2015
Human Polycystin-2 Transgene Dose-Dependently Rescues ADPKD Phenotypes in Pkd2 Mutant Mice
Li A, Tian X, Zhang X, Huang S, Ma Y, Wu D, Moeckel G, Somlo S, Wu G. Human Polycystin-2 Transgene Dose-Dependently Rescues ADPKD Phenotypes in Pkd2 Mutant Mice. American Journal Of Pathology 2015, 185: 2843-2860. PMID: 26435415, PMCID: PMC4607765, DOI: 10.1016/j.ajpath.2015.06.014.Peer-Reviewed Original ResearchConceptsAutosomal dominant polycystic kidney diseaseMouse modelADPKD phenotypeSevere cystic phenotypeWild-type miceDose-dependent mannerPolycystic kidney diseaseForms of ADPKDKidney diseasePancreatic cystsEffective treatmentFunctional restorationMutant miceTransgene doseMiceCyst formationReduced proliferationEpithelial cellsCystic phenotypeKidneyLiverFurther ameliorationPC2 activityPhenotypeMolecular genetic mechanisms
2009
Placental Insufficiency Associated with Loss of Cited1 Causes Renal Medullary Dysplasia
Sparrow DB, Boyle SC, Sams RS, Mazuruk B, Zhang L, Moeckel GW, Dunwoodie SL, de Caestecker MP. Placental Insufficiency Associated with Loss of Cited1 Causes Renal Medullary Dysplasia. Journal Of The American Society Of Nephrology 2009, 20: 777-786. PMID: 19297558, PMCID: PMC2663829, DOI: 10.1681/asn.2008050547.Peer-Reviewed Original ResearchConceptsRenal medullary dysplasiaPlacental insufficiencyTissue oxygenationLower urinary tract obstructionUrinary tract obstructionCITED1 expressionIntrauterine growth retardationTract obstructionRenal functionMedullary growthRenal dysplasiaDysplasiaGrowth retardationRenal medullaMutant miceDecreased numberInsufficiencyEarly nephrogenesisNumber of studiesMiceGenetic lossOxygenationUreteric budCircumstantial evidenceInfants