NFκB mitigates the pathological effects of misfolded α1-antitrypsin by activating autophagy and an integrated program of proteostasis mechanisms

von Mukherjee, Amitava, Hidvegi, Tunda, Araya, Patrick, Ewing, Michael, Stolz, Donna B., Perlmutter, David H.
Veröffentlicht in Cell Death Differ (2018)

Fluphenazine Reduces Proteotoxicity in C. elegans and Mammalian Models of Alpha-1-Antitrypsin Deficiency

von Li, Jie, Pak, Stephen C., O’Reilly, Linda P., Benson, Joshua A., Wang, Yan, Hidvegi, Tunda, Hale, Pamela, Dippold, Christine, Ewing, Michael, Silverman, Gary A., Perlmutter, David H.
Veröffentlicht 2014

An analog of glibenclamide selectively enhances autophagic degradation of misfolded α1-antitrypsin Z

von Wang, Yan, Cobanoglu, Murat C., Li, Jie, Hidvegi, Tunda, Hale, Pamela, Ewing, Michael, Chu, Andrew S., Gong, Zhenwei, Muzumdar, Radhika, Pak, Stephen C., Silverman, Gary A., Bahar, Ivet, Perlmutter, David H.
Veröffentlicht in PLoS One (2019)

Spontaneous hepatic repopulation in transgenic mice expressing mutant human α1-antitrypsin by wild-type donor hepatocytes

von Ding, Jianqiang, Yannam, Govardhana R., Roy-Chowdhury, Namita, Hidvegi, Tunda, Basma, Hesham, Rennard, Stephen I., Wong, Ronald J., Avsar, Yesim, Guha, Chandan, Perlmutter, David H., Fox, Ira J., Roy-Chowdhury, Jayanta
Veröffentlicht 2011

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

von Hidvegi, Tunda, Stolz, Donna B., Alcorn, John F., Yousem, Samuel A., Wang, Jieru, Leme, Adriana S., Houghton, A. McGarry, Hale, Pamela, Ewing, Michael, Cai, Houming, Garchar, Evelyn Akpadock, Pastore, Nunzia, Annunziata, Patrizia, Kaminski, Naftali, Pilewski, Joseph, Shapiro, Steven D., Pak, Stephen C., Silverman, Gary A., Brunetti-Pierri, Nicola, Perlmutter, David H.
Veröffentlicht in J Biol Chem (2015)