Publications


Rius, B; Mesgarzadeh, J; Romine, IC; Paxman, RJ; Kelly, JW; Wiseman, RL (2021) Pharmacologic Targeting of Plasma Cell Endoplasmic Reticulum Proteostasis to Reduce Amyloidogenic Light Chain Secretion, Blood Advances

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Vidal, RL; Sepulveda, D; Tronscoso-Escudero, P; Garcia-Huerta, P; Gonzalez, C; Plate, L; Jerez, C; Canovas, J; Rivera, C; Castillo, V; Cisternas, M; Leal, S; Martin, A; Grandjean, JMD; Lashuel, HA; Martins, AJM; Latapioa, V; Matus, S; Sardi,P; Wiseman, RL; Hetz, C (2021) Enforced Dimerization between XBP1s and ATF6f Enhances the Protective Effects of the Unfolded Protein Response (UPR) in Models of Neurodegeneration, Molecular Therapy

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Madhavan, A; Kok, BP; Grandjean, JMD; Albert, V; Sukiasyan, A; Rius, B; Powers, ET; Galmozzi, A; Saez, E; Wiseman, RL(2020) Pharmacologic IRE1/XBP1s Activation Promotes Systemic Adaptive Remodeling in Obesity, bioRxiv. *Co-corresponding author.

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Shin, M; Watson, ER; Novick, J; Griffin, P; Wiseman, RL; Lander, GC (2020) Structures of the Human LONP1 Protease Reveal Regulatory Steps Involved in Protease Activation, bioRxiv. *Co-corresponding author.

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Ibrahim, L; Mesgarzadeh, J; Xu, I; Powers, ET; Wiseman, RL; Bollong, MJ (2020) Defining the Functional Targets of Cap’n’Collar Transcription Factors NRF1, NRF2, and NRF3, Antioxidants

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Kroeger, H; Grandjean, JMD; Chiang, WCJ; Bindels, D; Mastey, R; Okalova, J; Nguyen, A; Powers, ET; Kelly, JW; Grimsey, NJ; Michaelides, M; Carroll, J; Wiseman, RL; Lin, JH (2020) The Endoplasmic Reticulum Proteostasis Regulator ATF6 is Essential for Human Cone Photoreceptor Development, bioRxiv

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Grandjean, JMD; Wiseman, RL (2020) Small molecule strategies to harness the unfolded protein response: where do we go from here?, J Biol Chem

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Grandjean, JMD; Madhavan, A; Cech, L; Seguinot, BO; Paxman, RJ; Smith, E; Scampavia,L; Powers, ET; Cooley, CB; Plate, L; Spicer, TP; Kelly, JW; Wiseman,RL (2020) Pharmacologic IRE1/XBP1s Activation Confers Targeted ER Proteostasis Reprogramming, Nat Chem Biol

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Kirak, O; Ke, E; Yang, JY; Schwarz, A; Plate, L; Nham, A; Abadejos, JR; Valencia, A; Wiseman, RL; Lui, KO; Ku, M (2020) Premature Activation of Immune Transcripiton Programs in Autoimmune-Predisposed Mouse Embryonic Stem Cells and Blastocysts, Int J Mol Sci

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Giadone, RM; Liberti, DC; Matte, TM; Rosarda, JD; Torress-Arancivia, C; Ghosh, S; Diedrich, JK; Pankow, S; Skvir, N; Yates, III, JR; Wilson, AA; Connors, LH; Kotton, DN; Wiseman, RL; Murphy, GJ (2020) Expression of Amyloidogenic Transthyretin Drives Hepatic Proteostasis Remodeling in an Induced Pluripotent Stem Cell Model of Systemic Amyloid Disease, Stem Cell Reports

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Garcia-Huerta, P; Troncoso-Escudero, P; Wu, D; Thiruvalluvan, A; Cisternas, M; Henriquez, DR; Plate, L; Chana-Cyevas, P; Saquel, C; Thielen, P; Longo, KA; Geddes, BJ; Lederkremer, GL; Sharma, N; Shenkman, M; Naphade, S; Ellerby, LM; Sardi, P; Spichiger, C; Richter, HG; Court, FA; Wiseman, RL; Gonzalez-Billault, C; Bergink, S; Vidal, RL; Hetz, C (2020) Insulin-like Growth Factor 2 (IGF2) Protects Against Huntington’s Disease Through the Extracellular Deposition of Protein Aggregates, Acta Neuropathologica.

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Shin, M; Puchades, C; Asmita, A; Adjei, E; Wiseman, RL; Karzai, WA; Lander GC (2020) Structural Basis for Distinct Operational Modes and Protease Activation in AAA+ Protease Lon, Science Advances.

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Romine, IC; Wiseman, RL (2020) Starting at the Beginning: Endoplasmic Reticulum Proteostasis and Systemic Amyloid Disease, Biochem J

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Lee, EJ; Chiang, WC; Kroeger, H; Bi, CX; Chao, D; Mastey, RR; Tsang SH; Chea, L; Lambert, SR; Grandjean JMD; Baumann, B; Audo, I; Kohl, S; Moore, AT; Wiseman, RL; Carroll, J; Lin, JH (2020) Multi-Exon Deletion Alleles of ATF6 Linked to Achromatopsia, JCI Insight.

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Puchades, CP; Ding, B; Song, A; Wiseman, RL; Lander GC; Glynn, SE (2019) Unique Structural Features of the Mitochondrial AAA+ Protease AFG3L2 Reveal the Molecular Basis for Activity in Health and Disease, Molecular Cell.

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Plate, L; Rius, B; Nguyen, B; Genereux, JC; Kelly, JW; Wiseman, RL (2019) Quantitative Interactome Proteomics Reveals a Molecular Basis for ATF6-dependent Regulation of a Destabilized, Amyloidogenic Protein, Cell Chem Biol.

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Carreras-Sureda, A; Jana, F; Urra, H; Durand, S; Mortenson, DE; Sagredo, A; Bustos, G; Hazari, Y; Ramos-Fernandez, E; Sassano, ML; Pihan, P; van Vliet, AR; Gonzalez-Quiroz, M; Torres, AK; Tapia-Rojas, C; Kerkhofs, M; Vicente, R; Kaufman, RJ; Inestrosa, NC; Gonzalez-Fillault, C; Wiseman, RL; Agostinis, P; Bultynck, G; Court, FA; Kroemer, G; Cardenas, JC; Hetz, C (2019) Non-canonical function of IRE1alpha determines mitochondria-associated endoplasmic reticulum composition to control calcium transfer and bioenergetics, Nat Cell Biol.

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Glembotski, CC; Rosarda, JD; Wiseman, RL (2019) Proteostasis and Beyond: ATF6 in Ischemic Disease, Trends in Molecular Medicine.

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Grandjean, JMD; Plate, L; Morimoto, RI; Bollong, MJ; Powers, ET; Wiseman RL (2019) Deconvoluting Stress-Responsive Signaling Pathways for Pharmacologic Activation Using Targeted RNA-sequencing, ACS Chemical Biology.

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Romine, IC; Wiseman, RL (2019) PERK Signaling Regulates Extracellular Proteostasis During Endoplasmic Reticulum Stress, Scientific Reports

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Blackwood, EA; Azizi, K; Thuerauf, DJ; Paxman, RJ; Plate, L; Kelly, JW; Wiseman, RL; Glembotski, CC (2019) Pharmacologic ATF6 Activation Confers Global Protection in Widespread Disease Models by Reprogramming Cellular Proteostasis, Nature Communications.

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Giadone, RM; Rosarda, JD; Reddy Akepati, P; Thomas, AC; Boldvbataar, B; James, MF; Wilson, AA; Sanchorawala, V; Connors, LH; Berk, JL; Wiseman, RL; Murphy, GJ (2018) A Genetically Diverse Library of ATTR Patient-Specific Induced Pluripotent Stem Cells for Disease Modeling and In Vitro Testing of Novel Therapeutics, Amyloid.

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Paxman, RJ; Plate, L; Blackwood, EA; Glembotski, CC; Powers, ET; Wiseman, RL; Kelly, JW (2018) A Small Molecule that Preferentially Activates the ATF6 Pathway of the Unfolded Protein Response is Metabolically Activated to Selectively Modify Endoplasmic Reticulum Proteins, eLIFE. co-corresponding author.

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Lebeau, J; Rainbolt, TK; Wiseman, RL (2018) Coordinating Mitochondrial Biology Through the Stress-Responsive Regulation of Mitochondrial Proteases, International Review of Cell and Molecular Biology.

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Cole, KS; Grandjean, JMD; Chen, K; Witt, CH; O’Day, J; Shoulders, MD; Wiseman, RL; Weerapana, E (2018) Characterization of an A-site Selective Protein Disulfide Isomerase A1 Inhibitor, Biochemistry.

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Lebeau, J; Saunders JM; Moraes, VWR; Madhavan, A, Madrazo, N; Anthony, MC; Wiseman, RL (2018) The PERK Arm of the Unfolded Protein Response Regulates Mitochondrial Morphology During Endoplasmic Reticulum Stress, Cell Reports.

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Kroeger, H; Grimsey, N; Paxman, R; Chiang, WC; Plate, L; Jones, Y; Shaw, PX; Trejo, J; Tsang, SH; Powers, ET; Kelly, JW; Wiseman, RL; Lin, JH (2018) The Unfolded Protein Response Regulator, ATF6, Promotes Mesodermal Differentiation, Science Signaling.

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Puchades C; Rampello, AJ; Shin, Mia; Giuliano, CJ; Wiseman, RL; Glynn, SE; Lander GC (2017) Structure of the mitochondrial inner membrane AAA+ protease YME1 reveals the mechanism of substrate processing, Science.

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Plate, L; Wiseman, RL (2017) Regulating Secretory Proteostasis Through the Unfolded Protein Response: From Function to Therapy, Trends in Cell Biology.

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Chen, KC; Qu, S; Chowdury, S; Noxon, IC; Schonhoft, JD; Plate, L; Powers, ET; Kelly, JW; Lander, GC; Wiseman, RL (2017) The Endoplasmic Reticulum HSP40 Co-chaperone ERdj3/DNAJB11 Assembles and Functions as a Tetramer, EMBO J, 36, 2296-2309.

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Chen, JJ; Genereux, JC; Suh, EH; Vartabedian, VF; Rius, Bibiana; Qu, S; Dendle, MTA; Kelly, JW; Wiseman, RL (2016) Endoplasmic Reticulum Proteostasis Influences the Oligomeric State of an Amyloidogenic Protein Secreted from Mammalian Cells, Cell Chemical Biology, 23, 1282-1293.

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Plate, Lars; Cooley, CB; Chen, JJ; Paxman, RJ; Gallagher, CM; Madoux, F; Genereux, JC; Dobbs, W; Garza, D; Spicer, TP; Scampavia, L; Brown, SJ; Rosen, H; Powers, ET; Walter, P; Hodder, P; Wiseman, RL; Kelly, JW (2016) Small Molecule ER Proteostasis Regulators that Reprogram the ER to Reduce Extracellular Protein Aggregation, eLIFE. co-corresponding author

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Plate, L; Paxman, RJ; Wiseman, RL, Kelly JW (2016) Modulating protein quality control, eLIFE. co-corresponding author

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Rainbolt, TK; Lebeau, J; Puchades, C; Wiseman, RL (2016) Reciprocal Degradation of YME1L and OMA1 Adapts Mitochondrial Proteolytic Activity During Stress, Cell Reports, 14, 2041-2049.

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Eisele, YS, Monteiro, C; Fearns, C; Encalada, SE; Wiseman, RL; Powers, ET; Kelly, JW (2015) Targeting Protein Aggregation for the Treatment of Degenerative Diseases, Nat Rev Drug Discov., 14, 759-780.

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Chen, JJ; Genereux, JC; Wiseman, RL (2015) Endoplasmic Reticulum Quality Control and Systemic Amyloid Disease: Impacting Protein Stability from the Inside Out, IUBMB Life, 67, 404-413.

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Murray, AN; Chen, W; Antonopoulos, A; Hanson, SR; Wiseman, RL; Dell, A; Haslam, SM; Powers, DL; Powers, ET; Kelly, JW (2015) Enhanced Aromatic Sequons Increase Oligosaccharyltransferase Glycosylation Efficiency and Glycan Homogeneity, Chemistry & Biology, 22, 1052-1062.

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Genereux, JC; Wiseman, RL (2015) Regulating Extracellular Proteostasis Through the Unfolded Protein Response, Prion, 9, 10-21.

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Baranczak, A; Liu, Yu; Connelly, Stephen; Han Du, W; Greiner, ER; Wiseman, RL; Eisele, YS; Bradbury, NC; Dong, J; Sharpless, KB; Noodleman, L; Wilson, IA; Encalada, SE; Kelly, JW (2015) A Fluorogenic Arylfluorosulfate for Intraorganellar Transthyretin Imaging in Living Cells and in C. elegans, J. Am. Chem. Soc., 137, 7404-7414.

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Rainbolt, TK; Saunders, JM; Wiseman RL (2015) YME1L Degradation Reduces Mitochondrial Proteolytic Capacity During Oxidative Stress, EMBO Reports, 16, 97-106.

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Genereux, JC; Qu, S; Zhou, M; Ryno, LM; Wang, S; Shoulders, MD; Kaufman, RJ; Lazmezas, CI; Kelly, JW; Wiseman RL (2015) Unfolded Protein Response-Induced ERdj3 Secretion Links ER Stress to Extracellular Proteostasis, EMBO J, 34, 4-19.

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Chen, JJ; Genereux, JC; Qu, S; Hulleman, JD; Shoulders, MD; Wiseman, RL (2014) Increasing ER Quality Control Stringency Through ATF6 Activation Reduces Secretion and Extracellular Aggregation of Destabilized Variants of an Amyloidogenic Protein, Chemistry & Biology, 21, 1564-1574.

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Cooley, CB; Ryno, LM; Plate, L; Morgan, GJ; Hulleman, JD; Kelly, JW; Wiseman RL (2014) Unfolded Protein Response Activation Reduces Secretion and Extracellular Aggregation of Amyloidogenic Immunoglobulin Light Chains, Proc. Natl. Acad. Sci. USA, 111, 13046-13051.

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Rappley, I; Monteiro, C; Novais, M; Baranczak, A; Solis, G; Wiseman, RL; Helmke, S; Maurer, MS; Coelho, T; Powers, ET; Kelly, JW (2014) Quantification of Transthyretin Kinetic Stability in Human Plasma Using Subunit Exchange, Biochemistry, 53, 1993-2006.

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Rainbolt, TK; Saunders, JM; Wiseman RL (2014) Stress-Responsive Adaptation of Mitochondria Through the ER Unfolded Protein Response. Trends in Endocrinology and Metabolism, 25, 528-537.

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Ryno, LM; Genereux, JC; Naito, T; Morimoto, RI; Powers, ET; Shoulders, MD; Wiseman, RL (2014) Characterizing the Altered Cellular Proteome Induced by Stress Independent Activation of Heat Shock Factor 1, ACS Chemical Biology, 9, 1273-1283.

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Rainbolt, TK; Atanassova, N; Genereux, JC; Wiseman, RL (2013) Stress-Regulated Translational Attenuation Adapts Mitochondrial Protein Import Through Tim17A Degradation, Cell Metabolism, 18, 908-919.

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Leung, A; Nah, SK.; Reid, W; Ebata, A; Koch, CM; Monti, S; Genereux, JC; Wiseman, RL; Wolozin, B; Connors, LH; Berk, JL; Seldin, DC; Mostoslavsky, G; Kotton, DN; Murphy, GJ (2013) Induced Pluripotent Stem Cell Modeling of Multisystemic, Hereditary Transthyretin Amyloidosis, Stem Cell Reports, 1, 451-463.

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Ryno, LM; Wiseman, RL; Kelly, JW (2013) Targeting Unfolded Protein Response Signaling Pathways to Ameliorate Protein Misfolding Diseases, Curr. Opin. Chem. Biol, 17, 346-352. co-corresponding author

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Shoulders, MD; Ryno, LM; Cooley, CB; Kelly, JW; Wiseman, RL (2013) Broadly Applicable Methodology for the Rapid and Dosable Small Molecule-Mediated Regulation of Transcription Factors in Human Cells, J. Am. Chem. Soc., 135, 8129-8135

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Shoulders, MD; Ryno, LM; Genereux, JC; Moresco, JJ; Tu, PG; Yates, III, JR; Su, AI; Kelly, JW; Wiseman, RL (2013) Stress-independent Activation of XBP1s and/or ATF6 Reveals Three Functionally Diverse ER Proteostasis Environments, Cell Reports, 3, 1279-1292

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Genereux, JC; Wiseman, RL (2011) Chemically targeting the emergent properties of a chaperone complex, Chem Biol, 18, 144-145

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