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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.


The small GTPase Cdc42 is necessary for primary ciliogenesis in renal tubular epithelial cells.

Zuo X, Fogelgren B, Lipschutz JH.

J Biol Chem. 2011 Jun 24;286(25):22469-77. doi: 10.1074/jbc.M111.238469. Epub 2011 May 4.


The exocyst protein Sec10 interacts with Polycystin-2 and knockdown causes PKD-phenotypes.

Fogelgren B, Lin SY, Zuo X, Jaffe KM, Park KM, Reichert RJ, Bell PD, Burdine RD, Lipschutz JH.

PLoS Genet. 2011 Apr;7(4):e1001361. doi: 10.1371/journal.pgen.1001361. Epub 2011 Apr 7.


Dynamin Binding Protein (Tuba) Deficiency Inhibits Ciliogenesis and Nephrogenesis in Vitro and in Vivo.

Baek JI, Kwon SH, Zuo X, Choi SY, Kim SH, Lipschutz JH.

J Biol Chem. 2016 Apr 15;291(16):8632-43. doi: 10.1074/jbc.M115.688663. Epub 2016 Feb 19.


The exocyst protein Sec10 is necessary for primary ciliogenesis and cystogenesis in vitro.

Zuo X, Guo W, Lipschutz JH.

Mol Biol Cell. 2009 May;20(10):2522-9. doi: 10.1091/mbc.E08-07-0772. Epub 2009 Mar 18.


Exocyst Sec10 protects renal tubule cells from injury by EGFR/MAPK activation and effects on endocytosis.

Fogelgren B, Zuo X, Buonato JM, Vasilyev A, Baek JI, Choi SY, Chacon-Heszele MF, Palmyre A, Polgar N, Drummond I, Park KM, Lazzara MJ, Lipschutz JH.

Am J Physiol Renal Physiol. 2014 Dec 15;307(12):F1334-41. doi: 10.1152/ajprenal.00032.2014. Epub 2014 Oct 8.


Arl13b and the exocyst interact synergistically in ciliogenesis.

Seixas C, Choi SY, Polgar N, Umberger NL, East MP, Zuo X, Moreiras H, Ghossoub R, Benmerah A, Kahn RA, Fogelgren B, Caspary T, Lipschutz JH, Barral DC.

Mol Biol Cell. 2016 Jan 15;27(2):308-20. doi: 10.1091/mbc.E15-02-0061. Epub 2015 Nov 18.


Cdc42 and sec10 Are Required for Normal Retinal Development in Zebrafish.

Choi SY, Baek JI, Zuo X, Kim SH, Dunaief JL, Lipschutz JH.

Invest Ophthalmol Vis Sci. 2015 May;56(5):3361-70. doi: 10.1167/iovs.14-15692.


The exocyst gene Sec10 regulates renal epithelial monolayer homeostasis and apoptotic sensitivity.

Polgar N, Lee AJ, Lui VH, Napoli JA, Fogelgren B.

Am J Physiol Cell Physiol. 2015 Aug 1;309(3):C190-201. doi: 10.1152/ajpcell.00011.2015. Epub 2015 Jun 3.


Survivin-induced abnormal ploidy contributes to cystic kidney and aneurysm formation.

Aboualaiwi WA, Muntean BS, Ratnam S, Joe B, Liu L, Booth RL, Rodriguez I, Herbert BS, Bacallao RL, Fruttiger M, Mak TW, Zhou J, Nauli SM.

Circulation. 2014 Feb 11;129(6):660-72. doi: 10.1161/CIRCULATIONAHA.113.005746. Epub 2013 Nov 14.


Nephrocystin-3 is required for ciliary function in zebrafish embryos.

Zhou W, Dai J, Attanasio M, Hildebrandt F.

Am J Physiol Renal Physiol. 2010 Jul;299(1):F55-62. doi: 10.1152/ajprenal.00043.2010. Epub 2010 May 12.


The Ciliopathy Protein CC2D2A Associates with NINL and Functions in RAB8-MICAL3-Regulated Vesicle Trafficking.

Bachmann-Gagescu R, Dona M, Hetterschijt L, Tonnaer E, Peters T, de Vrieze E, Mans DA, van Beersum SE, Phelps IG, Arts HH, Keunen JE, Ueffing M, Roepman R, Boldt K, Doherty D, Moens CB, Neuhauss SC, Kremer H, van Wijk E.

PLoS Genet. 2015 Oct 20;11(10):e1005575. doi: 10.1371/journal.pgen.1005575. eCollection 2015 Oct.


The Rho family of small GTPases is involved in epithelial cystogenesis and tubulogenesis.

Rogers KK, Jou TS, Guo W, Lipschutz JH.

Kidney Int. 2003 May;63(5):1632-44.


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.


Disruption of IFT complex A causes cystic kidneys without mitotic spindle misorientation.

Jonassen JA, SanAgustin J, Baker SP, Pazour GJ.

J Am Soc Nephrol. 2012 Apr;23(4):641-51. doi: 10.1681/ASN.2011080829. Epub 2012 Jan 26.


Knockdown of bicaudal C in zebrafish (Danio rerio) causes cystic kidneys: a nonmammalian model of polycystic kidney disease.

Bouvrette DJ, Sittaramane V, Heidel JR, Chandrasekhar A, Bryda EC.

Comp Med. 2010 Apr;60(2):96-106.


Exocyst Sec10 protects epithelial barrier integrity and enhances recovery following oxidative stress, by activation of the MAPK pathway.

Park KM, Fogelgren B, Zuo X, Kim J, Chung DC, Lipschutz JH.

Am J Physiol Renal Physiol. 2010 Mar;298(3):F818-26. doi: 10.1152/ajprenal.00596.2009. Epub 2010 Jan 6.


Activation of ERK accelerates repair of renal tubular epithelial cells, whereas it inhibits progression of fibrosis following ischemia/reperfusion injury.

Jang HS, Han SJ, Kim JI, Lee S, Lipschutz JH, Park KM.

Biochim Biophys Acta. 2013 Dec;1832(12):1998-2008. doi: 10.1016/j.bbadis.2013.07.001. Epub 2013 Jul 10.


Early steps in primary cilium assembly require EHD1/EHD3-dependent ciliary vesicle formation.

Lu Q, Insinna C, Ott C, Stauffer J, Pintado PA, Rahajeng J, Baxa U, Walia V, Cuenca A, Hwang YS, Daar IO, Lopes S, Lippincott-Schwartz J, Jackson PK, Caplan S, Westlake CJ.

Nat Cell Biol. 2015 Mar;17(3):228-240. doi: 10.1038/ncb3109. Epub 2015 Feb 16. Erratum in: Nat Cell Biol. 2015 Apr;17(4):531.


The ciliopathy gene cc2d2a controls zebrafish photoreceptor outer segment development through a role in Rab8-dependent vesicle trafficking.

Bachmann-Gagescu R, Phelps IG, Stearns G, Link BA, Brockerhoff SE, Moens CB, Doherty D.

Hum Mol Genet. 2011 Oct 15;20(20):4041-55. doi: 10.1093/hmg/ddr332. Epub 2011 Aug 4.

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