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Items: 1 to 20 of 181

1.

Pkd1 and Nek8 mutations affect cell-cell adhesion and cilia in cysts formed in kidney organ cultures.

Natoli TA, Gareski TC, Dackowski WR, Smith L, Bukanov NO, Russo RJ, Husson H, Matthews D, Piepenhagen P, Ibraghimov-Beskrovnaya O.

Am J Physiol Renal Physiol. 2008 Jan;294(1):F73-83. Epub 2007 Oct 10.

2.

Development of polycystic kidney disease in juvenile cystic kidney mice: insights into pathogenesis, ciliary abnormalities, and common features with human disease.

Smith LA, Bukanov NO, Husson H, Russo RJ, Barry TC, Taylor AL, Beier DR, Ibraghimov-Beskrovnaya O.

J Am Soc Nephrol. 2006 Oct;17(10):2821-31. Epub 2006 Aug 23.

3.

Nek8 regulates the expression and localization of polycystin-1 and polycystin-2.

Sohara E, Luo Y, Zhang J, Manning DK, Beier DR, Zhou J.

J Am Soc Nephrol. 2008 Mar;19(3):469-76. doi: 10.1681/ASN.2006090985. Epub 2008 Jan 30.

4.

Defects in ciliary localization of Nek8 is associated with cystogenesis.

Trapp ML, Galtseva A, Manning DK, Beier DR, Rosenblum ND, Quarmby LM.

Pediatr Nephrol. 2008 Mar;23(3):377-87. doi: 10.1007/s00467-007-0692-y. Epub 2008 Jan 9.

PMID:
18189147
5.

Mutations in NEK8 link multiple organ dysplasia with altered Hippo signalling and increased c-MYC expression.

Frank V, Habbig S, Bartram MP, Eisenberger T, Veenstra-Knol HE, Decker C, Boorsma RA, Göbel H, Nürnberg G, Griessmann A, Franke M, Borgal L, Kohli P, Völker LA, Dötsch J, Nürnberg P, Benzing T, Bolz HJ, Johnson C, Gerkes EH, Schermer B, Bergmann C.

Hum Mol Genet. 2013 Jun 1;22(11):2177-85. doi: 10.1093/hmg/ddt070. Epub 2013 Feb 14.

PMID:
23418306
6.

Loss of the ciliary kinase Nek8 causes left-right asymmetry defects.

Manning DK, Sergeev M, van Heesbeen RG, Wong MD, Oh JH, Liu Y, Henkelman RM, Drummond I, Shah JV, Beier DR.

J Am Soc Nephrol. 2013 Jan;24(1):100-12. doi: 10.1681/ASN.2012050490.

7.

NEK8 links the ATR-regulated replication stress response and S phase CDK activity to renal ciliopathies.

Choi HJ, Lin JR, Vannier JB, Slaats GG, Kile AC, Paulsen RD, Manning DK, Beier DR, Giles RH, Boulton SJ, Cimprich KA.

Mol Cell. 2013 Aug 22;51(4):423-39. doi: 10.1016/j.molcel.2013.08.006.

8.

CDK inhibitors R-roscovitine and S-CR8 effectively block renal and hepatic cystogenesis in an orthologous model of ADPKD.

Bukanov NO, Moreno SE, Natoli TA, Rogers KA, Smith LA, Ledbetter SR, Oumata N, Galons H, Meijer L, Ibraghimov-Beskrovnaya O.

Cell Cycle. 2012 Nov 1;11(21):4040-6. doi: 10.4161/cc.22375. Epub 2012 Oct 3.

9.

Novel NEK8 Mutations Cause Severe Syndromic Renal Cystic Dysplasia through YAP Dysregulation.

Grampa V, Delous M, Zaidan M, Odye G, Thomas S, Elkhartoufi N, Filhol E, Niel O, Silbermann F, Lebreton C, Collardeau-Frachon S, Rouvet I, Alessandri JL, Devisme L, Dieux-Coeslier A, Cordier MP, Capri Y, Khung-Savatovsky S, Sigaudy S, Salomon R, Antignac C, Gubler MC, Benmerah A, Terzi F, Attié-Bitach T, Jeanpierre C, Saunier S.

PLoS Genet. 2016 Mar 11;12(3):e1005894. doi: 10.1371/journal.pgen.1005894. eCollection 2016 Mar.

10.

Long-lasting arrest of murine polycystic kidney disease with CDK inhibitor roscovitine.

Bukanov NO, Smith LA, Klinger KW, Ledbetter SR, Ibraghimov-Beskrovnaya O.

Nature. 2006 Dec 14;444(7121):949-52. Epub 2006 Nov 22.

PMID:
17122773
11.

Nedd9 restrains renal cystogenesis in Pkd1-/- mice.

Nikonova AS, Plotnikova OV, Serzhanova V, Efimov A, Bogush I, Cai KQ, Hensley HH, Egleston BL, Klein-Szanto A, Seeger-Nukpezah T, Golemis EA.

Proc Natl Acad Sci U S A. 2014 Sep 2;111(35):12859-64. doi: 10.1073/pnas.1405362111. Epub 2014 Aug 19.

12.

NEK8 mutations affect ciliary and centrosomal localization and may cause nephronophthisis.

Otto EA, Trapp ML, Schultheiss UT, Helou J, Quarmby LM, Hildebrandt F.

J Am Soc Nephrol. 2008 Mar;19(3):587-92. doi: 10.1681/ASN.2007040490. Epub 2008 Jan 16.

13.

Protein kinase X (PRKX) can rescue the effects of polycystic kidney disease-1 gene (PKD1) deficiency.

Li X, Burrow CR, Polgar K, Hyink DP, Gusella GL, Wilson PD.

Biochim Biophys Acta. 2008 Jan;1782(1):1-9. Epub 2007 Sep 29.

14.

Progressive development of polycystic kidney disease in the mouse model expressing Pkd1 extracellular domain.

Kurbegovic A, Trudel M.

Hum Mol Genet. 2013 Jun 15;22(12):2361-75. doi: 10.1093/hmg/ddt081. Epub 2013 Feb 25.

PMID:
23439951
15.

The Nek8 protein kinase, mutated in the human cystic kidney disease nephronophthisis, is both activated and degraded during ciliogenesis.

Zalli D, Bayliss R, Fry AM.

Hum Mol Genet. 2012 Mar 1;21(5):1155-71. doi: 10.1093/hmg/ddr544. Epub 2011 Nov 21.

16.

Differences in the timing and magnitude of Pkd1 gene deletion determine the severity of polycystic kidney disease in an orthologous mouse model of ADPKD.

Rogers KA, Moreno SE, Smith LA, Husson H, Bukanov NO, Ledbetter SR, Budman Y, Lu Y, Wang B, Ibraghimov-Beskrovnaya O, Natoli TA.

Physiol Rep. 2016 Jun;4(12). pii: e12846. doi: 10.14814/phy2.12846.

17.

In vitro cystogenesis: the search for drugs antagonizing cyst development.

Ibraghimov-Beskrovnaya O, Bukanov NO.

Nephrol Ther. 2006 Jan;2 Suppl 2:S109-14.

PMID:
17373209
18.

Lowering of Pkd1 expression is sufficient to cause polycystic kidney disease.

Lantinga-van Leeuwen IS, Dauwerse JG, Baelde HJ, Leonhard WN, van de Wal A, Ward CJ, Verbeek S, Deruiter MC, Breuning MH, de Heer E, Peters DJ.

Hum Mol Genet. 2004 Dec 15;13(24):3069-77. Epub 2004 Oct 20.

PMID:
15496422
19.

A mouse model for polycystic kidney disease through a somatic in-frame deletion in the 5' end of Pkd1.

Starremans PG, Li X, Finnerty PE, Guo L, Takakura A, Neilson EG, Zhou J.

Kidney Int. 2008 Jun;73(12):1394-405. doi: 10.1038/ki.2008.111. Epub 2008 Apr 2.

20.

Triptolide reduces cyst formation in a neonatal to adult transition Pkd1 model of ADPKD.

Leuenroth SJ, Bencivenga N, Chahboune H, Hyder F, Crews CM.

Nephrol Dial Transplant. 2010 Jul;25(7):2187-94. doi: 10.1093/ndt/gfp777. Epub 2010 Feb 4.

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