Polycystin-1 cleavage and the regulation of transcriptional pathways
Merrick D, Bertuccio CA, Chapin HC, Lal M, Chauvet V, Caplan MJ. Polycystin-1 cleavage and the regulation of transcriptional pathways. Pediatric Nephrology 2013, 29: 505-511. PMID: 23824180, PMCID: PMC3844055, DOI: 10.1007/s00467-013-2548-y.Peer-Reviewed Original ResearchConceptsAutosomal dominant polycystic kidney diseaseFluid-filled renal cystsPolycystin-2Transcriptional pathwaysPolycystin-1Primary ciliaProtein productsPhysiological functionsCommon genetic causeParent proteinProteolytic cleavageCleavage fragmentsGenetic causeGenesEnd-stage renal diseaseDominant polycystic kidney diseasePolycystic kidney diseaseBiological activityPathwayRenal diseaseKidney diseaseCleavageRenal parenchymaFragmentsRenal cystsChapter 80 Autosomal Dominant Polycystic Kidney Disease
Somlo S, Torres V, Caplan M. Chapter 80 Autosomal Dominant Polycystic Kidney Disease. 2013, 2645-2688. DOI: 10.1016/b978-0-12-381462-3.00080-x.Peer-Reviewed Original ResearchAutosomal dominant polycystic kidney diseaseDominant polycystic kidney diseaseKidney diseasePolycystic kidney diseaseStructural kidney diseaseMultiple renal cystsAortic root dilatationTherapeutic clinical trialsMitral valve prolapseAbdominal wall herniasLiver bile ductsAge-dependent occurrenceBasic disease mechanismsNoncystic manifestationsRoot dilatationBile ductPolycystic liverSystemic disordersValve prolapseExtrarenal manifestationsClinical trialsPancreatic ductWall herniasRenal cystsClinical disease