Mechanisms of Action of Autophagy Modulators Dissected by Quantitative Systems Pharmacology Analysis by Qingya Shi, Fen Pei, Gary A. Silverman, Stephen C. Pak, David H. Perlmutter, Bing Liu, Ivet Bahar

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A pro-cathepsin L mutant is a luminal substrate for endoplasmic-reticulum-associated degradation in C. elegans. by Mark T Miedel, Nathan J Graf, Kate E Stephen, Olivia S Long, Stephen C Pak, David H Perlmutter, Gary A Silverman, Cliff J Luke

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Deficient and Null Variants of SERPINA1 Are Proteotoxic in a Caenorhabditis elegans Model of α1-Antitrypsin Deficiency. by Erin E Cummings, Linda P O'Reilly, Dale E King, Richard M Silverman, Mark T Miedel, Cliff J Luke, David H Perlmutter, Gary A Silverman, Stephen C Pak

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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

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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

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Model organisms contribute to diagnosis and discovery in the undiagnosed diseases network: current state and a future vision by Dustin Baldridge, Michael F. Wangler, Angela N. Bowman, Shinya Yamamoto, Undiagnosed Diseases Network, Tim Schedl, Stephen C. Pak, John H. Postlethwait, Jimann Shin, Lilianna Solnica-Krezel, Hugo J. Bellen, Monte Westerfield

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Automated high-content live animal drug screening using C. elegans expressing the aggregation prone serpin α1-antitrypsin Z. by Sager J Gosai, Joon Hyeok Kwak, Cliff J Luke, Olivia S Long, Dale E King, Kevin J Kovatch, Paul A Johnston, Tong Ying Shun, John S Lazo, David H Perlmutter, Gary A Silverman, Stephen C Pak

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SERPINB3 (SCCA1) inhibits cathepsin L and lysoptosis, protecting cervical cancer cells from chemoradiation by Songyan Wang, Cliff J. Luke, Stephen C. Pak, Victoria Shi, Liyun Chen, Jonathan Moore, Arlise P. Andress, Kay Jayachandran, Jin Zhang, Yi Huang, Marina Platik, Anthony A. Apicelli, Julie K. Schwarz, Perry W. Grigsby, Gary A. Silverman, Stephanie Markovina

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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

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CemOrange2 fusions facilitate multifluorophore subcellular imaging in C. elegans. by Brian J Thomas, Ira E Wight, Wendy Y Y Chou, Marco Moreno, Zachary Dawson, Arielle Homayouni, Huiyan Huang, Hyori Kim, Hanna Jia, Justin R Buland, Jennifer A Wambach, F Sessions Cole, Stephen C Pak, Gary A Silverman, Cliff J Luke

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Lysoptosis is an evolutionarily conserved cell death pathway moderated by intracellular serpins by Cliff J. Luke, Stephanie Markovina, Misty Good, Ira E. Wight, Brian J. Thomas, John M. Linneman, Wyatt E. Lanik, Olga Koroleva, Maggie R. Coffman, Mark T. Miedel, Qingqing Gong, Arlise Andress, Marlene Campos Guerrero, Songyan Wang, LiYun Chen, Wandy L. Beatty, Kelsey N. Hausmann, Frances V. White, James A. J. Fitzpatrick, Anthony Orvedahl, Stephen C. Pak, Gary A. Silverman

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Dominant negative variants in KIF5B cause osteogenesis imperfecta via down regulation of mTOR signaling. by Ronit Marom, Bo Zhang, Megan E Washington, I-Wen Song, Lindsay C Burrage, Vittoria C Rossi, Ava S Berrier, Anika Lindsey, Jacob Lesinski, Michael L Nonet, Jian Chen, Dustin Baldridge, Gary A Silverman, V Reid Sutton, Jill A Rosenfeld, Alyssa A Tran, M John Hicks, David R Murdock, Hongzheng Dai, MaryAnn Weis, Shalini N Jhangiani, Donna M Muzny, Richard A Gibbs, Richard Caswell, Carrie Pottinger, Deirdre Cilliers, Karen Stals, Undiagnosed Diseases Network, David Eyre, Deborah Krakow, Tim Schedl, Stephen C Pak, Brendan H Lee

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