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
Fetal Brain-Derived Exosomal miRNAs from Maternal Blood: Potential Diagnostic Biomarkers for Fetal Alcohol Spectrum Disorders (FASDs)
Darbinian N, Hampe M, Martirosyan D, Bajwa A, Darbinyan A, Merabova N, Tatevosian G, Goetzl L, Amini S, Selzer M. Fetal Brain-Derived Exosomal miRNAs from Maternal Blood: Potential Diagnostic Biomarkers for Fetal Alcohol Spectrum Disorders (FASDs). International Journal Of Molecular Sciences 2024, 25: 5826. PMID: 38892014, PMCID: PMC11172088, DOI: 10.3390/ijms25115826.Peer-Reviewed Original ResearchFetal alcohol spectrum disordersEffects of EtOH exposureAt-risk fetusesNon-drinking controlsFetal brain tissueVoluntary pregnancy terminationGestational ageFetal sexEtOH exposureMaternal bloodMother's serumEtOH-exposedPregnancy terminationFetal brainDrank alcoholMatched controlsPotential diagnostic biomarkersBrain-derived exosomesSonic hedgehogNeurodevelopmental disabilitiesDiagnostic biomarkersProtein levelsNeurodevelopmental disordersSpectrum disorderBrain tissue
2022
Mispatterning and interneuron deficit in Tourette Syndrome basal ganglia organoids
Brady M, Mariani J, Koca Y, Szekely A, King R, Bloch M, Landeros-Weisenberger A, Leckman J, Vaccarino F. Mispatterning and interneuron deficit in Tourette Syndrome basal ganglia organoids. Molecular Psychiatry 2022, 27: 5007-5019. PMID: 36447010, PMCID: PMC9949887, DOI: 10.1038/s41380-022-01880-5.Peer-Reviewed Original ResearchConceptsTourette syndromeInterneuron deficitsGABAergic interneuronsHealthy controlsNeurodevelopmental underpinningsNeuropathological deficitsBG circuitryNeuropsychiatric disordersDecreased differentiationT patientsInterneuronsAltered expressionPotential mechanismsCilia disruptionSonic hedgehogOrganoidsStem cellsTS individualsPluripotent stem cellsGli transcription factorsDeficitsOrganoid differentiationEarly stagesCholinergicPatientsNon-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
2021
Role of SHH in Patterning Human Pluripotent Cells towards Ventral Forebrain Fates
Brady MV, Vaccarino FM. Role of SHH in Patterning Human Pluripotent Cells towards Ventral Forebrain Fates. Cells 2021, 10: 914. PMID: 33923415, PMCID: PMC8073580, DOI: 10.3390/cells10040914.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsSonic hedgehogHuman neurodevelopmentHuman pluripotent cellsDiverse cellular compositionRole of SHHMaster regulatorPluripotent cellsCellular phenotypesVentral identityNeural organoidsExpression gradientsOrganoid systemsDisease modelingVitro systemPathway activationModel systemCellular compositionOrganoidsHuman developmentQuestions scientistsHedgehogBiology
2018
The ESCRT-III Protein CHMP1A Mediates Secretion of Sonic Hedgehog on a Distinctive Subtype of Extracellular Vesicles
Coulter ME, Dorobantu CM, Lodewijk GA, Delalande F, Cianferani S, Ganesh VS, Smith RS, Lim ET, Xu CS, Pang S, Wong ET, Lidov HGW, Calicchio ML, Yang E, Gonzalez DM, Schlaeger TM, Mochida GH, Hess H, Lee WA, Lehtinen MK, Kirchhausen T, Haussler D, Jacobs FMJ, Gaudin R, Walsh CA. The ESCRT-III Protein CHMP1A Mediates Secretion of Sonic Hedgehog on a Distinctive Subtype of Extracellular Vesicles. Cell Reports 2018, 24: 973-986.e8. PMID: 30044992, PMCID: PMC6178983, DOI: 10.1016/j.celrep.2018.06.100.Peer-Reviewed Original ResearchConceptsExtracellular vesiclesMultivesicular bodiesIntraluminal vesicle formationESCRT proteinsHedgehog secretionProgenitor maintenanceMolecular mechanistic insightsComplex proteinsCell communicationEV subtypesVesicle formationChmp1AAutosomal recessive microcephalyHuman cerebral organoidsSonic hedgehogProgenitor proliferationFunction mutationsImpairs secretionAdult functionMechanistic insightsCerebral organoidsNull micePontocerebellar hypoplasiaShhProtein
2010
Neurotransmitter signaling in postnatal neurogenesis: The first leg
Platel JC, Stamboulian S, Nguyen I, Bordey A. Neurotransmitter signaling in postnatal neurogenesis: The first leg. Brain Research Reviews 2010, 63: 60-71. PMID: 20188124, PMCID: PMC2862802, DOI: 10.1016/j.brainresrev.2010.02.004.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsFate determinationSonic hedgehogNeurotransmitter signalingDifferent cell typesSubventricular zoneMultitude of receptorsMaster regulatorGenetic signaturesMosaic natureGamma-aminobutyric acidMicroenvironmental signalsCell typesIntracellular pathwaysAstrocyte-like cellsFunction of neurotransmittersHedgehogSignalingNeurogenesisNeurogenic zonesAdult brainPostnatal neurogenesisPeripheral organsNeurotransmitter functionSynaptic integrationGenes
2007
Sonic hedgehog acts at multiple stages during pancreatic tumorigenesis
Morton J, Mongeau M, Klimstra D, Morris J, Lee Y, Kawaguchi Y, Wright C, Hebrok M, Lewis B. Sonic hedgehog acts at multiple stages during pancreatic tumorigenesis. Proceedings Of The National Academy Of Sciences Of The United States Of America 2007, 104: 5103-5108. PMID: 17372229, PMCID: PMC1828712, DOI: 10.1073/pnas.0701158104.Peer-Reviewed Original ResearchConceptsShh signalingActivation of phosphatidylinositol 3-kinase signalingSonic hedgehogPhosphatidylinositol 3-kinase signalingStability of Bcl-2Pancreatic duct epithelial cellsActivation of sonic hedgehogPancreatic tumorigenesisActivated K-rasDuct epithelial cellsCell cycle regulator cyclin D1Epithelial cellsBcl-x(LTranscriptional regulationRegulator cyclin D1Dependence of tumor cellsPancreatic tumor developmentProliferation of pancreatic duct epithelial cellsBcl-2Signaling pathwayResistance to therapeutic interventionsShhShh expressionCyclin D1Stages of pancreatic carcinogenesisPromotion of Hras-induced squamous carcinomas by a polymorphic variant of the Patched gene in FVB mice
Wakabayashi Y, Mao JH, Brown K, Girardi M, Balmain A. Promotion of Hras-induced squamous carcinomas by a polymorphic variant of the Patched gene in FVB mice. Nature 2007, 445: 761-765. PMID: 17230190, DOI: 10.1038/nature05489.Peer-Reviewed Original ResearchMeSH KeywordsAmino Acid SequenceAnimalsApoptosisCarcinoma, Squamous CellCell LineCell Transformation, NeoplasticCrosses, GeneticFemaleGene Expression Regulation, NeoplasticGenes, rasHSP40 Heat-Shock ProteinsHumansKruppel-Like Transcription FactorsMaleMiceMice, Inbred C57BLMice, TransgenicMolecular Sequence DataPatched ReceptorsPatched-1 ReceptorPolymorphism, Geneticras ProteinsReceptors, Cell SurfaceZinc Finger Protein Gli2ConceptsSquamous carcinomaTumor suppressor geneFVB/N miceSonic hedgehogSuppressor geneFVB/N strainBasal cell carcinomaPTCH geneSame target cellsCell lineage commitmentPatched geneHuman patched geneHost genetic backgroundClassical tumor suppressor geneCell carcinomaPtch alleleFVB miceN miceCarcinogen exposureC57BL/6 strainTumor typesLineage commitmentMouse homologueHybrid miceGenetic basis
2002
Regulation of Gli1 Transcriptional Activity in the Nucleus by Dyrk1*
Mao J, Maye P, Kogerman P, Tejedor F, Toftgard R, Xie W, Wu G, Wu D. Regulation of Gli1 Transcriptional Activity in the Nucleus by Dyrk1*. Journal Of Biological Chemistry 2002, 277: 35156-35161. PMID: 12138125, DOI: 10.1074/jbc.m206743200.Peer-Reviewed Original ResearchMeSH Keywords3T3 CellsAnimalsCell DifferentiationCell NucleusCOS CellsGene Expression RegulationHedgehog ProteinsMiceOncogene ProteinsPhosphorylationPrecipitin TestsProtein Serine-Threonine KinasesProtein TransportProtein-Tyrosine KinasesSignal TransductionTrans-ActivatorsTranscription FactorsTranscription, GeneticZinc Finger Protein GLI1ConceptsTranscriptional activityGene transcriptionDual-specificity protein kinaseNuclear export mutantGLI1 transcriptional activityMouse C3H10T1/2 cellsReporter gene assayCellular rolesExport mutantsTranscriptional regulationProtein kinaseKinase activityC3H10T1/2 cellsLEF-1Kinase 1DYRK1Sonic hedgehogC-JunGene assayCell nucleiShhTranscriptionRegulationGli1Pathway
2001
Sonic hedgehog-dependent emergence of oligodendrocytes in the telencephalon: evidence for a source of oligodendrocytes in the olfactory bulb that is independent of PDGFRalpha signaling.
Spassky N, Heydon K, Mangatal A, Jankovski A, Olivier C, Queraud-Lesaux F, Goujet-Zalc C, Thomas J, Zalc B. Sonic hedgehog-dependent emergence of oligodendrocytes in the telencephalon: evidence for a source of oligodendrocytes in the olfactory bulb that is independent of PDGFRalpha signaling. Development 2001, 128: 4993-5004. PMID: 11748136, DOI: 10.1242/dev.128.24.4993.Peer-Reviewed Original ResearchConceptsSpinal cordPlatelet-derived growth factor receptorOlfactory bulbOligodendrocyte progenitorsInduction of oligodendrocytesSource of oligodendrocytesAnterior entopeduncular areaMedial ganglionic eminenceExpression of PLPGrowth factor receptorRostral palliumTelencephalic oligodendrocytesGanglionic eminenceVentricular focusOligodendroglial lineageCordRostral telencephalonOligodendrocytesOligodendrocyte lineageFactor receptorVentricular progenitorsTelencephalonMouse telencephalonSonic hedgehogMolecular mechanismsvhnf1, the MODY5 and familial GCKD-associated gene, regulates regional specification of the zebrafish gut, pronephros, and hindbrain
Sun Z, Hopkins N. vhnf1, the MODY5 and familial GCKD-associated gene, regulates regional specification of the zebrafish gut, pronephros, and hindbrain. Genes & Development 2001, 15: 3217-3229. PMID: 11731484, PMCID: PMC312837, DOI: 10.1101/gad946701.Peer-Reviewed Original ResearchMeSH KeywordsAmino Acid SequenceAnimalsBody PatterningCloning, MolecularDiabetes Mellitus, Type 2Digestive SystemDNA-Binding ProteinsEndodermGene Expression ProfilingGene Expression Regulation, DevelopmentalHepatocyte Nuclear Factor 1-betaHumansIn Situ HybridizationKidneyKidney Diseases, CysticModels, BiologicalMolecular Sequence DataMutationNuclear ProteinsPhenotypeReverse Transcriptase Polymerase Chain ReactionRhombencephalonRNA, MessengerSequence Homology, Amino AcidTranscription FactorsZebrafishConceptsRegulate regional specificationLoss-of-function phenotypesFormation of kidney cystsIsolated mutant allelesInsertional mutagenesis screenDevelopment of multiple organsZebrafish gutPatterning genesMutagenesis screenMutant allelesOrgan primordiaExpression domainsVHNF1Gut endodermOtic vesiclePatterning defectsFish phenotypesSonic hedgehogExpression of Pdx1Molecular etiologyDevelopmental functionsMODY5Kidney cystsPhenotypeGenes
1998
Zebrafish segmentation and pair‐rule patterning
van Eeden F, Holley S, Haffter P, Nüsslein‐Volhard C. Zebrafish segmentation and pair‐rule patterning. Genesis 1998, 23: 65-76. PMID: 9706695, DOI: 10.1002/(sici)1520-6408(1998)23:1<65::aid-dvg7>3.0.co;2-4.Peer-Reviewed Original ResearchConceptsDrosophila segment polarity geneSegment polarity genesPair-rule patterningParaxial mesodermZebrafish homologueSomite boundariesPolarity genesPair-rule gene hairyPair-rule genesPair-rule phenotypeWild-type embryosGroup of genesGroup of mutantsGenetic screenVertebrate embryosDouble mutantSomite formationZebrafish segmentationHairy phenotypeMutantsMesodermSonic hedgehogGenesHomologuesHER1 expression
1996
A Mammalian patched Homolog Is Expressed in Target Tissues of sonic hedgehog and Maps to a Region Associated with Developmental Abnormalities (∗)
Hahn H, Christiansen J, Wicking C, Zaphiropoulos P, Chidambaram A, Gerrard B, Vorechovsky I, Bale A, Toftgard R, Dean M, Wainwright B. A Mammalian patched Homolog Is Expressed in Target Tissues of sonic hedgehog and Maps to a Region Associated with Developmental Abnormalities (∗). Journal Of Biological Chemistry 1996, 271: 12125-12128. PMID: 8647801, DOI: 10.1074/jbc.271.21.12125.Peer-Reviewed Original ResearchConceptsLimb budSonic hedgehogSegment polarity genesIntegral membrane proteinsVentral neural tubePolarity genesHydropathy analysisPatched proteinCorrect patterningPutative proteinImaginal discsMembrane proteinsDrosophila hedgehogHuman PatchedCDNA sequencePosterior ectodermMurine homologPatched expressionMouse embryosClose functional relationshipNeural tubeLarval segmentsRegion AssociatedAmino acidsHedgehog
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