Cystic Kidney Diseases in Children and Adults: Differences and Gaps in Clinical Management
Hanna C, Iliuta I, Besse W, Mekahli D, Chebib F. Cystic Kidney Diseases in Children and Adults: Differences and Gaps in Clinical Management. Seminars In Nephrology 2023, 43: 151434. PMID: 37996359, DOI: 10.1016/j.semnephrol.2023.151434.Peer-Reviewed Original ResearchConceptsCystic kidney diseaseKidney diseaseAutosomal dominant polycystic kidney diseaseAutosomal-dominant polycystic kidney diseasePolycystic kidney diseaseClinical managementDifferential diagnosisProgressive autosomal dominant polycystic kidney diseaseWide differential diagnosisKidney cystsV2 receptor antagonistKidney cystic diseaseDiagnosis of inherited disordersNext-generation sequencingRadiologic characteristicsKidney functionExtrarenal manifestationsFuture pregnanciesUnderlying conditionCystic diseaseLikely diagnosisFamily historyExtrarenal featuresPediatric diagnosesRecurrence riskDnajb11-Kidney Disease Develops from Reduced Polycystin-1 Dosage but not Unfolded Protein Response in Mice
Roy S, Li Z, Guo Z, Long K, Rehrl S, Tian X, Dong K, Besse W. Dnajb11-Kidney Disease Develops from Reduced Polycystin-1 Dosage but not Unfolded Protein Response in Mice. Journal Of The American Society Of Nephrology 2023, 34: 1521-1534. PMID: 37332102, PMCID: PMC10482070, DOI: 10.1681/asn.0000000000000164.Peer-Reviewed Original ResearchConceptsUnfolded protein responseAutosomal dominant tubulointerstitial kidney diseaseAutosomal dominant polycystic kidney diseasePolycystin-1Autosomal-dominant polycystic kidney diseaseProtein responseTubulointerstitial kidney diseaseKidney diseaseRenal failureRenal failure in adulthoodPolycystic kidney diseaseUnfolded protein response activationFull-length proteinProteins polycystin-1C-terminal fragmentCystic kidneysSite of maturationCystic kidney dysplasiaKidney disease pathogenesisHeterozygous inactivating mutationsHsp40 cochaperonesEndoplasmic reticulumMouse model studiesConditional allelesDNAJB11
This site is protected by hCaptcha and its Privacy Policy and Terms of Service apply