7(4):e1001361. doi: 10.1371/journal.pgen.1001361. Epub 2011 Apr 7
74	2011	kidney	U.S	53	Clin J Am Soc Nephrol	Journal Article	21088290	Torres VE, Grantham JJ, Chapman AB, Mrug M, Bae KT, King BF Jr, Wetzel LH, Martin D, Lockhart ME, Bennett WM, Moxey-Mims M, Abebe KZ, Lin Y, Bost JE. Potentially modifiable factors affecting the progression of autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 2011 Mar;6(3):640-7. doi: 10.2215/CJN.03250410. Epub 2010 Nov 18
75	2010	therapeutic use	U.S	53	Adv Chronic Kidney Dis	Journal Article	20219618	Chapman AB, Stepniakowski K, Rahbari-Oskoui F. Hypertension in autosomal dominant polycystic kidney disease. Adv Chronic Kidney Dis. 2010 Mar;17(2):153-63. doi: 10.1053/j.ackd.2010.01.001.
76	2015	classification	Germany	52	Pediatr Nephrol	Journal Article	24584572	Bergmann C. ARPKD and early manifestations of ADPKD: the original polycystic kidney disease and phenocopies. Pediatr Nephrol. 2015 Jan;30(1):15-30. doi: 10.1007/s00467-013-2706-2. Epub 2014 Mar 1
77	2011	antidiuretic hormone receptor antagonists	Japan	52	Clin J Am Soc Nephrol	Controlled Clinical Trial	21903984	Higashihara E, Torres VE, Chapman AB, Grantham JJ, Bae K, Watnick TJ, Horie S, Nutahara K, Ouyang J, Krasa HB, Czerwiec FS. Tolvaptan in autosomal dominant polycystic kidney disease: three years' experience. Clin J Am Soc Nephrol. 2011 Oct;6(10):2499-507. doi: 10.2215/CJN.03530411. Epub 2011 Sep 8.
78	2010	pathology	U.S	52	J Am Soc Nephrol	Journal Article	20705705	Luyten A, Su X, Gondela S, Chen Y, Rompani S, Takakura A, Zhou J. Aberrant regulation of planar cell polarity in polycystic kidney disease. J Am Soc Nephrol. 2010 Sep;21(9):1521-32. doi: 10.1681/ASN.2010010127. Epub 2010 Aug 12
79	2016	genetic association studies	South Korea	51	J Am Soc Nephrol	Journal Article	26453610	Hwang YH, Conklin J, Chan W, Roslin NM, Liu J, He N, Wang K, Sundsbak JL, Heyer CM, Haider M, Paterson AD, Harris PC, Pei Y. Refining Genotype-Phenotype Correlation in Autosomal Dominant Polycystic Kidney Disease. J Am Soc Nephrol. 2016 Jun;27(6):1861-8. doi: 10.1681/ASN.2015060648. Epub 2015 Oct 9
80	2013	pathology	U.S	51	J Am Soc Nephrol	Journal Article	24009235	Freedman BS, Lam AQ, Sundsbak JL, Iatrino R, Su X, Koon SJ, Wu M, Daheron L, Harris PC, Zhou J, Bonventre JV. Reduced ciliary polycystin-2 in induced pluripotent stem cells from polycystic kidney disease patients with PKD1 mutations. J Am Soc Nephrol. 2013 Oct;24(10):1571-86. doi: 10.1681/ASN.2012111089. Epub 2013 Sep 5.
81	2011	antidiuretic hormone receptor antagonists	U.S	51	Am J Kidney Dis	Comparative Study	21333426	Torres VE, Meijer E, Bae KT, Chapman AB, Devuyst O, Gansevoort RT, Grantham JJ, Higashihara E, Perrone RD, Krasa HB, Ouyang JJ, Czerwiec FS. Rationale and design of the TEMPO (Tolvaptan Efficacy and Safety in Management of Autosomal Dominant Polycystic Kidney Disease and its Outcomes) 3-4 Study. Am J Kidney Dis. 2011 May;57(5):692-9. doi: 10.1053/j.ajkd.2010.11.029. Epub 2011 Feb 17.
82	2010	drug therapy	Italy	51	Clin J Am Soc Nephrol	Journal Article	20185596	Caroli A, Antiga L, Cafaro M, Fasolini G, Remuzzi A, Remuzzi G, Ruggenenti P. Reducing polycystic liver volume in ADPKD: effects of somatostatin analogue octreotide. Clin J Am Soc Nephrol. 2010 May;5(5):783-9. doi: 10.2215/CJN.05380709. Epub 2010 Feb 25
83	2016	food	U.S	50	J Am Soc Nephrol	Journal Article	26538633	Warner G, Hein KZ, Nin V, Edwards M, Chini CC, Hopp K, Harris PC, Torres VE, Chini EN. Food Restriction Ameliorates the Development of Polycystic Kidney Disease. J Am Soc Nephrol. 2016 May;27(5):1437-47. doi: 10.1681/ASN.2015020132. Epub 2015 Nov 4
84	2013	physiopathology	U.S	50	Gastroenterology	Clinical Trial	23041322	Gunay-Aygun M, Font-Montgomery E, Lukose L, Tuchman Gerstein M, Piwnica-Worms K, Choyke P, Daryanani KT, Turkbey B, Fischer R, Bernardini I, Sincan M, Zhao X, Sandler NG, Roque A, Douek DC, Graf J, Huizing M, Bryant JC, Mohan P, Gahl WA, Heller T. Characteristics of congenital hepatic fibrosis in a large cohort of patients with autosomal recessive polycystic kidney disease. Gastroenterology. 2013 Jan;144(1):112-121.e2. doi: 10.1053/j.gastro.2012.09.056. Epub 2012 Oct 3.
85	2019	polycystic kidney, autosomal	France	49	Lancet	Journal Article	30819518	Cornec-Le Gall E, Alam A, Perrone RD. Autosomal dominant polycystic kidney disease. Lancet. 2019 Mar 2;393(10174):919-935. doi: 10.1016/S0140-6736(18)32782-X. Epub 2019 Feb 25.
86	2011	epidemiology	China	49	Stroke	Clinical Trial	21164130	Xu HW, Yu SQ, Mei CL, Li MH. Screening for intracranial aneurysm in 355 patients with autosomal-dominant polycystic kidney disease. Stroke. 2011 Jan;42(1):204-6. doi: 10.1161/STROKEAHA.110.578740. Epub 2010 Dec 16.
87	2013	drug therapy	U.S	48	Hepatology	Comparative Study	23172758	Masyuk TV, Radtke BN, Stroope AJ, Banales JM, Gradilone SA, Huang B, Masyuk AI, Hogan MC, Torres VE, Larusso NF. Pasireotide is more effective than octreotide in reducing hepatorenal cystogenesis in rodents with polycystic kidney and liver diseases. Hepatology. 2013 Jul;58(1):409-21. doi: 10.1002/hep.26140. Epub 2013 Mar 6.
88	2011	etiology	U.S	48	Clin J Am Soc Nephrol	Journal Article	20829421	Menon V, Rudym D, Chandra P, Miskulin D, Perrone R, Sarnak M. Inflammation, oxidative stress, and insulin resistance in polycystic kidney disease. Clin J Am Soc Nephrol. 2011 Jan;6(1):7-13. doi: 10.2215/CJN.04140510. Epub 2010 Sep 9
89	2015	metabolism	U.S	47	J Am Soc Nephrol	Comparative Study	25475747	Hogan MC, Bakeberg JL, Gainullin VG, Irazabal MV, Harmon AJ, Lieske JC, Charlesworth MC, Johnson KL, Madden BJ, Zenka RM, McCormick DJ, Sundsbak JL, Heyer CM, Torres VE, Harris PC, Ward CJ. Identification of Biomarkers for PKD1 Using Urinary Exosomes. J Am Soc Nephrol. 2015 Jul;26(7):1661-70. doi: 10.1681/ASN.2014040354. Epub 2014 Dec 4
90	2011	autophagy	U.S	47	Am J Physiol Renal Physiol	Journal Article	21270095	Belibi F, Zafar I, Ravichandran K, Segvic AB, Jani A, Ljubanovic DG, Edelstein CL. Hypoxia-inducible factor-1α (HIF-1α) and autophagy in polycystic kidney disease (PKD). Am J Physiol Renal Physiol. 2011 May;300(5):F1235-43. doi: 10.1152/ajprenal.00348.2010. Epub 2011 Jan 26.
91	2010	cytology	U.S	47	Gastroenterology	Journal Article	20399209	Gradilone SA, Masyuk TV, Huang BQ, Banales JM, Lehmann GL, Radtke BN, Stroope A, Masyuk AI, Splinter PL, LaRusso NF. Activation of Trpv4 reduces the hyperproliferative phenotype of cystic cholangiocytes from an animal model of ARPKD. Gastroenterology. 2010 Jul;139(1):304-14.e2. doi: 10.1053/j.gastro.2010.04.010. Epub 2010 Apr 14.
92	2017	genetics	Singapore	46	Nat Genet	Journal Article	28530676	Lu H, Galeano MCR, Ott E, Kaeslin G, Kausalya PJ, Kramer C, Ortiz-Brüchle N, Hilger N, Metzis V, Hiersche M, Tay SY, Tunningley R, Vij S, Courtney AD, Whittle B, Wühl E, Vester U, Hartleben B, Neuber S, Frank V, Little MH, Epting D, Papathanasiou P, Perkins AC, Wright GD, Hunziker W, Gee HY, Otto EA, Zerres K, Hildebrandt F, Roy S, Wicking C, Bergmann C. Mutations in DZIP1L, which encodes a ciliary-transition-zone protein, cause autosomal recessive polycystic kidney disease. Nat Genet. 2017 Jul;49(7):1025-1034. doi: 10.1038/ng.3871. Epub 2017 May 22.
93	2016	therapeutic use	Italy	46	J Am Soc Nephrol	Journal Article	26534924	Chiaravalli M, Rowe I, Mannella V, Quilici G, Canu T, Bianchi V, Gurgone A, Antunes S, D'Adamo P, Esposito A, Musco G, Boletta A. 2-Deoxy-d-Glucose Ameliorates PKD Progression. J Am Soc Nephrol. 2016 Jul;27(7):1958-69. doi: 10.1681/ASN.2015030231. Epub 2015 Nov 3
94	2011	physiology	U.K	46	Biochim Biophys Acta	Journal Article	21658447	Wilson PD. Apico-basal polarity in polycystic kidney disease epithelia. Biochim Biophys Acta. 2011 Oct;1812(10):1239-48. doi: 10.1016/j.bbadis.2011.05.008. Epub 2011 Jun 1.
95	2016	metabolism	U.S	45	EBioMedicine	Journal Article	27077126	Menezes LF, Lin CC, Zhou F, Germino GG. Fatty Acid Oxidation is Impaired in An Orthologous Mouse Model of Autosomal Dominant Polycystic Kidney Disease. EBioMedicine. 2016 Jan 26;5:183-92. doi: 10.1016/j.ebiom.2016.01.027. eCollection 2016 Mar.
96	2015	physiology	U.S	45	Nat Rev Nephrol	Journal Article	25870007	Chebib FT, Sussman CR, Wang X, Harris PC, Torres VE. Vasopressin and disruption of calcium signalling in polycystic kidney disease. Nat Rev Nephrol. 2015 Aug;11(8):451-64. doi: 10.1038/nrneph.2015.39. Epub 2015 Apr 14
97	2010	urine	Netherlands	45	Am J Kidney Dis	Comparative Study	20888104	Meijer E, Boertien WE, Nauta FL, Bakker SJ, van Oeveren W, Rook M, van der Jagt EJ, van Goor H, Peters DJ, Navis G, de Jong PE, Gansevoort RT. Association of urinary biomarkers with disease severity in patients with autosomal dominant polycystic kidney disease: a cross-sectional analysis. Am J Kidney Dis. 2010 Nov;56(5):883-95. doi: 10.1053/j.ajkd.2010.06.023.
98	2016	genetics	Saudi Arabia	44	Genome Biol	Journal Article	27894351	Shaheen R, Szymanska K, Basu B, Patel N, Ewida N, Faqeih E, Al Hashem A, Derar N, Alsharif H, Aldahmesh MA, Alazami AM, Hashem M, Ibrahim N, Abdulwahab FM, Sonbul R, Alkuraya H, Alnemer M, Al Tala S, Al-Husain M, Morsy H, Seidahmed MZ, Meriki N, Al-Owain M, AlShahwan S, Tabarki B, Salih MA, Faquih T, El-Kalioby M, Ueffing M, Boldt K, Logan CV, Parry DA, Al Tassan N, Monies D, Megarbane A, Abouelhoda M, Halees A, Johnson CA, Alkuraya FS. Characterizing the morbid genome of ciliopathies. Genome Biol. 2016 Nov 28;17(1):242. doi: 10.1186/s13059-016-1099-5.
99	2016	therapeutic use	U.S	44	Clin J Am Soc Nephrol	Clinical Trial, Phase III	26912543	Torres VE, Higashihara E, Devuyst O, Chapman AB, Gansevoort RT, Grantham JJ, Perrone RD, Ouyang J, Blais JD, Czerwiec FS. Effect of Tolvaptan in Autosomal Dominant Polycystic Kidney Disease by CKD Stage: Results from the TEMPO 3:4 Trial. Clin J Am Soc Nephrol. 2016 May 6;11(5):803-11. doi: 10.2215/CJN.06300615. Epub 2016 Feb 23.