Enhancing Autophagy with Drugs or Lung-directed Gene Therapy Reverses the Pathological Effects of Respiratory Epithelial Cell Proteinopathy.

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作者： Tunda,Hidvegi ^[1] ; Donna B,Stolz ^[2] ; John F,Alcorn ^[1] ; Samuel A,Yousem ^[3] ; Jieru,Wang ^[4] ; Adriana S,Leme ^[5] ; A McGarry,Houghton ^[5] ; Pamela,Hale ^[1] ; Michael,Ewing ^[1] ; Houming,Cai ^[1] ; Evelyn Akpadock,Garchar ^[1] ; Nunzia,Pastore ^[6] ; Patrizia,Annunziata ^[6] ; Naftali,Kaminski ^[5] ; Joseph,Pilewski ^[5] ; Steven D,Shapiro ^[5] ; Stephen C,Pak ^[1] ; Gary A,Silverman ^[7] ; Nicola,Brunetti-Pierri ^[8] ; David H,Perlmutter ^[9]

作者单位： From the Departments of Pediatrics, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania 15224. ^[1] Cell Biology, and. ^[2] Pathology University of Pittsburgh School of Medicine and. ^[3] From the Departments of Pediatrics. ^[4] Medicine. ^[5] Department of Translational Medicine, Federico II University, Naples, Italy, 80138. ^[6] From the Departments of Pediatrics, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania 15224, Cell Biology, and. ^[7] Department of Translational Medicine, Federico II University, Naples, Italy, 80138 Telethon Institute of Genetics and Medicine, Pozzuoli, Naples, Italy, 80131, and. ^[8] From the Departments of Pediatrics, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania 15224, Cell Biology, and david.perlmutter@chp.edu. ^[9]

关键词 autophagy chronic obstructive pulmonary disease (COPD)misfolded α1antitrypsin protein misfolding proteostasis pulmonary fibrosis α1antitrypsin deficiency

主题词动物(Animals);自噬(Autophagy);疾病模型, 动物(Disease Models, Animal);上皮细胞(Epithelial Cells);肺(Lung);小鼠(Mice);α1抗胰蛋白酶缺乏症(alpha 1-Antitrypsin Deficiency)

DOI 10.1074/jbc.M115.691253

PMID 26494620

发布时间 2021-02-05

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