2006
Prevention of mesangial sclerosis by bone marrow transplantation
Guo J, Ardito TA, Kashgarian M, Krause DS. Prevention of mesangial sclerosis by bone marrow transplantation. Kidney International 2006, 70: 910-913. PMID: 16850025, DOI: 10.1038/sj.ki.5001698.Peer-Reviewed Original ResearchConceptsBone marrow transplantationMesangial sclerosisMarrow transplantationUrinary albumin lossSimilar therapeutic effectsOnset of diseaseWild-type BMIntrarenal administrationRenal functionRenal histologyRenal diseaseDisease onsetRenal pathologyBM cellsTherapeutic effectEngraftment levelsRenal cellsAlbumin lossKidney samplesMiceSclerosisTransplantationUntreated controlsDiseaseAdministrationAn Inducible Mouse Model for PAX2-Dependent Glomerular Disease: Insights into a Complex Pathogenesis
Wagner KD, Wagner N, Guo JK, Elger M, Dallman MJ, Bugeon L, Schedl A. An Inducible Mouse Model for PAX2-Dependent Glomerular Disease: Insights into a Complex Pathogenesis. Current Biology 2006, 16: 793-800. PMID: 16631587, DOI: 10.1016/j.cub.2006.02.072.Peer-Reviewed Original ResearchConceptsInducible mouse modelGlomerular diseaseMouse modelAngiotensin-converting enzyme inhibitorEnd-stage renal failureTreatment of miceHealthy adult animalsRenal failureRenal functionRenal diseaseComplex pathogenesisNephrin expressionEnzyme inhibitorsDiseaseAdult animalsPodocytesDedifferentiated phenotypePersistent expressionMature podocytesPAX2MicePax genesFunctional relevanceTranscriptional repressorActivation
2005
Bone Marrow Transplantation Can Attenuate the Progression of Mesangial Sclerosis
Guo J, Schedl A, Krause DS. Bone Marrow Transplantation Can Attenuate the Progression of Mesangial Sclerosis. Stem Cells 2005, 24: 406-415. PMID: 16150922, DOI: 10.1634/stemcells.2005-0139.Peer-Reviewed Original ResearchConceptsBone marrow transplantationBM transplantationWild-type BMMesangial sclerosisMarrow transplantationBeneficial effectsLong-term beneficial effectsLower urinary albuminBM-derived cellsDonor BM cellsWild-type recipientsSignificant beneficial effectLong-term improvementProlongation of lifespanMaximal ameliorationRenal diseaseUrinary albuminRenal parenchymaBM cellsTherapeutic effectAlbuminuriaTransplantationTherapeutic potentialLethal doseHeterozygous mice
2002
Early Gonadal Development: Exploring Wt1 and Sox9 Function
Guo J, Hammes A, Chaboissier M, Vidal V, Xing Y, Wong F, Schedl A. Early Gonadal Development: Exploring Wt1 and Sox9 Function. Novartis Foundation Symposia 2002, 244: 23-34. PMID: 11990794, DOI: 10.1002/0470868732.ch3.Peer-Reviewed Original ResearchConceptsSox9 functionSex determinationSex-determining gene SRYSex-determining signalWilms' tumor suppressorFemale sex reversalBipotential primordiumGonad formationGene SRYXX gonadsTransgenic experimentsFirst geneGonadal primordiumMouse developmentPosttranscriptional modificationsKnockout experimentsSex reversalHuman SOX9Distinct functionsSRY geneGonadal anlageSOX9 geneTumor suppressorImportant regulatorGenesWT1 is a key regulator of podocyte function: reduced expression levels cause crescentic glomerulonephritis and mesangial sclerosis
Guo JK, Menke AL, Gubler MC, Clarke AR, Harrison D, Hammes A, Hastie ND, Schedl A. WT1 is a key regulator of podocyte function: reduced expression levels cause crescentic glomerulonephritis and mesangial sclerosis. Human Molecular Genetics 2002, 11: 651-659. PMID: 11912180, DOI: 10.1093/hmg/11.6.651.Peer-Reviewed Original ResearchConceptsTumor 1 geneExpression levelsWilms' tumor 1 geneMesangial sclerosisYeast artificial chromosome transgenic mouse modelWT1 resultsDistinct renal diseasesGenetic evidenceDenys-Drash patientsGene dosageCrescentic glomerulonephritisKey regulatorMolecular explanationDominant mutationsEnd-stage renal failureCell typesPodocyte functionGenes NPHS1WAGR syndromeLevel of WT1Transgenic mouse modelWT1GenesGlomerular basement membraneRenal failure
2001
Two Splice Variants of the Wilms' Tumor 1 Gene Have Distinct Functions during Sex Determination and Nephron Formation
Hammes A, Guo J, Lutsch G, Leheste J, Landrock D, Ziegler U, Gubler M, Schedl A. Two Splice Variants of the Wilms' Tumor 1 Gene Have Distinct Functions during Sex Determination and Nephron Formation. Cell 2001, 106: 319-329. PMID: 11509181, DOI: 10.1016/s0092-8674(01)00453-6.Peer-Reviewed Original ResearchMeSH KeywordsActive Transport, Cell NucleusAlternative SplicingAnimalsAnimals, NewbornApoptosisBase SequenceCell SurvivalDAX-1 Orphan Nuclear ReceptorDisorders of Sex DevelopmentDNA-Binding ProteinsExonsFemaleGenes, Wilms TumorGlomerulosclerosis, Focal SegmentalGonadsMaleMiceMutagenesisNephronsNuclear ProteinsProtein IsoformsReceptors, Retinoic AcidRepressor ProteinsRNA Splice SitesRNA, MessengerSex Determination ProcessesSex-Determining Region Y ProteinSyndromeTranscription FactorsWT1 ProteinsConceptsTumor 1 geneDistinct functionsKTS isoformsSex determination pathwaySplice variantsDistinct molecular functionsZinc finger 3XY sex reversalWilms' tumor 1 geneMolecular functionsWT1 resultsAlternative splicingSex determinationSex reversalFinger 3Nephron formationHomozygous mutantsSpecific isoformsImportant regulatorExpression levelsIsoformsKidney defectsFrasier syndromeHeterozygous miceMouse strains