Publications

Stanton, C; Buasakdi, C; Sun, J; Levitan, I; Bora, P; Kutseikin, S; Wiseman, RL#; Bollong, MJ# (2024) The Glycolytic Metabolite Methylglyoxal Covalently Inactivates the NLRP3 Inflammasome, bioRXIV

Touvier, T; Veneri, FA; Claessens, A; Ferri, C; Mastrangelo, R; Sorgiati, N; Bianchi, F; Valenzano, S; Del Carro, U; Rivellini, C; Duong, P; Shy, ME; Kelly, JW; Svaren, J; Wiseman, RL, D’Antonio, M (2024) Activation of XBP1s attenuates disease severity in models of proteotoxic Charcot-Marie-Tooth type 1B, bioRXIV

Madrazo, N; Khattar, Z; Powers, ET; Rosarda, JD#; Wiseman, RL# (2024) Mapping Stress-Responsive Signaling Pathways Induced by Mitochondrial Proteostasis Perturbations, Mol Bio Cell #Co-corresponding authors.

Hofman, C; Blackwood, EA; Sandmann, C; Herzog, N; Gross, J; Plate, L; Wiseman, RL; Kaufman, RJ; Katus, HA; Jakobi, T; Volkers, Mirko; Glembotski, CC; Doroudgar, S (2024) ATF4 Protects Against Protein Folding During Cardiac Hypertrophy, J of Mol and Cellular Cardiology

Stanton, C; Sun, J; Nutsch, K; Rosarda, JD; Nguyen, T; Li-Ma, C; Kutseikin, S; Saez, E; Teijaro, JR; Wiseman, RL#, Bollong, MJ# (2024) Covalent Targeting as a Common Mechanism for Inhibiting NLRP3 Inflammasome Assembly, ACS Chem Bio #Co-corresponding authors.

Riesenberg, BP; Gandy, EJ; Kennedy, AS; Hunt, EG; Hurst, KE; Sedlacek, EL; Gao, P; Emanuele, MK; Modliszewski, J; Green, JM; Milner, JJ; Wiseman, RL; Thaxton, J (2023) Adaptive IRE1 Signaling Elicits T Cell Metabolic Remodeling and Tumor Control, bioRXIV

Perea, V^; Baron, KR^; Dolina, V; Aviles, Giovanni; Rosarda, JD; Guo, X; Kampmann, M; Wiseman, RL (2023) Pharmacologic Activation of a Compensatory Integrated Stress Response Kinase Promotes Mitochondrial Remodeling in PERK-deficient Cells, Cell Chemical Biology, ^equal contribution

Ibrahim, L; Stanton, C; Nutsch, K; Nguyen, T; Li-Ma, C; Ko, Y; Lander, GC; Wiseman, RL#; Bollong, MJ# (2023) Succinylation of a KEAP1 Sensor Lysine Promotes NRF2 Activation, Cell Chem Biol. #Co-corresponding authors.

Rosarda, JD; Stanton, CR; Chen, EB; Bollong, MJ; Wiseman, RL (2023) Pharmacologic Targeting of PDIA1 Inhibits NLRP3 Inflammasome Assembly and Activation, Israel Journal of Chemistry (Jeff Kelly Wolf Prize Issue)

Rosarda, JD; Giles, S; Harkins-Perry, S; Mills, E; Friedlander, M; Wiseman, RL; Eade, KT (2023) Imbalanced Unfolded Protein Response Signaling Contributes to 1-Deoxysphingolipid Retinal Toxicity, Nature Communications

Yang, J; Baron, KR, Pride, DE; Schneemann, A; Guo, X; Chen, W; Song, AS; Aviles, G; Kampmann, M; Wiseman, RL#; Lander, GC# (2023) DELE1 oligomerization promotes integrated stress response activation, Nat Struct Mol Biol, #Co-corresponding author

Perea, V; Cole, C; Lebeau, J; Dolina, V; Baron, KR; Madhavan, A; Kelly, JW; Grotjahn, DA; Wiseman, RL (2023) PERK signaling promotes mitochondrial elongation by remodeling membrane phosphatidic acid, EMBO J

Ko, Y; Hong, M; Klee, S; Kumar, S; Ibrahim, L; Nutsch, K; Stanton, C; Sondermann, P; Sandoval, B; Bulos, ML; Iaconelli, J; Chatterjee, AK; Wiseman, RL; Schultz, PG; Bollong, MJ (2023) S-lactoyl modification of KEAP1 by a reactive glycolytic metabolite activates NRF2 signaling, PNAS

Barad, BA; Medina, M; Fuentes, D; Wiseman, RL; Grotjahn, DA (2023) A surface morphometrics toolkit to quantify organellar membrane ultrastructure using cryo-electron tomography, J Cell Biol

Kline, GM; Paxman, RJ; Lin, CY; Madrazo, N; Grandjean, JM; Lee, K; Nugroho, K; Powers, ET; Wiseman, RL#; Kelly, JW# (2023) Divergent proteome reactivity influences arm-selective activation of pharmacologic endoplasmic reticulum proteostsasis regulators, ACS Chemical Biology. #Co-corresponding authors.

Jo, J; Kim, J; Ibrahim, L; Kumar, M; Iaconelli, J; So Tran, C; Moon, HR; Jung, Y; Wiseman, RL; Lairson, LL; Schultz, PG; Chatterjee, AK; Bollong, M; Yun, H (2023) Optimization of 3-aminotetrahydrothiophene 1,1-dioxides with improved potency and efficacy as non-electrophilic antioxidant response elements (ARE) activators, Bioorg Med Chem Lett

Sun, S; Wang, C; Zhao, P; Kline, G; Grandjean, JMD; Jiang, Xin; Laubaudiniere, R; Wiseman, RL; Kelly, JW; Balch, WE (2023) Gaussian Process Captures the Conversion of the Pathogenic Alpha-1-Antitrypsin Fold by ATF6 Enhanced Proteostasis, Cell Chem Biol

Lee, EJ; Diaz-Aguilar, MS; Min, H; Choi, J; Valdez Duran, DA; Grandjean, JMD; Wiseman, RL; Kroeger, H; Lin, JH (2022) Endoplasmic Reticulum and Mitochondrial Dysfunction in Achromatopsia Patient Retinal Organoids, Am Journal of Pathology

Pariollaud, M; Ibrahim, LH; Irizarry, E; Mello, RM; Chan, AB; Altman, BJ; Shaw, RJ; Bollong, MJ; Wiseman, RL, Lamia, KA (2022) Circadian disruption enhances HSF1 signaling and tumorigenesis in Kras-driven lung cancer, Science Advances

Chaudary, V; Ah Kioon, MD; Hwang, SM; Lakin, K; Kirou, KK; Zhang-Sun, J; Wiseman, RL; Spiera, RF; Crow MK; Gordon, JK; Cubillos-Ruis, JR; Barrat, FJ (2022) Chronic activation of pDCs in autoimmunity is linked to dysregulated ER stress and metabolic responses, J Exp Medicine

Mesgarzadeh, JS; Romine, IC; Smith-Cohen, EM; Grandjean, JMD; Kelly, JW; Genereux, JC; Wiseman, RL (2022) ATF6 activation reduces amyloidogenic transthyretin secretion through increased interactions with endoplasmic reticulum proteostasis factors, Cells

Deng, M; Cotter, E; Wang, YJ, Fu, X; Whittsette, AL; Lynch, JW; Wiseman, RL; Kelly, JW; Keramidas, A; Mu, TW (2022) Pharmacologic activation of ATF6 remodels the proteostasis network to rescue pathogenic GABAA receptors, Cell & Bioscience

Wiseman, RL; Mesgarzadeh, JS; Hendershot, LM (2022) Reshaping endoplasmic quality control through the unfolded protein response, Mol Cell

Mesgarzadeh, JS; Buxbaum, JN; Wiseman, RL (2022) Stress responsive regulation of extracellular proteostasis, J Cell Biol

Yang, J^; Song, A^; Wiseman, RL#; Lander, GC# (2022) Cryo-EM structure of hexameric yeast Lon (PIM1) highlights importance of conserved structural elements, J Biol Chem, ^co-first authors, #Co-corresponding author

Madhavan, A; Kok, BP; Grandjean, JMD; Albert, V; Sukiasyan, A; Rius, B; Powers, ET; Galmozzi, A; Saez, E#; Wiseman, RL# (2022) Pharmacologic IRE1/XBP1s Activation Promotes Systemic Adaptive Remodeling in Obesity, Nature Communications. #Co-corresponding author.

Rosarda, JD; Baron, KR; Nutsch, K; Kline, GM; Stanton, C; Kelly, JW; Bollong, MJ; Wiseman, RL (2021) Metabolically Activated Proteostasis Regulators Protect Against Glutamate Toxicity by Activating NRF2, ACS Chem Biol.

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 (2021) The Endoplasmic Reticulum Proteostasis Regulator ATF6 is Essential for Human Cone Photoreceptor Development, PNAS

Shin, M; Watson, ER; Novick, J; Griffin, P; Wiseman, RL#; Lander, GC# (2021) Structures of the Human LONP1 Protease Reveal Regulatory Steps Involved in Protease Activation, Nature Communications. #Co-corresponding author.

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

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

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

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

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

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

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

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.

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.

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

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.

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.

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.

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.

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

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.

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

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.

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.

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.

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.

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.

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.

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.

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.

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

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.

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.

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

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

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.

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.

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.

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.

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

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.

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

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.

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.

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.

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.

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.

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.

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.

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.

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

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

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

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