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

por Mukherjee, Amitava, Hidvegi, Tunda, Araya, Patrick, Ewing, Michael, Stolz, Donna B., Perlmutter, David H.
Publicado en Cell Death Differ (2018)

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

por 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.
Publicado 2014

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

por 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.
Publicado en PLoS One (2019)

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

por 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
Publicado 2011

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

por 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.
Publicado en J Biol Chem (2015)