Search Results - David H. Perlmutter
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Fluphenazine reduces proteotoxicity in C. elegans and mammalian models of alpha-1-antitrypsin deficiency. by Jie Li, Stephen C Pak, Linda P O'Reilly, Joshua A Benson, Yan Wang, Tunda Hidvegi, Pamela Hale, Christine Dippold, Michael Ewing, Gary A Silverman, David H Perlmutter
Published in PLoS ONE (2014-01-01)Get full text
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An analog of glibenclamide selectively enhances autophagic degradation of misfolded α1-antitrypsin Z. by Yan Wang, Murat C Cobanoglu, Jie Li, Tunda Hidvegi, Pamela Hale, Michael Ewing, Andrew S Chu, Zhenwei Gong, Radhika Muzumdar, Stephen C Pak, Gary A Silverman, Ivet Bahar, David H Perlmutter
Published in PLoS ONE (2019-01-01)Get full text
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A fluorescent reporter for rapid assessment of autophagic flux reveals unique autophagy signatures during C. elegans post-embryonic development and identifies compounds that modula... by Zachary D. Dawson, Hemalatha Sundaramoorthi, Suk Regmi, Bo Zhang, Stephanie Morrison, Sara M. Fielder, Jessie R. Zhang, Hieu Hoang, David H. Perlmutter, Cliff J. Luke, Gary A. Silverman, Stephen C. Pak
Published in Autophagy Reports (2024-12-01)Get full text
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A pathogenic Tau mutation drives autophagy-lysosome dysfunction that limits Tau degradation in a model of frontotemporal dementia by Farzaneh S. Mirfakhar, Jacob A. Marsh, Chihiro Sato, Kylie J. Schache, Miguel A. Minaya, Roland E. Dolle, Stephen C. Pak, Gary A. Silverman, David H. Perlmutter, Shannon L. Macauley, Celeste M. Karch
Published in Nature Communications (2026-03-01)Get full text
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Resolution of hepatic fibrosis after ZFN-mediated gene editing in the PiZ mouse model of human α1-antitrypsin deficiency by Yanfeng Li, Chandan Guha, Patrik Asp, Xia Wang, Tatyana L. Tchaikovskya, Kenneth Kim, Matthew Mendel, Gregory J. Cost, David H. Perlmutter, Namita Roy-Chowdhury, Ira J. Fox, Anthony Conway, Jayanta Roy-Chowdhury
Published in Hepatology Communications (2023-03-01)Get full text
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Multiple Genes Core to ERAD, Macroautophagy and Lysosomal Degradation Pathways Participate in the Proteostasis Response in α1-Antitrypsin DeficiencySummary by Jie Li, Francesca Moretti, Tunda Hidvegi, Sanja Sviben, James A.J. Fitzpatrick, Hemalatha Sundaramoorthi, Stephen C. Pak, Gary A. Silverman, Britta Knapp, Ireos Filipuzzi, John Alford, John Reece-Hoyes, Florian Nigsch, Leon O. Murphy, Beat Nyfeler, David H. Perlmutter
Published in Cellular and Molecular Gastroenterology and Hepatology (2024-01-01)Get full text
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