2015
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. Phosphoinositide 3-Kinase-C2α Regulates Polycystin-2 Ciliary Entry and Protects against Kidney Cyst Formation. Journal Of The American Society Of Nephrology 2015, 27: 1135-1144. PMID: 26271513, PMCID: PMC4814170, DOI: 10.1681/asn.2014100967.Peer-Reviewed Original ResearchConceptsPI3K-C2αCiliary componentsPolycystin-2Primary ciliaRecycling endosome compartmentKidney cyst formationDuct 3 cellsCiliary entryCilium baseElongation defectsCargo proteinsCilium morphogenesisSubcellular locationPhosphoinositide 3Endosome compartmentTubule developmentProliferation signalsCiliary transportCyst formationCystic kidney diseaseIschemia/reperfusion-induced renal damageGenetic modelsCiliaCyst developmentKey mediator
2014
Cyst growth, polycystins, and primary cilia in autosomal dominant polycystic kidney disease
Lee SH, Somlo S. Cyst growth, polycystins, and primary cilia in autosomal dominant polycystic kidney disease. Kidney Research And Clinical Practice 2014, 33: 73-78. PMID: 26877954, PMCID: PMC4714135, DOI: 10.1016/j.krcp.2014.05.002.Peer-Reviewed Original ResearchPrimary ciliaAutosomal dominant polycystic kidney diseaseCarboxy-terminal tailDominant polycystic kidney diseaseExtracellular stimuliPolycystic kidney diseasePolycystin functionChannel proteinsPolycystinsPKD1 geneCystic kidney diseaseCiliaCalcium signalsRenal epitheliumProteinIntact ciliaKidney diseaseGenesCyst growthCurrent understandingReduced levelsPathwayComplete inactivationInactivationRecent dataFilling the Holes in Cystic Kidney Disease Research
Guay-Woodford LM, Henske E, Igarashi P, Perrone RD, Reed-Gitomer B, Somlo S, Torres VE, Ketchum CJ, Star RA, Flessner MF, Rasooly RS. Filling the Holes in Cystic Kidney Disease Research. Clinical Journal Of The American Society Of Nephrology 2014, 9: 1799-1801. PMID: 24903391, PMCID: PMC4186512, DOI: 10.2215/cjn.03410414.Peer-Reviewed Original ResearchConceptsKidney diseaseCystic kidney diseaseKidney Research National DialoguePublic health problemKidney disease researchKidney functionDisease progressionCystic diseaseHealth problemsDiseaseCyst formationNational InstituteDisease researchVariable responseDiabetesPathogenesisDigestiveProgressionPrevention