Induced pluripotent stem cells model personalized variations in liver disease resulting from α1‐antitrypsin deficiency
Tafaleng, Edgar N., Chakraborty, Souvik, Han, Bing, Hale, Pamela, Wu, Wanquan, Soto‐Gutierrez, Alejandro, Feghali‐Bostwick, Carol A., Wilson, Andrew A., Kotton, Darrell N., Nagaya, Masaki, Strom, Stephen C., Roy‐Chowdhury, Jayanta, Stolz, Donna B., Perlmutter, David H., Fox, Ira J.
Published in Hepatology (Baltimore, Md.) (01.07.2015)
Published in Hepatology (Baltimore, Md.) (01.07.2015)
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An analog of glibenclamide selectively enhances autophagic degradation of misfolded α1-antitrypsin Z
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
Published in PloS one (01.01.2019)
Published in PloS one (01.01.2019)
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Fluphenazine reduces proteotoxicity in C. elegans and mammalian models of alpha-1-antitrypsin deficiency
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
Published in PloS one (31.01.2014)
Published in PloS one (31.01.2014)
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Autophagy-Enhancing Drug Promotes Degradation of Mutant α₁-Antitrypsin Z and Reduces Hepatic Fibrosis
Hidvegi, Tunda, Ewing, Michael, Hale, Pamela, Dippold, Christine, Beckett, Caroline, Kemp, Carolyn, Maurice, Nicholas, Mukherjee, Amitava, Goldbach, Christina, Watkins, Simon, Michalopoulos, George, Perlmutter, David H
Published in Science (American Association for the Advancement of Science) (09.07.2010)
Published in Science (American Association for the Advancement of Science) (09.07.2010)
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Journal Article
An Autophagy-Enhancing Drug Promotes Degradation of Mutant α 1 -Antitrypsin Z and Reduces Hepatic Fibrosis
Hidvegi, Tunda, Ewing, Michael, Hale, Pamela, Dippold, Christine, Beckett, Caroline, Kemp, Carolyn, Maurice, Nicholas, Mukherjee, Amitava, Goldbach, Christina, Watkins, Simon, Michalopoulos, George, Perlmutter, David H.
Published in Science (American Association for the Advancement of Science) (09.07.2010)
Published in Science (American Association for the Advancement of Science) (09.07.2010)
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Regulation of PGC1α Downstream of the Insulin Signaling Pathway Plays a Role in the Hepatic Proteotoxicity of Mutant α1-Antitrypsin Deficiency Variant Z
Rudnick, David A., Huang, Jiansheng, Hidvegi, Tunda, Chu, Andrew S., Hale, Pamela, Munanairi, Admire, Dietzen, Dennis J., Cliften, Paul F., Tycksen, Eric, Lutkewitte, Andrew J., Finck, Brian N., Pak, Stephen C., Silverman, Gary A., Perlmutter, David H.
Published in Gastroenterology (New York, N.Y. 1943) (01.07.2022)
Published in Gastroenterology (New York, N.Y. 1943) (01.07.2022)
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Induced pluripotent stem cells model personalized variations in liver disease resulting from [alpha]1-antitrypsin deficiency
Tafaleng, Edgar N, Chakraborty, Souvik, Han, Bing, Hale, Pamela, Wu, Wanquan, Soto-Gutierrez, Alejandro, Feghali-Bostwick, Carol A, Wilson, Andrew A, Kotton, Darrell N, Nagaya, Masaki, Strom, Stephen C, Roy-Chowdhury, Jayanta, Stolz, Donna B, Perlmutter, David H, Fox, Ira J
Published in Hepatology (Baltimore, Md.) (01.07.2015)
Published in Hepatology (Baltimore, Md.) (01.07.2015)
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Regulator of G Signaling 16 Is a Marker for the Distinct Endoplasmic Reticulum Stress State Associated with Aggregated Mutant α1-Antitrypsin Z in the Classical Form of α1-Antitrypsin Deficiency
Hidvegi, Tunda, Mirnics, Karoly, Hale, Pamela, Ewing, Michael, Beckett, Caroline, Perlmutter, David H.
Published in The Journal of biological chemistry (21.09.2007)
Published in The Journal of biological chemistry (21.09.2007)
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Journal Article
An analog of glibenclamide selectively enhances autophagic degradation of misfolded [alpha]1-antitrypsin Z
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
Published in PloS one (23.01.2019)
Published in PloS one (23.01.2019)
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A genome-wide RNAi screen identifies potential drug targets in a C. elegans model of α1-antitrypsin deficiency
O'Reilly, Linda P, Long, Olivia S, Cobanoglu, Murat C, Benson, Joshua A, Luke, Cliff J, Miedel, Mark T, Hale, Pamela, Perlmutter, David H, Bahar, Ivet, Silverman, Gary A, Pak, Stephen C
Published in Human molecular genetics (01.10.2014)
Published in Human molecular genetics (01.10.2014)
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Enhancing Autophagy with Drugs or Lung-directed Gene Therapy Reverses the Pathological Effects of Respiratory Epithelial Cell Proteinopathy
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.
Published in The Journal of biological chemistry (11.12.2015)
Published in The Journal of biological chemistry (11.12.2015)
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Clathrin-Mediated Endocytosis of Neutrophil Elastase and Cathepsin G by Lung Tumor Epithelial Cells
Gregory, Alyssa D., Hale, Pamela D., Perlmutter, David H., Shapiro, Steven D., Houghton, A. McGarry
Published in Blood (16.11.2012)
Published in Blood (16.11.2012)
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Journal Article
Induced pluripotent stem cells model personalized variations in liver disease due to α1-antitrypsin deficiency
Tafaleng, Edgar N., Chakraborty, Souvik, Han, Bing, Hale, Pamela, Wu, Wanquan, Soto-Gutierrez, Alejandro, Feghali-Bostwick, Carol A., Wilson, Andrew A., Kotton, Darrell N., Nagaya, Masaki, Strom, Stephen C., Chowdhury, Jayanta R., Stolz, Donna B., Perlmutter, David H., Fox, Ira J.
Published in Hepatology (Baltimore, Md.) (13.04.2015)
Published in Hepatology (Baltimore, Md.) (13.04.2015)
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Journal Article
The Proteasome Participates in Degradation of Mutant α1-Antitrypsin Z in the Endoplasmic Reticulum of Hepatoma-derived Hepatocytes
Teckman, Jeffrey H., Burrows, Jon, Hidvegi, Tunde, Schmidt, Bela, Hale, Pamela D., Perlmutter, David H.
Published in The Journal of biological chemistry (01.11.2001)
Published in The Journal of biological chemistry (01.11.2001)
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COX-2 expression is induced by UVB exposure in human skin : Implications for the development of skin cancer
BUCKMAN, S. Y, GRESHAM, A, HALE, P, HRUZA, G, ANAST, J, MASFERRER, J, PENTLAND, A. P
Published in Carcinogenesis (New York) (01.05.1998)
Published in Carcinogenesis (New York) (01.05.1998)
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The proteasome participates in degradation of mutant alpha 1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes
Teckman, J H, Burrows, J, Hidvegi, T, Schmidt, B, Hale, P D, Perlmutter, D H
Published in The Journal of biological chemistry (30.11.2001)
Published in The Journal of biological chemistry (30.11.2001)
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A patient with pseudo-Addison's disease and falsely elevated thyroxine due to interference in serum cortisol and free thyroxine immunoassays by two different mechanisms
Saleem, Mohamed, Lewis, John G, Florkowski, Christopher M, Mulligan, Guy P, George, Peter M, Hale, Pamela
Published in Annals of clinical biochemistry (01.03.2009)
Published in Annals of clinical biochemistry (01.03.2009)
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Journal Article
An Autophagy-Enhancing Drug Promotes Degradation of Mutant [alpha]1-Antitrypsin Z and Reduces Hepatic Fibrosis
Hidvegi, Tunda, Ewing, Michael, Hale, Pamela, Dippold, Christine, Beckett, Caroline, Kemp, Carolyn, Nicholas, Maurice, Mukherjee, Amitava, Goldbach, Christina, Watkins, Simon, Michalopoulos, George, Perlmutter, David H
Published in Science (American Association for the Advancement of Science) (09.07.2010)
Published in Science (American Association for the Advancement of Science) (09.07.2010)
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Journal Article