Type 2 innate immunity promotes the development of pulmonary fibrosis in Hermansky-Pudlak syndrome
Sorkhdini P, Klubock-Shukla K, Sheth S, Yang D, Yang A, Norbrun C, Introne W, Gochuico B, Zhou Y. Type 2 innate immunity promotes the development of pulmonary fibrosis in Hermansky-Pudlak syndrome. JCI Insight 2024, 9: e178381. PMID: 39405112, PMCID: PMC11601950, DOI: 10.1172/jci.insight.178381.Peer-Reviewed Original ResearchHermansky-Pudlak syndromeDevelopment of pulmonary fibrosisIdiopathic pulmonary fibrosisPulmonary fibrosisHermansky-Pudlak syndrome patientType 2 innate immunityOverexpression of CHI3L1Progressive pulmonary fibrosisPrimary lung fibroblastsFibrosis-related genesFibroproliferative repairILC2 accumulationILC2 populationSorted ILC2sLung ILC2sCo-culture experimentsILC2sFibrosis developmentFibrosisLung tissueHermansky-PudlakWild typeLung fibroblastsCo-cultureFibroproliferation