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Proximal tubule proliferation is insufficient to induce rapid cyst formation after cilia disruption.

Sharma N, Malarkey EB, Berbari NF, O'Connor AK, Vanden Heuvel GB, Mrug M, Yoder BK.

J Am Soc Nephrol. 2013 Feb;24(3):456-64. doi: 10.1681/ASN.2012020154. Epub 2013 Feb 14.


Differential expression of Cux-1 and p21 in polycystic kidneys from Pkd1 null and cpk mice.

Sharma M, Brantley JG, Alcalay NI, Zhou J, Heystek E, Maser RL, Vanden Heuvel GB.

Kidney Int. 2005 Feb;67(2):432-42.


Loss of primary cilia upregulates renal hypertrophic signaling and promotes cystogenesis.

Bell PD, Fitzgibbon W, Sas K, Stenbit AE, Amria M, Houston A, Reichert R, Gilley S, Siegal GP, Bissler J, Bilgen M, Chou PC, Guay-Woodford L, Yoder B, Haycraft CJ, Siroky B.

J Am Soc Nephrol. 2011 May;22(5):839-48. doi: 10.1681/ASN.2010050526. Epub 2011 Apr 14.


Renal cilia display length alterations following tubular injury and are present early in epithelial repair.

Verghese E, Weidenfeld R, Bertram JF, Ricardo SD, Deane JA.

Nephrol Dial Transplant. 2008 Mar;23(3):834-41. Epub 2007 Oct 25.


Ectopic expression of Cux1 is associated with reduced p27 expression and increased apoptosis during late stage cyst progression upon inactivation of Pkd1 in collecting ducts.

Paul BM, Vassmer D, Taylor A, Magenheimer L, Carlton CG, Piontek KB, Germino GG, Vanden Heuvel GB.

Dev Dyn. 2011 Jun;240(6):1493-501. doi: 10.1002/dvdy.22625. Epub 2011 Apr 4.


Toxic tubular injury in kidneys from Pkd1-deletion mice accelerates cystogenesis accompanied by dysregulated planar cell polarity and canonical Wnt signaling pathways.

Happé H, Leonhard WN, van der Wal A, van de Water B, Lantinga-van Leeuwen IS, Breuning MH, de Heer E, Peters DJ.

Hum Mol Genet. 2009 Jul 15;18(14):2532-42. doi: 10.1093/hmg/ddp190. Epub 2009 Apr 28.


Disruption of intraflagellar transport in adult mice leads to obesity and slow-onset cystic kidney disease.

Davenport JR, Watts AJ, Roper VC, Croyle MJ, van Groen T, Wyss JM, Nagy TR, Kesterson RA, Yoder BK.

Curr Biol. 2007 Sep 18;17(18):1586-94. Epub 2007 Sep 6.


Acute kidney injury and aberrant planar cell polarity induce cyst formation in mice lacking renal cilia.

Patel V, Li L, Cobo-Stark P, Shao X, Somlo S, Lin F, Igarashi P.

Hum Mol Genet. 2008 Jun 1;17(11):1578-90. doi: 10.1093/hmg/ddn045. Epub 2008 Feb 9.


Pkd1 inactivation induced in adulthood produces focal cystic disease.

Takakura A, Contrino L, Beck AW, Zhou J.

J Am Soc Nephrol. 2008 Dec;19(12):2351-63. doi: 10.1681/ASN.2007101139. Epub 2008 Sep 5.


Third-hit signaling in renal cyst formation.

Weimbs T.

J Am Soc Nephrol. 2011 May;22(5):793-5. doi: 10.1681/ASN.2011030284. Epub 2011 Apr 14. No abstract available.


The role of cilia in the pathogenesis of cystic kidney disease.

Dell KM.

Curr Opin Pediatr. 2015 Apr;27(2):212-8. doi: 10.1097/MOP.0000000000000187. Review.


Cdc42 deficiency causes ciliary abnormalities and cystic kidneys.

Choi SY, Chacon-Heszele MF, Huang L, McKenna S, Wilson FP, Zuo X, Lipschutz JH.

J Am Soc Nephrol. 2013 Sep;24(9):1435-50. doi: 10.1681/ASN.2012121236. Epub 2013 Jun 13.


Cystogenesis and elongated primary cilia in Tsc1-deficient distal convoluted tubules.

Armour EA, Carson RP, Ess KC.

Am J Physiol Renal Physiol. 2012 Aug 15;303(4):F584-92. doi: 10.1152/ajprenal.00141.2012. Epub 2012 Jun 6.


The tuberous sclerosis proteins regulate formation of the primary cilium via a rapamycin-insensitive and polycystin 1-independent pathway.

Hartman TR, Liu D, Zilfou JT, Robb V, Morrison T, Watnick T, Henske EP.

Hum Mol Genet. 2009 Jan 1;18(1):151-63. doi: 10.1093/hmg/ddn325. Epub 2008 Oct 9.


Polycystic kidneys caused by sustained expression of Cux1 isoform p75.

Cadieux C, Harada R, Paquet M, Côté O, Trudel M, Nepveu A, Bouchard M.

J Biol Chem. 2008 May 16;283(20):13817-24. doi: 10.1074/jbc.M709332200. Epub 2008 Mar 20.


Phosphoinositide 3-Kinase-C2α Regulates Polycystin-2 Ciliary Entry and Protects against Kidney Cyst Formation.

Franco I, Margaria JP, De Santis MC, Ranghino A, Monteyne D, Chiaravalli M, Pema M, Campa CC, Ratto E, Gulluni F, Perez-Morga D, Somlo S, Merlo GR, Boletta A, Hirsch E.

J Am Soc Nephrol. 2016 Apr;27(4):1135-44. doi: 10.1681/ASN.2014100967. Epub 2015 Aug 13.


Hyperglycemia in the absence of cilia accelerates cystogenesis and induces renal damage.

Sas KM, Yin H, Fitzgibbon WR, Baicu CF, Zile MR, Steele SL, Amria M, Saigusa T, Funk J, Bunni MA, Siegal GP, Siroky BJ, Bissler JJ, Bell PD.

Am J Physiol Renal Physiol. 2015 Jul 1;309(1):F79-87. doi: 10.1152/ajprenal.00652.2014. Epub 2015 Apr 22.


Scattered Deletion of PKD1 in Kidneys Causes a Cystic Snowball Effect and Recapitulates Polycystic Kidney Disease.

Leonhard WN, Zandbergen M, Veraar K, van den Berg S, van der Weerd L, Breuning M, de Heer E, Peters DJ.

J Am Soc Nephrol. 2015 Jun;26(6):1322-33. doi: 10.1681/ASN.2013080864. Epub 2014 Oct 31.

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