2025
Multicilia dynamically transduce Sonic Hedgehog signaling to regulate choroid plexus functions
Mao S, Song R, Jin S, Pang S, Jovanovic A, Zimmerman A, Li P, Wu X, Wendland M, Lin K, Chen W, Choksi S, Chen G, Holtzman M, Reiter J, Wan Y, Xuan Z, Xiang Y, Xu C, Upadhyayula S, Hess H, He L. Multicilia dynamically transduce Sonic Hedgehog signaling to regulate choroid plexus functions. Cell Reports 2025, 44: 115383. PMID: 40057957, DOI: 10.1016/j.celrep.2025.115383.Peer-Reviewed Original ResearchConceptsCSF productionChoroid plexusCerebrospinal fluidSonic hedgehog signalingWater channel AQP1Increased CSF productionHedgehog signalingChoroid plexus functionMotile ciliaMulticiliaSensory ciliaShh signalingNeonatal hydrocephalusSonic hedgehogCiliary lengthRegulate CSF productionSignal intensityCiliary ultrastructureChoroidEpithelial monolayersAQP1Developmental dynamicsCiliaATP1A2Plexus
2024
Fibroblast expression of transmembrane protein smoothened governs microenvironment characteristics after acute kidney injury
Gui Y, Fu H, Palanza Z, Tao J, Lin Y, Min W, Qiao Y, Bonin C, Hargis G, Wang Y, Yang P, Kreutzer D, Wang Y, Liu Y, Yu Y, Liu Y, Zhou D. Fibroblast expression of transmembrane protein smoothened governs microenvironment characteristics after acute kidney injury. Journal Of Clinical Investigation 2024, 134: e165836. PMID: 38713523, PMCID: PMC11213467, DOI: 10.1172/jci165836.Peer-Reviewed Original ResearchNidogen-1Expression of transmembrane proteinsCell-matrix interactionsAcute kidney injuryExtracellular matrix proteinsWnt signaling pathwayGlobal proteomeHedgehog signalingTransmembrane proteinsTubular cell apoptosisSignaling pathwayCell apoptosisMatrix proteinsIntegrin B1Kidney fibroblastsMesenchymal cell activationKidney injuryHedgehogProteinMitigate acute kidney injurySMOPreserved kidney functionAcute kidney injury pathogenesisFibroblastsPhosphoproteome
2022
Non-cell-autonomous activation of hedgehog signaling contributes to disease progression in a mouse model of renal cystic ciliopathy
Hsieh CL, Jerman SJ, Sun Z. Non-cell-autonomous activation of hedgehog signaling contributes to disease progression in a mouse model of renal cystic ciliopathy. Human Molecular Genetics 2022, 31: 4228-4240. PMID: 35904445, PMCID: PMC9759329, DOI: 10.1093/hmg/ddac175.Peer-Reviewed Original ResearchConceptsHh pathwayAutonomous activationMesenchymal cellsPolycystic kidney diseaseEpithelial cellsCre miceGli inhibitor GANT61Reporter mouse linePrimary ciliaHH signalingHedgehog signalingPKD pathogenesisArl13bSonic hedgehogMutant kidneysPKD modelPKD progressionHh activationKidney functionKidney diseaseCyst progressionCo-culture systemMouse linesMouse modelDistal nephron
2020
Hedgehog Signaling Demarcates a Niche of Fibrogenic Peribiliary Mesenchymal Cells
Gupta V, Gupta I, Park J, Bram Y, Schwartz RE. Hedgehog Signaling Demarcates a Niche of Fibrogenic Peribiliary Mesenchymal Cells. Gastroenterology 2020, 159: 624-638.e9. PMID: 32289375, PMCID: PMC8204800, DOI: 10.1053/j.gastro.2020.03.075.Peer-Reviewed Original ResearchConceptsCholestatic injuryStellate cellsLiver tissueStromal cellsLiver diseaseBile ductBiliary treePortal tractsMesenchymal cellsPrimary sclerosing cholangitisAlcoholic liver diseaseEpithelial cellsMyofibroblast phenotypeQuantitative reverse transcription polymerase chain reactionBile duct ligationReverse transcription-polymerase chain reactionTranscription-polymerase chain reactionCanals of HeringControl liver tissueHedgehog signalingSclerosing cholangitisHepatic injuryHepatocellular injuryNonalcoholic steatohepatitisPortal fibroblasts
2019
Cell-Autonomous Hedgehog Signaling Is Not Required for Cyst Formation in Autosomal Dominant Polycystic Kidney Disease
Ma M, Legué E, Tian X, Somlo S, Liem KF. Cell-Autonomous Hedgehog Signaling Is Not Required for Cyst Formation in Autosomal Dominant Polycystic Kidney Disease. Journal Of The American Society Of Nephrology 2019, 30: 2103-2111. PMID: 31451534, PMCID: PMC6830786, DOI: 10.1681/asn.2018121274.Peer-Reviewed Original ResearchConceptsHedgehog pathwayPolycystin-1Polycystin-2Autosomal dominant polycystic kidney diseaseMain causal genePolycystic kidney diseaseKidney cyst formationEpithelial cellsLevels of HedgehogCiliary genesDominant polycystic kidney diseaseMutant mouse kidneysRenal epithelial cellsCausal genesSignal transductionCell signalingGenetic manipulationPrimary ciliaCyst formationMultipass transmembraneHedgehog signalingConditional inactivationUnknown pathwayHedgehogKidney phenotype
2016
Polycomb-Mediated Repression and Sonic Hedgehog Signaling Interact to Regulate Merkel Cell Specification during Skin Development
Perdigoto CN, Dauber KL, Bar C, Tsai PC, Valdes VJ, Cohen I, Santoriello FJ, Zhao D, Zheng D, Hsu YC, Ezhkova E. Polycomb-Mediated Repression and Sonic Hedgehog Signaling Interact to Regulate Merkel Cell Specification during Skin Development. PLOS Genetics 2016, 12: e1006151. PMID: 27414999, PMCID: PMC4944976, DOI: 10.1371/journal.pgen.1006151.Peer-Reviewed Original ResearchConceptsMerkel cell specificationCell specificationPrimary hair folliclesLoss of PolycombImportance of ShhCell differentiation programHair follicle functionSonic hedgehog (Shh) signalingPRC2 targetsSpecialized keratinocytesEpidermal progenitorsDevelopmental programHair folliclesEpigenetic processesDifferentiation programHedgehog signalingShh ligandMature Merkel cellsShh signalingSkin developmentMurine dorsal skinEpidermal cellsMerkel cellsPRC2 lossCell formationDeletion of ADP Ribosylation Factor-Like GTPase 13B Leads to Kidney Cysts
Li Y, Tian X, Ma M, Jerman S, Kong S, Somlo S, Sun Z. Deletion of ADP Ribosylation Factor-Like GTPase 13B Leads to Kidney Cysts. Journal Of The American Society Of Nephrology 2016, 27: 3628-3638. PMID: 27153923, PMCID: PMC5118478, DOI: 10.1681/asn.2015091004.Peer-Reviewed Original ResearchConceptsSevere patterning defectsMultiple model organismsSmall GTPase essentialDefective hedgehog signalingCystic kidneysNumber of phenotypesKidney cyst formationKidney cystsJoubert syndromeGTPase essentialZebrafish leadsPatterning defectsBiogenesis defectsModel organismsCilia biogenesisLoss of functionCyst progressionDefective ciliaHistone deacetylase inhibitorsHuman mutationsNull mutationHedgehog signalingHypomorphic natureRescue experimentsNeural tube
2013
Heat‐shock–mediated conditional regulation of hedgehog/gli signaling in zebrafish
Shen M, Ozacar A, Osgood M, Boeras C, Pink J, Thomas J, Kohtz J, Karlstrom R. Heat‐shock–mediated conditional regulation of hedgehog/gli signaling in zebrafish. Developmental Dynamics 2013, 242: 539-549. PMID: 23441066, DOI: 10.1002/dvdy.23955.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsAnimals, Genetically ModifiedEmbryo, NonmammalianGene Expression Regulation, DevelopmentalGreen Fluorescent ProteinsHeat-Shock ResponseHedgehog ProteinsHSP70 Heat-Shock ProteinsOncogene ProteinsPromoter Regions, GeneticSignal TransductionTrans-ActivatorsZebrafishZebrafish ProteinsZinc Finger Protein GLI1ConceptsTransgenic linesConditional regulationHeat shockEmbryonic central nervous systemZebrafish transgenic linesNew reporter linesHeat shock activationInducible transgenic linesHedgehog/GLIStem cell proliferationSingle heat shockHh responseHh functionFunction phenotypesPostembryonic rolesReporter linesPromoter elementsHedgehog signalingJuvenile stageSitu hybridizationCell proliferationEarly embryonic lossQuantitative polymerase chain reactionLife cycleNew experimental tools
2011
A Novel Protein LZTFL1 Regulates Ciliary Trafficking of the BBSome and Smoothened
Seo S, Zhang Q, Bugge K, Breslow DK, Searby CC, Nachury MV, Sheffield VC. A Novel Protein LZTFL1 Regulates Ciliary Trafficking of the BBSome and Smoothened. PLOS Genetics 2011, 7: e1002358. PMID: 22072986, PMCID: PMC3207910, DOI: 10.1371/journal.pgen.1002358.Peer-Reviewed Original ResearchConceptsCiliary traffickingBBS proteinsBardet-Biedl syndrome proteinsLeucine zipper transcriptionHedgehog signal transducerG protein-coupled receptorsProtein-coupled receptorsCiliary entryBBSome subunitsProtein traffickingSyndrome proteinProtein complexesCellular processesNovel proteinPrimary ciliaHedgehog signalingSignal transducerBBSomeImportant regulatorTraffickingLZTFL1Ciliary functionProteinBBS3Cilia
2010
Activation of Hedgehog Signaling by the Environmental Toxicant Arsenic May Contribute to the Etiology of Arsenic-Induced Tumors
Fei D, Li H, Kozul C, Black K, Singh S, Gosse J, DiRenzo J, Martin K, Wang B, Hamilton J, Karagas M, Robbins D. Activation of Hedgehog Signaling by the Environmental Toxicant Arsenic May Contribute to the Etiology of Arsenic-Induced Tumors. Cancer Research 2010, 70: 1981-1988. PMID: 20179202, PMCID: PMC2831120, DOI: 10.1158/0008-5472.can-09-2898.Peer-Reviewed Original ResearchConceptsArsenic exposureBladder cancerEnvironmental toxicant arsenicBladder cancer patientsSignificant health problemVariety of tumorsHedgehog signalingCancer patientsHealth problemsHigh levelsTumor samplesCancerHedgehog activitySame cancerHuman carcinogenesisMillions of peopleEtiologyTumorsExposureProgressionHedgehogTissue culture cellsActivationSignalingPatients
2009
Cilia localization is essential for in vivo functions of the Joubert syndrome protein Arl13b/Scorpion
Duldulao NA, Lee S, Sun Z. Cilia localization is essential for in vivo functions of the Joubert syndrome protein Arl13b/Scorpion. Development 2009, 136: 4033-4042. PMID: 19906870, PMCID: PMC2778746, DOI: 10.1242/dev.036350.Peer-Reviewed Original ResearchConceptsCilia formationVivo functionJoubert syndromeSeries of deletionsSonic hedgehog (Shh) signalingCilia localizationCiliary localizationNull mutantsPoint mutantsGene productsHedgehog signalingArl13bCiliary defectsMutantsKidney ductsZebrafishCiliaAutosomal recessive disorderAbnormal ultrastructureScorpionsRecessive disorderLocalizationRecent studiesKnockdownSignaling
2002
HEDGEHOG SIGNALING AND HUMAN DISEASE
Bale AE. HEDGEHOG SIGNALING AND HUMAN DISEASE. Annual Review Of Genomics And Human Genetics 2002, 3: 47-65. PMID: 12142354, DOI: 10.1146/annurev.genom.3.022502.103031.Peer-Reviewed Original ResearchConceptsEarly Drosophila developmentHedgehog pathwayDrosophila developmentDevelopmental genesDrosophila melanogasterHuman embryogenesisHedgehog signalingDevelopmental pathwaysGenetic studiesHuman diseasesCell growthEmbryogenesisPathwayKey roleMelanogasterVertebratesImportant roleCentral nervous systemAxial skeletonGenesNervous systemSignalingMutationsDifferentiationRegulation
1999
The hedgehog signalling pathway in tumorigenesis and development
Wicking C, Smyth I, Bale A. The hedgehog signalling pathway in tumorigenesis and development. Oncogene 1999, 18: 7844-7851. PMID: 10630637, DOI: 10.1038/sj.onc.1203282.Peer-Reviewed Original ResearchConceptsDownstream targetsNovel downstream targetTumor formationEmbryonic patterningDysregulation of hedgehogResponsive genesHuman patched geneRange of tissuesHedgehog signalingConstitutive activationMolecular processesTumorigenesis resultsCell typesHedgehogCell surfaceReceptor complexPatched genePathwayGenesKey membersTumorigenesisSporadic formsDysregulationSignalingTumor types
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