Enhancing autophagy with drugs or lung-directed gene therapy reverses the pathological effects of respiratory epithelial cell proteinopathy

Tunda Hidvegi; Donna B. Stolz; John F. Alcorn; Samuel A. Yousem; Jieru Wang; Adriana S. Leme; A. Mc Garry Houghton; Pamela Hale; Michael Ewing; Houming Cai; Evelyn Akpadock Garchar; Nunzia Pastore; Patrizia Annunziata; Naftali Kaminski; Joseph Pilewski; Steven D. Shapiro; Stephen C. Pak; Gary A. Silverman; Nicola Brunetti-Pierri; David H. Perlmutter

doi:10.1074/jbc.M115.691253

Enhancing autophagy with drugs or lung-directed gene therapy reverses the pathological effects of respiratory epithelial cell proteinopathy

Tunda Hidvegi, Donna B. Stolz, John F. Alcorn, Samuel A. Yousem, Jieru Wang, Adriana S. Leme, A. Mc Garry Houghton, Pamela Hale, Michael Ewing, Houming Cai, Evelyn Akpadock Garchar, Nunzia Pastore, Patrizia Annunziata, Naftali Kaminski, Joseph Pilewski, Steven D. Shapiro, Stephen C. Pak, Gary A. Silverman, Nicola Brunetti-Pierri, David H. Perlmutter

Research output: Contribution to journal › Article › peer-review

27 Scopus citations

Abstract

Background: Several rare lung proteinopathies are characterized by lung fibrosis due to accumulation of misfolded proteins in epithelial cells. Results: The PiZ mouse models the pathological characteristics of these lung proteinopathies, and the pathology is ameliorated by autophagy enhancing therapies. Conclusion: Autophagy represents a key proteostasis mechanism for lung proteinopathies and a potential therapeutic target. Significance: The PiZ mouse is an attractive animal model of lung proteinopathies.

Original language	English
Pages (from-to)	29742-29757
Number of pages	16
Journal	Journal of Biological Chemistry
Volume	290
Issue number	50
DOIs	https://doi.org/10.1074/jbc.M115.691253
State	Published - Dec 11 2015

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Hidvegi, T., Stolz, D. B., Alcorn, J. F., Yousem, S. A., Wang, J., Leme, A. S., Houghton, A. M. G., Hale, P., Ewing, M., Cai, H., Garchar, E. A., Pastore, N., Annunziata, P., Kaminski, N., Pilewski, J., Shapiro, S. D., Pak, S. C., Silverman, G. A., Brunetti-Pierri, N., & Perlmutter, D. H. (2015). Enhancing autophagy with drugs or lung-directed gene therapy reverses the pathological effects of respiratory epithelial cell proteinopathy. Journal of Biological Chemistry, 290(50), 29742-29757. https://doi.org/10.1074/jbc.M115.691253

@article{b212ada0af914ec691e8f8820b003721,

title = "Enhancing autophagy with drugs or lung-directed gene therapy reverses the pathological effects of respiratory epithelial cell proteinopathy",

abstract = "Background: Several rare lung proteinopathies are characterized by lung fibrosis due to accumulation of misfolded proteins in epithelial cells. Results: The PiZ mouse models the pathological characteristics of these lung proteinopathies, and the pathology is ameliorated by autophagy enhancing therapies. Conclusion: Autophagy represents a key proteostasis mechanism for lung proteinopathies and a potential therapeutic target. Significance: The PiZ mouse is an attractive animal model of lung proteinopathies.",

author = "Tunda Hidvegi and Stolz, {Donna B.} and Alcorn, {John F.} and Yousem, {Samuel A.} and Jieru Wang and Leme, {Adriana S.} and Houghton, {A. Mc Garry} and Pamela Hale and Michael Ewing and Houming Cai and Garchar, {Evelyn Akpadock} and Nunzia Pastore and Patrizia Annunziata and Naftali Kaminski and Joseph Pilewski and Shapiro, {Steven D.} and Pak, {Stephen C.} and Silverman, {Gary A.} and Nicola Brunetti-Pierri and Perlmutter, {David H.}",

note = "Publisher Copyright: {\textcopyright} 2015 by The American Society for Biochemistry and Molecular Biology, Inc.",

year = "2015",

month = dec,

day = "11",

doi = "10.1074/jbc.M115.691253",

language = "English",

volume = "290",

pages = "29742--29757",

journal = "Journal of Biological Chemistry",

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Hidvegi, T, Stolz, DB, Alcorn, JF, Yousem, SA, Wang, J, Leme, AS, Houghton, AMG, 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 2015, 'Enhancing autophagy with drugs or lung-directed gene therapy reverses the pathological effects of respiratory epithelial cell proteinopathy', Journal of Biological Chemistry, vol. 290, no. 50, pp. 29742-29757. https://doi.org/10.1074/jbc.M115.691253

TY - JOUR

T1 - Enhancing autophagy with drugs or lung-directed gene therapy reverses the pathological effects of respiratory epithelial cell proteinopathy

AU - Hidvegi, Tunda

AU - Stolz, Donna B.

AU - Alcorn, John F.

AU - Yousem, Samuel A.

AU - Wang, Jieru

AU - Leme, Adriana S.

AU - Houghton, A. Mc Garry

AU - Hale, Pamela

AU - Ewing, Michael

AU - Cai, Houming

AU - Garchar, Evelyn Akpadock

AU - Pastore, Nunzia

AU - Annunziata, Patrizia

AU - Kaminski, Naftali

AU - Pilewski, Joseph

AU - Shapiro, Steven D.

AU - Pak, Stephen C.

AU - Silverman, Gary A.

AU - Brunetti-Pierri, Nicola

AU - Perlmutter, David H.

PY - 2015/12/11

Y1 - 2015/12/11

N2 - Background: Several rare lung proteinopathies are characterized by lung fibrosis due to accumulation of misfolded proteins in epithelial cells. Results: The PiZ mouse models the pathological characteristics of these lung proteinopathies, and the pathology is ameliorated by autophagy enhancing therapies. Conclusion: Autophagy represents a key proteostasis mechanism for lung proteinopathies and a potential therapeutic target. Significance: The PiZ mouse is an attractive animal model of lung proteinopathies.

AB - Background: Several rare lung proteinopathies are characterized by lung fibrosis due to accumulation of misfolded proteins in epithelial cells. Results: The PiZ mouse models the pathological characteristics of these lung proteinopathies, and the pathology is ameliorated by autophagy enhancing therapies. Conclusion: Autophagy represents a key proteostasis mechanism for lung proteinopathies and a potential therapeutic target. Significance: The PiZ mouse is an attractive animal model of lung proteinopathies.

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U2 - 10.1074/jbc.M115.691253

DO - 10.1074/jbc.M115.691253

M3 - Article

C2 - 26494620

AN - SCOPUS:84949648966

SN - 0021-9258

VL - 290

SP - 29742

EP - 29757

JO - Journal of Biological Chemistry

JF - Journal of Biological Chemistry

IS - 50

ER -

Enhancing autophagy with drugs or lung-directed gene therapy reverses the pathological effects of respiratory epithelial cell proteinopathy

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