Ngā hua rapu - David H. Perlmutter

The polymerized protein response (PPR) is activated by genetic variants that polymerize in the ER and is mediated by NFκBp50 mā Admire Munanairi, David A. Rudnick, Jiansheng Huang, David H. Perlmutter

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Fluphenazine reduces proteotoxicity in C. elegans and mammalian models of alpha-1-antitrypsin deficiency. mā 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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A fluorescent reporter for rapid assessment of autophagic flux reveals unique autophagy signatures during C. elegans post-embryonic development and identifies compounds that modula... mā 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

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A pathogenic Tau mutation drives autophagy-lysosome dysfunction that limits Tau degradation in a model of frontotemporal dementia mā 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

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Multiple Genes Core to ERAD, Macroautophagy and Lysosomal Degradation Pathways Participate in the Proteostasis Response in α1-Antitrypsin DeficiencySummary mā 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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