2025
Cyclic GMP-AMP synthase expression is enhanced in systemic sclerosis-associated interstitial lung disease and stimulates inflammatory myofibroblast activation
Yu S, Hu B, Sun Y, Peng X, Lee C, Woo S, McGovern J, Zielonka J, Saber T, Ghincea A, Gandhi S, Walia A, Pivarnik T, Ishikawa G, Shao S, Sun H, Gunes B, Kujawski S, Perez S, Odell W, Hinchcliff M, Varga J, Feghali-Bostwick C, Sauler M, Gomez J, Ryu C, Herzog E. Cyclic GMP-AMP synthase expression is enhanced in systemic sclerosis-associated interstitial lung disease and stimulates inflammatory myofibroblast activation. European Respiratory Journal 2025, 66: 2401564. PMID: 40374521, PMCID: PMC12332468, DOI: 10.1183/13993003.01564-2024.Peer-Reviewed Original ResearchPrecision cut lung slicesSSc-ILD lung tissuesType 1 interferonSSc-ILDProduction of cytokinesBronchoalveolar lavageHuman precision cut lung slicesLung tissueLung fibroblastsLungs of patientsInterstitial lung diseasePulmonary fibrosis modelBleomycin mouse modelIsolated lung fibroblastsCultured fibroblastsPerturbs innate immunityFibrotic stimuliSingle cell RNA sequencing datasetsSystemic sclerosisHuman lung fibroblastsLung diseaseMouse modelCyclic GMP-AMP synthaseFibrosis modelTherapeutic approaches
2023
Identification of Paired-related Homeobox Protein 1 as a key mesenchymal transcription factor in pulmonary fibrosis
Marchal-Duval E, Homps-Legrand M, Froidure A, Jaillet M, Ghanem M, Lou D, Justet A, Maurac A, Vadel A, Fortas E, Cazes A, Joannes A, Giersh L, Mal H, Mordant P, Piolot T, Truchin M, Mounier C, Schirduan K, Korfei M, Gunther A, Mari B, Jaschinski F, Crestani B, Mailleux A. Identification of Paired-related Homeobox Protein 1 as a key mesenchymal transcription factor in pulmonary fibrosis. ELife 2023, 12: e79840. PMID: 37261432, PMCID: PMC10275639, DOI: 10.7554/elife.79840.Peer-Reviewed Original ResearchConceptsIdiopathic pulmonary fibrosisMesenchymal transcription factorsPulmonary fibrosisMouse precision-cut lung slicesMouse model of lung fibrosisTGF-bModel of lung fibrosisLung fibroblastsPrecision-cut lung slicesBleomycin mouse modelIdiopathic pulmonary fibrosis treatmentHuman lung fibroblast proliferationIPF human lung fibroblastsMatrix remodelingTranscription factorsInhibiting Smad2/3 phosphorylationLung fibroblast proliferationHuman lung fibroblastsS phase cyclinsLung fibrosisHomeobox protein 1Remodeling in vivoLung fibrogenesisSmad2/3 phosphorylationTargeted inhibition
2022
Ameliorating Fibrosis in Murine and Human Tissues with END55, an Endostatin-Derived Fusion Protein Made in Plants
Mlakar L, Garrett S, Watanabe T, Sanderson M, Nishimoto T, Heywood J, Helke K, Pilewski J, Herzog E, Feghali-Bostwick C. Ameliorating Fibrosis in Murine and Human Tissues with END55, an Endostatin-Derived Fusion Protein Made in Plants. Biomedicines 2022, 10: 2861. PMID: 36359382, PMCID: PMC9687961, DOI: 10.3390/biomedicines10112861.Peer-Reviewed Original ResearchAnti-fibrotic effectsIdiopathic pulmonary fibrosisSystemic sclerosisPulmonary fibrosisLung fibrosisMouse modelSignificant anti-fibrotic effectEffective anti-fibrotic therapiesBleomycin mouse modelLungs of patientsAnti-fibrotic therapiesPrevention of skinSecond mouse modelCollagen XVIII/endostatinGrowth factor-β1Matrix-degrading enzymesLung transplantationSignificant morbidityHuman tissuesLung diseaseHealth burdenEffective treatmentFactor-β1Organ fibrosisFibrosis
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