Enhancing Autophagy with Drugs or Lung-directed Gene Therapy Reverses the Pathological Effects of Respiratory Epithelial Cell Proteinopathy*
Hidvegi T, Stolz DB, Alcorn JF, Yousem SA, Wang J, Leme AS, Houghton AM, Hale P, Ewing M, Cai H, Garchar EA, Pastore N, Annunziata P, Kaminski N, Pilewski J, Shapiro SD, Pak SC, Silverman GA, Brunetti-Pierri N, Perlmutter DH. Enhancing Autophagy with Drugs or Lung-directed Gene Therapy Reverses the Pathological Effects of Respiratory Epithelial Cell Proteinopathy*. Journal Of Biological Chemistry 2015, 290: 29742-29757. PMID: 26494620, PMCID: PMC4705969, DOI: 10.1074/jbc.m115.691253.Peer-Reviewed Original ResearchConceptsSpontaneous pulmonary fibrosisPulmonary fibrosisΑ1-antitrypsin ZPathological effectsSevere pulmonary fibrosisRespiratory epithelial cellsPiZ miceRestrictive deficitsActivation of autophagyLeukocyte infiltrationSurfactant protein AAnimal modelsC deficiencyFibrosisProteinopathiesSkeletal muscleEpithelial cellsIntracellular accumulationAutophagolysosomal systemLungMiceAttractive targetAutophagyDrugsRecent studies