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The Kelly Group

Dr. Kelly has authored over 300 peer-reviewed publications published in prominent scientific journals including Science,NatureCellNew England Journal of MedicineProceedings of the National Academy of SciencesJournal of the American Chemical SocietyBiochemistry, and Journal of Medicinal Chemistry. For reprint requests, please click here.

2017

  • Alvarez-Garcia O, Matsuzaki T, Olmer M, Plate L, Kelly JW, Lotz MK. Regulated in development and DNA damage response 1 deficiency impairs autophagy and mitochondrial biogenesis in articular cartilage and increases severity of experimental osteoarthritis. Arthritis and Rheumatology 2017; 69:1418-1428.

  • Ardejani MS, Powers ET, Kelly JW. Using Cooperatively Folded Peptides to Measure Interaction Energies and Conformational Propensities. Acc Chem Res 2017; 50:1875-1882.

  • Chen KC, Qu S, Chowdhury S, Noxon IC, Schonhoft JD, Plate L, Powers ET, Kelly JW, Lander GC, Wiseman RL. The endoplasmic reticulum HSP40 co-chaperone ERdj3/DNAJB11 assembles and functions as a tetramer. EMBO J. 2017; 36:2296-2309.

  • Connelly S, Mortenson DE, Choi S, Wilson IA, Powers ET, Kelly JW, Johnson SM. Semi-quantitative models for identifying potent and selective transthyretin amyloidogenesis inhibitors. Bioorg Med Chem Lett. 2017; 27:3441-3449.

  • Fernandes L, Moraes N, Sagrillo F, Magalhães A, de Moraes M, Romão L, Kelly JW, Foguel D, Grimster N, Palhano F. An ortho-Iminoquinone Compound Reacts with Lysine Inhibiting Aggregation while Remodeling Mature Amyloid Fibrils. ACS Chemical Neuroscience 2017; 8:1704-1712

  • Schonhoft JD, Monteiro C, Plate L, Eisele YS, Kelly JM, Boland D, Parker CG, Cravatt BF, Teruya S, Helmke S, Maurer M, Berk J, Sekijima Y, Novais M, Coelho T, Powers ET, Kelly JW. Peptide Probes Detect Misfolded Transthyretin Oligomers in Plasma of Hereditary Amyloidosis Patients. Sci Trans Med 2017; 9:doi: 10.1126/scitranslmed.aam7621.

2016

  • Ankarcrona M, Winblad B, Monteiro C, Fearns C, Powers ET, Johansson J, Westermark GT, Presto J, Ericzon B-G, Kelly JW. Current and Future Treatment of Amyloid Diseases. J Intern Med 2016; 280:177-202

  • Baranczak A, Kelly JW. A Current Pharmacologic Agent versus The Promise of Next Generation Therapeutics to Ameliorate Protein Misfolding and/or Aggregation Diseases. Curr Op Chem Biol 2016; 32:10-21

  • Chen JJ, Genereux JC, Suh EH, Vartabedian VF, Rius B, Qu S, Dendle MT, Kelly JW, Wiseman RL. Endoplasmic Reticulum Proteostasis Influences the Oligomeric State of an Amyloidogenic Protein Secreted from Mammalian Cells. Cell Chem Biol. 2016; epub:doi: 10.1016/j.chembiol.2016.09.001.

  • Chen W, Dong J, Li S, Liu Y, Wang Y, Yoon L, Wu P, Sharpless KB, Kelly JW. Synthesis of Sulfotyrosine-Containing Peptides by Incorporating Fluorosulfated Tyrosine Using an Fmoc-Based Solid-Phase Strategy. Angew Chem Int Ed Engl. 2016; 55:1835-8.

  • Chen W, Dong J, Plate L, Mortenson DE, Brighty GJ, Li S, Liu Y, Galmozzi A, Lee PS, Hulce JJ, Cravatt BF, Saez E, Powers ET, Wilson IA, Sharpless KB, Kelly JW. Arylfluorosulfates Inactivate Intracellular Lipid Binding Protein(s) through Chemoselective SuFEx Reaction with a Binding-site Tyr Residue. J Am Chem Soc 2016; 138:7353-7364.

  • Chen W, Kong L, Connelly S, Dendle JM, Liu Y, Wilson IA, Powers ET, Kelly JW. Stabilizing the CH2 domain of an Antibody by Engineering in an Enhanced Aromatic Sequon. ACS Chem Biol 2016; 11:1852-1861.

  • Coelho T, Merlini G, Bulawa CE, Fleming JA, Judge DP, Kelly JW, Maurer MS, Plante-Bordeneuve V, Labaudiniere R, Mundayat R, Riley S, Lombardo I, Huertas P. Mechanism of Action and Clinical Application of Tafamidis in Hereditary Transthyretin Amyloidosis. Neurol Ther 2016; 5:1-25

  • Dave K, Jager M, Nguyen H, Kelly JW, Gruebele M. High Resolution mapping of the folding transition state of a WW domain. J Mol Biol 2016; 428:1617-1636.

  • Kurian SM, Novais M, Whisenant T, Gelbart T, Buxbaum JN, Kelly JW, Coelho T, Salomon DR. Peripheral Blood Cell Gene Expression Diagnostic for Identifying Symptomatic Transthyretin Amyloidosis Patients: Male and Female Specific Signatures. Theranostics 2016; 6:1792-809.

  • Lim KH, Dasari AKR, Hung I, Gan Z, Kelly JW, Wemmer DE. Structural Changes Associated with Transthyretin Misfolding and Amyloid Formation Revealed by Solution and Solid-State NMR. Biochemistry 2016; 55:1941-1944.

  • Morgan GJ, Kelly JW. The Kinetic Stability of a Full-Length Antibody Light Chain Dimer Determines whether Endoproteolysis Can Release Amyloidogenic Variable Domains. J Mol Biol. 2016; epubdoi: 10.1016/j.jmb.2016.08.021

  • Plate L, 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. Small Molecule Proteostasis Regulators that Reprogram the ER to Reduce Extracellular Protein Aggregation. eLife 2016; 5;e15550

2015

  • Baranczak A, Liu Y, Connelly S, Du WG, Greiner ER, Genereux JC, Wiseman RL, Eisele YS, Bradbury NC, Dong J, Noodleman L, Sharpless KB, Wilson IA, Encalada SE, Kelly JW. A fluorogenic aryl fluorosulfate for intraorganellar transthyretin imaging in living cells and in Caenorhabditis elegans. J Am Chem Soc. 2015;137:7404-14

  • Cho YH, Baranczak A, Helmke S, Teruya S, Horn EM, Maurer MS, Kelly JW. Personalized Medicine Approach for Optimizing the Dose of Tafamidis to Potentially Ameliorate Wild-type Transthyretin Amyloidosis (Cardiomyopathy). Amyloid 2015;22: 175-180.

  • Cho YH, Zhang X, Pobre KFR, Liu Y, Powers DL, Kelly JW, Gierasch LM, Powers ET. Individual and Collective Contributions of Chaperoning and Degradation to Protein Homeostasis in E. coli. Cell Reports 2015; 11: 321-333

  • Eisele YS, Monteiro C, Fearns C, Encalada SE, Wiseman RL, Powers ET, Kelly JW. Targeting Protein Aggregation to Ameliorate Degenerative Diseases. Nature Rev. Drug Discovery 2015; 14: 759-780.

  • Genereux JC, Qu S, Zhou M, Cooley CB, Ryno LM, Madrazo N, Rainbolt TK, Wang S, Shoulders MD, Kaufman RJ, Lasmezas CI, Kelly JW, Wiseman RL. Unfolded Protein Response-induced ERdj3 Secretion Links ER Stress to Extracellular Proteostasis. EMBO J. 2015; 34, 4-19.

  • Hulleman JD, Kelly JW. Genetic Ablation of N-linked Glycosylation Reveals Two Key Folding Pathways for R345W Fibulin-3, a Protein Associated with Retinal Degeneration. FASEB J. 2015; 29, 565-575.

  • Liu Y, Zhang X, Chen W, Tan YL, Kelly JW. Fluorescence Turn-On Folding Sensor To Monitor Proteome Stress in Live Cells. J Am Chem Soc. 2015;137:11303-11.

  • Murray AN, Chen W, Antonopoulos A, Hanson SR, Wiseman RL, Dell A, Haslam SM, Powers, D.L.; Powers, E.T.; Kelly, J.W. Enhanced Aromatic Sequons Increase Oligosaccharyltransferase Glycosylation Efficiency and Glycan Homogeneity. Chem. Biol. 2015; 22: 1052-1062.

2014

  • Cooley CB, Ryno LM, Plate L, Morgan GJ, Hulleman JD, Kelly JW, Wiseman RL. Unfolded protein response activation reduces secretion and extracellular aggregation of amyloidogenic immunoglobulin light chain.  Proc Natl Acad Sci U S A.  2014 111 13046-13051

  • Greiner ER, Kelly JW, Palhano FL. Immunoprecipitation of Amyloid Fibrils by the Use of an Antibody that Recognizes a Generic Epitope Common to Amyloid Fibrils.  PLoS One  2014 9 e105433

  • Hebert DN, Lamriben L, Powers ET, Kelly JW. The intrinsic and extrinsic effects of N-linked glycans on glycoproteostasis. .  Nat Chem Biol.  2014 10 902-10

  • Liu Y, Tan YL, Zhang X, Bhabha G, Ekiert DC, Genereux JC, Cho Y, Kipnis Y, Bjelic S, Baker D, Kelly JW. Small molecule probes to quantify the functional fraction of a specific protein in a cell with minimal folding equilibrium shifts.  Proc Natl Acad Sci U S A. .  2014 111 4449-54

  • Liu Y, Zhang X, Tan YL, Bhabha G, Ekiert D, Kipnis Y, Bjelic S, Baker d, Kelly JW. De novo-designed enzymes as small-molecule-regulated fluorescence imaging tags and fluorescent reporters. J. Am. Chem. Soc. 2014 136 13102-13105

  • Rappley I, Monteiro C, Novais M, Baranczak A, Solis G, Wiseman RL, Helmke S, Maurer MS, Coelho T, Powers ET, Kelly JW. Quantification of transthyretin kinetic stability in human plasma using subunit exchange. Biochemistry  2014 53 1993-2006

  • Tan YL, Genereux JC, Pankow S, Aerts JM, Yates JR 3rd, Kelly JW. ERdj3 is an Endoplasmic Reticulum Degradation Factor for Mutant Glucocerebrosidase Variants Linked to Gaucher's Disease. Chem Biol. 2014 21 967-76>

  • Zhang X, Liu Y, Genereux JC, Nolan C, Singh M, Kelly JW. The Heat-Shock Response Transcriptional Program Enables High-Yield and High-Quality Recombinant Protein Production in Escherichia coli. ACS Chem Biol.  2014 Jul 22. 9 1945-1949

2013

  • Baranczak A, Connelly S, Liu Y, Choi S, Grimster N, Powers ET, Wilson IA, Kelly JW. Fluorogenic Small Molecules Requiring Reaction with a Specific Protein to Create a Fluorescent Conjugate for Biological Imaging - ­What we Know and What we Need to Learn. Biopolymers 2013 101:484-495

  • Chen W, Enck S, Price JL, Powers DL, Powers ET, Wong CH, Dyson HJ, Kelly JW. Structural and energetic basis of carbohydrate-aromatic packing interactions in proteins. J. Am. Chem. Soc.2013;135:9877-9884.

  • Fearns C, Connelly S, Powers ET, Kelly JW. Development of Therapeutic Strategies for the Transthyretin Amyloidoses. InAmyloid Fibrils and Prefibrillar Aggregates. Molecular and Biological Properties. Otzen DE (ed.) Wiley-VCH, Weinheim, Germany. 2013; 373-394.

  • Grimster NP, Connelly S, Baranczak A, Dong J, Krasnova LB, Sharpless KB, Powers ET, Wilson IA, Kelly JW. Aromatic Sulfonyl Fluorides Covalently Kinetically Stabilize Transthyretin to Prevent Amyloidogenesis while Affording a Fluorescent Conjugate.J. Am. Chem. Soc. 2013; 135:5656-5668.

  • Hulleman JD, Brown SJ, Rosen H, Kelly JW. A High-Throughput Cell-Based Gaussia Luciferase Assay for Identifying Modulators of Fibulin-3 Secretion. J. Biomol. Screen. 2013 18:647-658.

  • Murray AN, Palhano FL, Bieschke J, Kelly JW. Surface adsorption considerations when working with amyloid fibrils in multi-well plates and eppendorf tubes. Protein Sci. 2013 22:1531-1541.

  • Ong DS, Wang YJ, Tan YL, Yates JR, Mu TW, Kelly JW. FKBP10 Depletion Enhances Glucocerebrosidase Proteostasis in Gaucher disease Fibroblasts. Chem. Biol. 2013; 20:403-415.

  • Palhano FL, Lee J, Grimster NP, Kelly JW. Toward the Molecular Mechanism(s) by Which EGCG Treatment Remodels Mature Amyloid Fibrils.J Am Chem Soc. 2013 135:7503-7510.

  • Penchala SC, Connelly S, Wang Y, Park MS, Zhao L, Baranczak A, Rappley I, Vogel H, Liedtke M, Witteles RM, Powers ET, Reixach N, Chan WK, Wilson IA, Kelly JW, Graef IA, Alhamadsheh MM. AG10 inhibits amyloidogenesis and cellular toxicity of the familial amyloid cardiomyopathy-associated V122I transthyretin. Proc. Natl. Acad. Sci. USA. 2013;110:9992-9997.

  • Ryno LM, Wiseman RL, Kelly JW. Targeting unfolded protein response signaling pathways to ameliorate protein misfolding diseases. Curr Opin Chem Biol. 2013 17:346-352.

  • Shoulders MD, Ryno LM, Genereux JC, Moresco JJ, Tu PG, Wu C, Yates JR 3rd, Su AI, Kelly JW, Wiseman RL. Stress-Independent Activation of XBP1s and/or ATF6 Reveals Three Functionally Diverse ER Proteostasis Environments. Cell Rep. 2013 3:1279-92.

  • Shoulders MD, Ryno LM, Cooley CB, Kelly JW, Wiseman RL. Broadly Applicable Methodology for the Rapid and Dosable Small Molecule-Mediated Regulation of Transcription Factors in Human Cells. J. Am. Chem. Soc. 2013 135:8129-8132.

  • Suh EH, Liu Y, Connelly S, Genereux JC, Wilson IA, Kelly JW. Stilbene vinyl sulfonamides as fluorogenic sensors of and traceless covalent kinetic stabilizers of transthyretin that prevent amyloidogenesis. J. Am. Chem. Soc. 135:17869-80.

2012

  • Bulawa CE, Connelly S, DeVit M, Wang L, Weigel C, Fleming J, Packman J, Powers ET, Wiseman RL, Foss TR, Wilson IA, Kelly JW, Labaudiniere R. Tafamidis, A Potent and Selective Transthyretin Kinetic Stabilizer That Inhibits the Amyloid CascadeProc. Natl. Acad. Sci. 2012; 109:9629-9634.

  • Coelho T, Maia LF, Martins da Silva A, Cruz MW, Planté-Bordeneuve V, Lozeron P, Suhr OB, Campistol JM, Conceiçao I, Schmidt H, Trigo P, Kelly JW, Labaudiniere R, Chan J, Packman J, Wilson A, Grogan DR. Tafamidis for transthyretin familial amyloid polyneuropathy: a randomized, controlled trial. Neurology 2012; 79:785-792.

  • Solomon JP, Page LJ, Balch WE, Kelly JW. Gelsolin Amyloidosis: Genetics, Biochemistry, Pathology and Possible Strategies for Therapeutic Intervention. Crit. Rev. Biochem. Mol. Biol. 2012; 47:282-296.

  • Price JL, Culyba EK, Chen W, Murray A, Hanson SR, Wong C-H, Powers ET, Kelly JW. N-glycosylation of the Enhanced Aromatic Sequon to Increase Glycoprotein Stability.Biopolymers 2012; 98:195-211.

  • Johnson SM, Connelly S, Fearns C, Powers ET, Kelly JW. The Transthyretin Amyloidoses: From Delineating the Molecular Mechanism of Aggregation Linked to Pathology to a Regulatory Agency Approved Drug. J. Mol. Biol.2012; 421:185-203.

  • Hulleman JD, Balch WE, Kelly JW. Translational Attenuation Differentially Alters the Fate of Disease-associated Fibulin Proteins. FASEB J. 2012; 26:4548-4560.

2011

  • Hulleman JD, Kaushal S, Balch WE, Kelly JW. Compromised EFEMP1 Secretion Associated with Macular Dystrophy Remedied by Proteostasis Network Alteration. Mol. Biol. Cell2011; 22:4765-4775.

  • Price JL, Powers ET, Kelly JW. N-PEGylation of a Reverse Turn is Stabilizing in Multiple Sequence Contexts unlike N-GlcNAcylation. ACS Chem. Biol. 2011; 6:1188-1192.

  • Price JL, Powers DL, Powers, ET, Kelly JW. Glycosylation of the Enhanced Aromatic Sequon is Similarly Stabilizing in Three Distinct Reverse Turn Contexts. Proc. Natl. Acad. Sci. USA 2011; 108:14127-14132.

  • Lee J, Culyba EK, Powers ET, Kelly JW. Amyloid-β forms fibrils by nucleated conformational conversion of oligomers.Nat. Chem. Biol. 2011; 7:602-609.

  • Bourgault S, Choi S, Buxbaum JN, Kelly JW, Price JL, Reixach N. Mechanisms of cardiomyocyte toxicity inhibition by resveratrol analogs. Biochem. Biophys. Res. Commun. 2011;410:707-713.

  • Wiseman RL, Kelly JW. Cell biology. Phosphatase inhibition delays translational recovery. Science 2011; 332:44-45.

  • Solomon JP, Bourgault S, Powers ET, Kelly JW. Heparin Binds 8 kDa Gelsolin Cross-β-Sheet Oligomers and Accelerates Amyloidogenesis by Hastening Fibril Extension. Biochemistry2011; 50:2486-2498.

  • Culyba EK, Price JL, Hanson SR, Dhar A, Wong CH, Gruebele M, Powers ET, Kelly JW. Protein native-state stabilization by placing aromatic side chains in N-glycosylated reverse turns.Science 2011; 331:571-575.

  • Bourgault S, Solomon JP, Reixach N, Kelly JW. Sulfated glycosaminoglycans accelerate transthyretin amyloidogenesis by quaternary structural conversion.Biochemistry 2011; 50:1001-1015.

  • Ong DS, Kelly JW. Chemical and/or biological therapeutic strategies to ameliorate protein misfolding diseases. Curr Opin Cell Biol. 2011; 23:231-238.

2010

  • Choi S, Ong DST, Kelly JW. A Stilbene that Binds Selectively to Transthyretin in Cells and Remains Dark Until it Undergoes a Chemoselective Reaction to Create a Bright Blue Fluorescent Conjugate. J. Am. Chem. Soc. 2010;132:16043-16051. 

  • Choi S, Connelly S, Reixach N, Wilson IA, Kelly JW. Highly Chemoselective Small Molecules That Covalently Modify One Lysine in a Non-enzyme Protein in Plasma. Nat. Chem. Biol.2010; 6:133-139.

  • Choi S, Reixach N, Connelly S, Johnson SM, Wilson IA, Kelly JW. A Substructure Combination Strategy to Create Potent and Selective Transthyretin Kinetic Stabilizers that Prevent Amyloidogenesis and Cytotoxicity. J. Am. Chem. Soc. 2010;132:1359-1370

  • Connelly S, Choi S, Johnson SM, Kelly JW, Wilson IA. Structure-Based Design of Kinetic Stabilizers That Ameliorate the Transthyretin Amyloidoses. Curr. Op. Struct. Biol. 2010;20:54-62.

  • Hutt DM, Herman DM, Rodriques APC, Noel S, Pilewski JM, Matteson J, Hoch B, Kellner W, Kelly JW, Richardson J, Thomas PJ, Matsumura Y, Skach WR, Gentzsh M, Riordan JR, Sorscher EJ, Okiyonad T, Lukacs GL, Frizzell RA, Manning G, Gottesfeld JM, Balch WE. Reduced Histone Deacetylase 7 Activity Restores Function to Misfolded CFTR in Cystic Fibrosis. Nat. Chem. Biol. 2010;6:25-33.

  • Johnson SM, Wiseman RL, Reixach N, Paulsson J, Choi S, Powers ET, Buxbaum JN, Kelly JW. Understanding and Ameliorating the Transthyretin Amyloidoses in Protein Misfolding Diseases: Current and Emerging Principles Eds: Ramirez-Alvarado M, Kelly JW, Dobson C. 2010 Wiley Blackwell.

  • Naik S, Haque I, Degner N, Kornlayev B, Bomhoff G, Hodges J, Khorassani A-A, Katayama H, Morris J, Kelly JW, Seed J, Fisher MT. Identifying Protein Stabilizing Ligands using GroEL.Biopolymers 2010; 93:237-251.

  • Ong DST, Mu T-W, Palmer, AE, Kelly JW. Endoplasmic reticulum Ca2+ increases enhance mutant glucocerebrosidase proteostasis. Nat. Chem. Biol. 2010;6:424-432.

  • Price JL, Shental-Bechor D, Dhar A, Turner MJ, Powers ET, Gruebele M,Levy Y, Kelly JW. Context-Dependent Effects of Asparagine Glycosylation on Pin WW Folding Kinetics and Thermodynamics. J. Am. Chem. Soc. 2010;132:15359-15367.

2009

  • Bieschke J, Cohen E, Murray A, Dillin A, Kelly JW. A Kinetic Assessment of the C.elegans Amyloid Disaggregation Activity Enables Uncoupling of Disassembly and Proteolysis. Protein Sci. 2009; 18:2231-2241.

  • Bunagan MR, Gao J, Kelly JW, Gai F. Probing the Folding Transition State Structure of the Villin Headpiece Subdomain via Sidechain and Backbone Mutagenesis. J. Am. Chem. Soc. 2009; 131:7470-7476.

  • Choi S, Reixach N, Connelly S, Johnson SM, Wilson IA, Kelly JW. A Substructure Combination Strategy to Create Potent and Selective Transthyretin Kinetic Stabilizers that Prevent Amyloidogenesis and Cytotoxicity. J. Am. Chem. Soc. 2009; 132:1359-1370.

  • Cohen E, Cohen T-B, Paulsson JF, Du D, Estepa G, Pham HM, Kelly JW, Masliah E, Dillin A. Reduced IGF-1 Signaling Delays Age-associated Proteotoxicity in Mice. Cell 2009; 139:1157-1169.

  • Fowler DM, Kelly JW. Aggregating Knowledge about Prions and Amyloid. Cell 2009; 137:20-22.

  • Fuller AA, Du D, Liu F, Davoren JE, Kroon G, Powers ET, Wipf P, Gruebele M, Kelly JW. Evaluating β-turn Mimics as β-sheet Folding Nucleators. Proc. Natl. Acad. Sci. 2009; 106:11067-11072.

  • Gao J, Bosco DA, Powers ET, Kelly JW. Localized Thermodynamic Coupling between Hydrogen Bonding and Microenvironment Polarity Significantly Stabilizes Proteins. Nat. Struct. Mol. Biol. 2009; 16:684-690.

  • Hanson SR, Culyba EK, Hsu T-L, Wong C-H, Kelly JW, Powers ET. The Core Trisaccharide of an N-linked Glycoprotein Intrinsically Accelerates Folding and Enhances Stability. Proc. Natl. Acad. Sci. 2009; 106:3131-3136.

  • Jager M, Dendle M, Kelly JW. Sequence Determinants of Thermodynamic Stability in a WW domain–an all β-sheet protein. Protein Sci. 2009; 18:1806-1813.

  • Johnson SW, Connelly S, Wilson IA, Kelly JW. Toward Optimization of the Second Aryl Substructure Common to Transthyretin Amyloidogenesis Inhibitors Using Biochemical and Structural Studies. J. Med. Chem. 2009; 52:1115-1125.

  • Page LJ, Suk J-Y, Bazhenova L, Fleming SM, Wood M, Guo LT, Misizin AP, Kisilevsky R, Shelton DG, Balch WE, Kelly JW. Secretion of Amyloidogenic Gelsolin Progressively Compromises Protein Homeostasis Leading to the Intracellular Aggregation of Proteins. Proc. Natl. Acad. Sci. 2009; 106:11125-11130.

  • Powers ET, Morimoto RI, Dillin A, Kelly JW, Balch WE. Biological and Chemical Approaches to Diseases of Proteostasis Deficiency. Ann. Rev. Biochem. 2009; 78;959-91.

  • Solomon JP, Yonemoto IT, Murray AN, Price JL, Powers ET, Kelly JW. Plasma Gelsolin 5 and 8 kDa Fragments Form Amyloid by a Nucleated Polymerization Mechanism, while the 68 kDa Fragment is Not Amyloidogenic. Biochemistry 2009; 48:11370-11380.

  • Usui K, Hulleman JD, Paulsson JF, Siegel SJ, Powers ET, Kelly JW. Site-specific modification of Alzheimer’s peptides by cholesterol oxidation products enhance aggregation energetics and neurotoxicity. Proc. Natl. Acad. Sci. 2009; 106:18563-18568.

  • Watt B, van Niel G, Fowler DM, Hurbain I, Luk KC, Stayrook SE, Lemmon MA, Raposo G, Shorter J, Kelly JW, Marks MS. N-Terminal Domains Elicit Formation of Functional Pmel17 Amyloid Fibrils. J. Biol. Chem. 2009; 284:35543-35555.

  • Yonemoto IT, Wood MR, Balch WE, Kelly JW. A General Strategy for the Bacterial Expression of Amyloidogenic Peptides using BCL-XL-1/2 Fusions. Protein Sci. 2009; 18:1978-1986.

2008

  • Balch WE, Morimoto RI, Dillin A, Kelly JW. Adapting Proteostasis for Disease Intervention. Science 2008; 319:916-919.

  • Bieschke J, Siegel SJ, Fu Y, Kelly JW. Alzheimer's Abeta Peptides Containing an Isostructural Backbone Mutation Afford Distinct Aggregate Morphologies but Analogous Cytotoxicity. Evidence for a Common Low-Abundance Toxic Structure(s)? Biochemistry 2008; 47:50-59.

  • Gao J, Kelly W. Toward quantification of protein backbone-backbone hydrogen bonding energies: An energetic analysis of an amide-to-ester mutation in an alpha-helix within a protein. Protein Sci. 2008; 17:1096-1101.

  • Hurschman Babbes AR, Powers ET, Kelly JW. Quantification of the thermodynamically linked quaternary and tertiary structural stabilities of transthyretin and its disease-associated variants: the relationship between stability and amyloidosis. Biochemistry 2008; 47:6969-84.

  • Jager M, Deechongkit S, Koepf EK, Nguyen H, Gao J, Powers ET, Gruebele M, Kelly JW. Understanding the mechanism of beta-sheet folding from a chemical and biological perspective. Biopolymers 2008; 90:751-758.

  • Johnson SM, Connelly S, Wilson IA, Kelly JW. Biochemical and structural evaluation of highly selective 2-arylbenzoxazole-based transthyretin amyloidogenesis inhibitors. J. Med. Chem. 2008; 51:260-270.

  • Johnson SM, Connelly S, Wilson IA, Kelly JW. Toward optimization of the linker substructure common to transthyretin amyloidogenesis inhibitors using biochemical and structural studies. J. Med. Chem. 2008; 51:6348-6358.

  • Liu F, Du D, Fuller AA, Davoren JE, Wipf P, Kelly JW, Gruebele M. An experimental survey of the transition between two-state and downhill protein folding scenarios. Proc. Natl. Acad. Sci. U.S.A. 2008; 105:2369-2374.

  • Mu TW, Fowler DM, Kelly JW. Partial restoration of mutant enzyme homeostasis in three distinct lysosomal storage disease cell lines by altering calcium homeostasis. PLoS Biology 2008; 6:e26.

  • Mu TW, Ong DST, Wang YJ, Balch WE, Yates JR III, Segatori L, Kelly JW. Chemical and biological approaches synergize to ameliorate protein-folding diseases. Cell 2008; 134:769-781.

  • Palaninathan SK, Mohamedmohaideen NN, Snee WC, Kelly JW, Sacchettini JC. Structural insights into pH-induced conformational changes within the native human transthyretin tetramer. J. Biol. Chem. 2008; 382:1157-1167.

  • Reixach N, Foss TR, Santelli E, Pascual J, Kelly JW, Buxbaum JN. Human-Murine Transthyretin Heterotetramers Are Kinetically Stable and Non-amyloidogenic: A lesson in the generation of transgenic models of diseases involving oligomeric proteins. J. Biol. Chem. 2008; 283:2098-2107.

  • Sekijima Y, Kelly JW, Kieda S. Pathogenesis of and therapeutic strategies to ameliorate the transthyretin amyloidoses. Curr. Pharm. Design 2008; 14:3219-3230.

  • Yonemoto IT, Kroon GJ, Dyson HJ, Balch WE, Kelly JW. Amylin proprotein processing generates progressively more amyloidogenic peptides that initially sample the helical state. Biochemistry 2008; 47:9900-9910.

2007

  • Dillin A, Kelly JW. Medicine. The yin-yang of sirtuins. Science 2007; 317:461-462.

  • Fowler DM, Koulov AV, Balch WE, Kelly JW. Functional amyloid--from bacteria to humans. Trends Biochem. Sci. 2007; 32:217-224.

  • Jager M, Dendle M, Fuller AA, Kelly JW. A cross-strand Trp-Trp pair stabilizes the hPin1 WW domain at the expense of function. Protein Sci. 2007; 16:2306-2313.

  • Jager M, Nguyen H, Dendle M, Gruebele M, Kelly JW. Influence of hPin1 WW N-terminal domain boundaries on function, protein stability, and folding. Protein Sci. 2007; 16:1495-1501.

  • Munch J, Rucker E, Standker L, Adermann K, Goffinet C, Schindler M, Wildum S, Chinnadurai R, Rajan D, Specht A, Gimenez-Gallego G, Sanchez PC, Fowler DM, Koulov A, Kelly JW, Mothes W, Grivel JC, Margolis L, Keppler OT, Forssmann WG, Kirchhoff F. Semen-derived amyloid fibrils drastically enhance HIV infection. Cell 2007; 131:1059-1071.

  • Siegel SJ, Bieschke J, Powers ET, Kelly JW. The oxidative stress metabolite 4-hydroxynonenal promotes Alzheimer protofibril formation. Biochemistry 2007; 46:1503-1510.

  • Stewart CR, Wilson LM, Zhang Q, Pham CL, Waddington LJ, Staples MK, Stapleton D, Kelly JW, Howlett GJ. Oxidized cholesterol metabolites found in human atherosclerotic lesions promote apolipoprotein C-II amyloid fibril formation. Biochemistry 2007; 46:5552-5561.

  • Wiseman RL, Koulov A, Powers E, Kelly JW, Balch WE. Protein energetics in maturation of the early secretory pathway. Curr. Opin. Cell Biol. 2007; 19:359-367.

  • Wiseman RL, Powers ET, Buxbaum JN, Kelly JW, Balch WE. An adaptable standard for protein export from the endoplasmic reticulum. Cell 2007; 131:809-821.

  • Yu Z, Sawkar AR, Kelly JW. Pharmacologic chaperoning as a strategy to treat Gaucher disease. FEBS J 2007; 274:4944-4950.

  • Yu Z, Sawkar AR, Whalen LJ, Wong CH, Kelly JW. Isofagomine- and 2,5-anhydro-2,5-imino-D-glucitol-based glucocerebrosidase pharmacological chaperones for Gaucher disease intervention. J. Med. Chem. 2007; 50:94-100.

2006

  • Wang X, Venable J, LaPointe P, Hutt DM, Koulov AV, Coppinger J, Gurkan C, Kellner W, Matteson J, Plutner H, Riordan JR, Kelly JW, Yates JR III, Balch WE. Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis. Cell 2006; 127:803-815.

  • Tojo K, Sekijima Y, Kelly JW, Ikeda S-i. Diflunisal stabilizes familial amyloid polyneuropathy-associated transthyretin variant tetramers in serum against dissociation required for amyloidogenesis. Neuroscience Res. 2006; 56:441-449.

  • Fu Y, Gao J, Bieschke J, Dendle MA, Kelly JW. Amide-to-E-olefin versus amide-to-ester backbone H-bond perturbations: evaluating the O-O repulsion for extracting H-bond energies. J. Am. Chem. Soc. 2006; 128:15948-15949.

  • Zhang Y, Kim Y, Genoud N, Gao J, Kelly JW, Pfaff SL, Gill GN, Dixon JE, Noel JP. Determinants for dephosphorylation of the RNA polymerase II C-terminal domain by Scp1. Mol. Cell 2006; 24:759-770.

  • Reixach N, Adamski-Werner SL, Kelly JW, Koziol J, Buxbaum JN. Cell based screening of inhibitors of transthyretin aggregation. Biochem. Biophys. Res. Comm. 2006; 348:889-897.

  • Cordeiro Y, Kraineva J, Suarez MC, Tempesta AG, Kelly JW, Silva JL, Winter R, Foguel D. Fourier transform infrared spectroscopy provides a fingerprint for the tetramer and for the aggregates of transthyretin. Biophys. J. 2006; 91:957-967.

  • Bieschke J, Zhang Q, Bosco DA, Lerner RA, Powers ET, Wentworth P, Kelly JW. Small molecule oxidation products trigger disease-associated protein misfolding. Acc. Chem. Res. 2006; 39:611-619.

  • Kelly JW. Proteins downhill all the way. Nature 2006; 442:255-256.

  • Cohen E, Bieschke J, Perciavalle RM, Kelly JW, Dillin A. Opposing activities protect against age onset proteotoxicity. Science 2006; 313:1604-1610.

  • Sekijima Y, Dendle MA, Kelly JW. Orally administered diflunisal stabilizes transthyretin against dissociation required for amyloidogenesis. Amyloid 2006; 13:236-249.

  • Jager M, Zhang Y, Bieschke J, Nguyen H, Dendle M, Bowman M, Noel JP, Gruebele M, Kelly JW. The structure-function-folding relationship in a WW domain. Proc. Natl. Acad. Sci. USA 2006; 103:10648-10653.

  • Sawkar AR, Schmitz M, Zimmer K-P, Reczek D, Edmunds T, Balch WE, Kelly JW. Chemical chaperones and permissive temperatures alter the cellular localization of Gaucher disease associated glucocerebrosidase variants. ACS Chem. Biol. 2006; 1:235-251.

  • Kelly JW, Balch WE. The integration of cell and chemical biology in protein folding. Nat. Chem. Biol. 2006; 2:224-227.

  • Sorgjerd K, Ghafouri B, Jonsson B-H, Kelly JW, Blond SY, Hammarstrom P. Retention of misfolded mutant transthyretin by the chaperone BiP/GRP78 mitigates amyloidogenesis. J. Mol. Biol. 2006; 356:469-482.

  • Bosco DA, Fowler DM, Zhang Q, Nieva J, Powers ET, Wentworth P, Lerner RA, Kelly JW. Elevated levels of oxidized cholesterol metabolites in Lewy body disease brains accelerate α-synuclein fibrilization. Nat. Chem. Biol.2006; 2:249-253.

  • Sawkar AR, D'Haeze W, Kelly JW. Therapeutic strategies to ameliorate lysosomal storage disorders - a focus on Gaucher disease. Cell. Mol. Life Sci. 2006; 63:1179-1192.

  • Sekijima Y, Dendle MT, Wiseman RL, White JT, D'Haeze W, Kelly JW. R104H may suppress transthyretin amyloidogenesis by thermodynamic stabilization, but not by the kinetic mechanism characterizing T119M interallelic trans-suppression. Amyloid 2006; 13:57-66.

  • Deechongkit S, Nguyen H, Jager M, Powers ET, Gruebele M, Kelly JW. β-sheet folding mechanisms from perturbation energetics. Curr. Opin. Struct. Biol. 2006; 16:94-101.

  • Suk JY, Zhang F, Balch WE, Linhardt R, Kelly JW. Heparin accelerates gelsolin amyloidogenesis. Biochemistry 2006; 45:2234-2242.

  • Fowler DM, Koulov AV, Alory-Jost C, Marks MS, Balch WE, Kelly JW. Functional amyloid formation within mammalian tissue. PLoS Biol. 2006; 4:e6.

2005

  • Page LJ, Suk J Y, Huff ME, Lim H-J, Venable J, Yates III J, Kelly JW, Balch WE. Metalloendoprotease cleavage triggers gelsolin amyloidogenesis. EMBO J. 2005; 24:4124-4132.

  • Wiseman RL, Powers ET, Kelly JW. Partitioning conformational intermediates between competing refolding and aggregation pathways: insights into transthyretin amyloid disease. Biochemistry 2005; 44:16612-16623.

  • Fu Y, Bieschke J, Kelly JW. E-Olefin dipeptide isostere incorporation into a polypeptide backbone enables hydrogen bond perturbation: probing the requirements for Alzheimer's amyloidogenesis. J. Am. Chem. Soc. 2005; 127:15366-15367.

  • Powers ET, Deechongkit S, Kelly JW. Backbone-backbone H-bonds make context-dependent contributions to protein folding kinetics and thermodynamics: lessons from amide-to-ester mutations. Adv. Protein Chem. 2005; 72:39-78.

  • Foss TR, Wiseman RL, Kelly JW. The pathway by which the tetrameric protein transthyretin dissociates. Biochemistry 2005; 44:15525-15533.

  • Sawkar AR, Adamski-Werner SL, Cheng W-C, Wong C-H, Beutler E, Zimmer K-P, Kelly JW. Gaucher disease-associated glucocerebrosidases show mutation-dependent chemical chaperoning profiles. Chem. Biol. 2005; 12:1235-1244.

  • Kelly JW. Form and function instructions. Nature 2005; 437:486-487.

  • Johnson SM, Wiseman RL, Sekijima Y, Green NS, Adamski-Werner SL, Kelly JW. Native state kinetic stabilization as a strategy to ameliorate protein misfolding diseases: a focus on the transthyretin amyloidoses. Acc. Chem. Res. 2005; 38:911-921.

  • Green NS, Foss T, Kelly JW. Genistein, a natural product from soy, is a potent inhibitor of transthyretin amyloidosis. Proc. Natl. Acad. Sci. USA 2005; 102:14545-14550.

  • Nguyen H, Jager M, Kelly JW, Gruebele M. Engineering a β-sheet protein toward the folding speed limit. J. Phys. Chem. B 2005; 109:15182-15186.

  • Frankenfield KN, Powers ET, Kelly JW. Influence of the N-terminal domain on the aggregation properties of the prion protein. Protein Sci. 2005; 14:2154-2166.

  • Wiseman RL, Green NS, Kelly JW. Kinetic stabilization of an oligomeric protein under physiological conditions demonstrated by a lack of subunit exchange - implications for transthyretin amyloidosis. Biochemistry 2005; 44:9265-9274.

  • Premkumar L, Sawkar AR, Boldin-Adamsky S, Toker L, Silman I, Kelly JW, Futerman AH, Sussman JL. X-ray structure of human acid-β-glucosidase covalently bound to conduritol-B-epoxide: implications for Gaucher disease. J. Biol. Chem. 2005; 280:23815-23819.

  • Zhang Q, Kelly JW. Cys-10 mixed disulfide modifications exacerbate transthyretin familial variant amyloidogenicity: a likely explanation for variable clinical expression of amyloidosis and the lack of pathology in C10S/V30M transgenic mice. Biochemistry 2005; 44:9079-9085.

  • Deechongkit S, Powers ET, You S-L, Kelly JW. Controlling the morphology of cross β-sheet assemblies by rational design. J. Am. Chem. Soc. 2005; 127:8562-8570.

  • Bieschke J, Zhang Q, Powers ET, Lerner RA, Kelly JW. Oxidative metabolites accelerate Alzheimer's amyloidogenesis by a two-step mechanism, eliminating the requirement for nucleation. Biochemistry 2005; 44:4977-4983.

  • You S-L, Kelly JW. Total synthesis of didmolamides A and B. Tetrahedron Lett. 2005; 46:2567-2570.

  • Petrassi HM, Johnson SM, Purkey HE, Chiang KP, Walkup T, Jiang X, Powers ET, Kelly JW. Potent and selective structure-based dibenzofuran inhibitors of transthyretin amyloidogenesis: kinetic stabilization of the native state. J. Am. Chem. Soc. 2005; 127:6662-6671.

  • Wiseman RL, Johnson SM, Kelker MS, Foss T, Wilson IA, Kelly JW. Kinetic stabilization of an oligomeric protein by a single ligand binding event. J. Am. Chem. Soc. 2005; 127:5540-5551.

  • Sekijima Y, Wiseman RL, Matteson J, Hammarstrom P, Miller SR, Sawkar AR, Balch WE, Kelly JW. The biological and chemical basis for tissue-selective amyloid disease. Cell 2005; 121:73-85.

  • Foss TR, Kelker MS, Wiseman RL, Wilson IA, Kelly JW. Kinetic stabilization of the native state by protein engineering: implications for inhibition of transthyretin amyloidogenesis. J. Mol. Biol. 2005; 347:841-854.


  • Razavi H, Powers ET, Purkey HE, Adamski-Werner SL, Chiang KP, Dendle MTA, Kelly JW. Design, synthesis, and evaluation of oxazole transthyretin amyloidogenesis inhibitors. Bioorg. Med. Chem. Lett. 2005; 15:1075-1078.

  • Johnson SM, Petrassi HM, Palaninathan SK, Mohamedmohaideen NN, Purkey HE, Nichols C, Chiang KP, Walkup T, Sacchettini JC, Sharpless KB, Kelly JW. Bisaryloxime ethers as potent inhibitors of transthyretin amyloid fibril formation. J. Med. Chem. 2005; 48:1576-1587.

  • You S-L, Kelly JW. The total synthesis of bistratamides F-I. Tetrahedron 2005; 61:241-249.

2004

  • You S-L, Deechongkit S, Kelly JW. Solid-phase synthesis and stereochemical assignments of tenuecyclamides A-D employing heterocyclic amino acids derived from commercially available Fmoc α-amino acids. Org. Lett. 2004; 6:2627-2630.

  • Deechongkit S, Nguyen H, Powers ET, Dawson PE, Gruebele M, Kelly JW. Context-dependent contributions of backbone hydrogen bonding to β-sheet folding energetics. Nature 2004; 430:101-105.

  • Purkey HE, Palaninathan SK, Kent KC, Smith C, Safe SH, Sacchettini JC, Kelly JW. Hydroxylated polychlorinated biphenyls selectively bind transthyretin in blood and inhibit amyloidogenesis: rationalizing rodent PCB toxicity. Chemistry & Biology 2004; 11:1719-1728.

  • You S-L, Kelly JW. Highly efficient enantiospecific synthesis of imidazoline-containing amino acids using bis(triphenyl)oxodiphosphonium trifluoromethanesulfonate. Org. Lett. 2004; 6:1681-1683.

  • Hurshman AR, White JP, Powers ET, Kelly JW. Transthyretin aggregation under partially denaturing conditions is a downhill polymerization. Biochemistry 2004; 43:7365-7381.

  • Reixach N, Deechongkit S, Jiang X, Kelly JW, Buxbaum JN. Tissue damage in the amyloidoses: transthyretin monomers and nonnative oligomers are the major cytotoxic species in tissue culture. Proc. Natl. Acad. Sci. USA 2004; 101:2817-2822.

  • Zhang Q, Powers ET, Nieva J, Huff ME, Dendle MA, Bieschke J, Glabe CG, Eschenmoser A, Wentworth P, Lerner RA, Kelly JW. Metabolite-initiated protein misfolding may trigger Alzheimer's disease. Proc. Natl. Acad. Sci. USA 2004; 101:4752-4757.

  • Miller SR, Sekijima Y, Kelly JW. Native state stabilization by NSAIDs inhibits transthyretin amyloidogenesis from the most common familial disease variants. Lab. Invest. 2004; 84:545-552.

  • Deechongkit S, You S-L, Kelly JW. Synthesis of all nineteen appropriately protected chiral α-hydroxy acid equivalents of the α-amino acids for Boc solid-phase depsi-peptide synthesis. Org. Lett. 2004; 6:497-500.

  • You S-L, Kelly JW. Highly efficient biomimetic total synthesis and structural verification of bistratamides E and J from Lissoclinum bistratumChem. Eur. J. 2004; 10:71-75.

  • Adamski-Werner SL, Palaninathan SK, Sacchettini JC, Kelly JW. Diflunisal analogues stabilize the native state of transthyretin. Potent inhibition of amyloidogenesis. J. Med. Chem. 2004; 47:355-374.

  • Kelker MS, Foss TR, Peti W, Teyton L, Kelly JW, Wuthrich K, Wilson IA. Crystal structure of human triggering receptor expressed on myeloid cells 1 (TREM-1) at 1.47 Å. J. Mol. Biol. 2004; 342:1237-1248.

  • Page LJ, Huff ME, Kelly JW, Balch WE. Ca2+ binding protects against gelsolin amyloidosis. Biochem. Biophys. Res. Comm. 2004; 322:1105-1110.

  • Deechongkit S, Dawson PE, Kelly JW. Toward assessing the position-dependent contributions of backbone hydrogen bonding to β-sheet folding thermodynamics employing amide-to-ester perturbations. J. Am. Chem. Soc. 2004; 126:16762-16771.

2003

  • Huff ME, Balch WE, Kelly JW. Pathological and functional amyloid formation orchestrated by the secretory pathway. Curr. Opin. Struct. Biol. 2003; 13:674-682.

  • Kustedjo K, Deechongkit S, Kelly JW, Cravatt BF. Recombinant expression, purification, and comparative characterization of Torsion A and its torsion dystonia-associated variant ΔE-Torsin A. Biochemistry 2003; 42:15333-15341.

  • You S-L,Kelly JW. Total synthesis of dendroamide A: oxazole and thiazole construction using an oxodiphosphonium salt. J. Org. Chem. 2003; 68:9506-9509.

  • DiDonato M, Craig L, Huff ME, Thayer MM, Cardosa RMF, Kassmann CJ, Lo TP, Bruns CK, Powers ET, Kelly JW, Getzoff ED, Tainer JA. ALS mutants of human superoxide dismutase form fibrous aggregates via framework destabilization. J. Mol. Biol. 2003; 332:601-615.

  • Green NS, Palaninathan SK, Sacchettini JC, Kelly JW. Synthesis and characterization of potent bivalent amyloidosis inhibitors that bind prior to transthyretin tetramerization. J. Am. Chem. Soc. 2003; 125:13404-13414.

  • Foguel D, Suarez MC, Ferrão-Gonzales AD, Porto TCR, Palmieri L, Einsiedler CM, Andrade LR, Lashuel HA, Lansbury PT, Kelly JW, Silva JL. Dissociation of amyloid fibrils of α-synuclein and transthyretin by pressure reveals their reversible nature and the formation of water-excluded cavities. Proc. Natl. Acad. Sci. USA 2003; 100:9831-9836.

  • Huff ME, Page LJ, Balch WE, Kelly JW. Gelsolin domain 2 Ca2+ affinity determines susceptibility to furin proteolysis and familial amyloidosis of Finnish type. J. Mol. Biol. 2003; 334:119-127.

  • Calarese DA, Scanlan CN, Zwick MB, Deechongkit S, Mimura Y, Kunert R, Zhu P, Wormald MR, Stanfield RL, Roux KH, Kelly JW, Rudd PM, Dwek RA, Katinger H, Burton DR, Wilson IA. Antibody domain exchange is an immunological solution to carbohydrate cluster recognition. Science 2003; 300:2065-2071.

  • Ferrão-Gonzales AD, Palmieri L, Valory M, Silva JL, Lashuel H, Kelly JW, Foguel D. Hydration and packing are crucial to amyloidogenesis as revealed by pressure studies on transthyretin variants that either protect or worsen amyloid disease. J. Mol. Biol. 2003; 328:963-974.

  • Zhang Q, Kelly JW. Cys 10 mixed disulfides make transthyretin more amyloidogenic under mildly acidic conditions. Biochemistry 2003; 42:8756-8761.

  • Kelly JW, Balch WE. Amyloid as a natural product. J. Cell Biol. 2003;161:461-462.

  • Cohen FE, Kelly JW. Therapeutic approaches to protein-misfolding diseases. Nature 2003; 426:905-910.

  • Lu JR, Perumal S, Powers ET, Kelly JW, Webster JRP, Penfold J. Adsorption of β-hairpin peptides on the surface of water: a neutron refection study. J. Am. Chem. Soc. 2003; 125:3751-3757.

  • Razavi H, Palaninathan SK, Powers ET, Wiseman RL, Purkey HE, Mohamedmohaideen NN, Deechongkit S, Chiang KP, Dendle MTA, Sacchettini JC, Kelly JW. Benzoxazoles as transthyretin amyloid fibril inhibitors: synthesis, evaluation, and mechanism of action. Angew. Chem. Int. Ed. 2003; 42:2758-2761.

  • Hammarstrom P, Sekijima Y, White JP, Wiseman RL, Lim A, Costello CE, Altland K, Garzuly F, Budka H, Kelly JW. D18G transthyretin is monomeric, aggregation prone, and not detectable in plasma and cerebrospinal fluid: a prescription for central nervous system amyloidosis? Biochemistry 2003; 42:6656-6663.

  • Nguyen H, Jäger M, Moretto A, Gruebele M, Kelly JW. Tuning the free-energy landscape of a WW domain by temperature, mutation, and truncation. Proc. Natl. Acad. Sci. USA 2003; 100:3948-3953.

  • Colfer S, Kelly JW, Powers ET. Factors governing the self-assembly of a β-hairpin peptide at the air-water interface. Langmuir 2003; 19:1312-1318.

  • Sekijima Y, Hammarstrom P, Matsumura M, Shimizu Y, Iwata M, Tokuda T, Ikeda S-i, Kelly JW. Energetic characteristics of the new transthyretin variant A25T may explain its atypical central nervous system pathology. Lab. Invest. 2003; 83:409-418.

  • Hammarstrom P, Wiseman RL, Powers ET, Kelly JW. Prevention of transthyretin amyloid disease by changing protein misfolding energetics. Science 2003; 299:713-716.

  • You S-L, Razavi H, Kelly JW. A biomimetic synthesis of thiazolines using hexaphenyloxodiphosphonium trifluoromethanesulfonate. Angew. Chem. Int. Ed. 2003; 42:83-85.

2002

  • Kelly JW. Towards an understanding of amyloidogenesis. Nat. Struct. Biol. 2002; 9:323-325.

  • Sawkar AR, Cheng W-C, Beutler E, Wong C-H, Balch WE, Kelly JW. Chemical chaperones increase the cellular activity of N370S β-glucosidase: a therapeutic strategy for Gaucher disease. Proc. Natl. Acad. Sci USA 2002; 99:15428-15433.

  • Kaul R, Deechongkit S, Kelly JW. Synthesis of a negatively charged dibenzofuran-based β-turn mimetic and its incorporation into the WW miniprotein-enhanced solubility without a loss of thermodynamic stability. J. Am. Chem. Soc. 2002; 124:11900-11907.

  • Hammarstrom P, Jiang X, Hurshman AR, Powers ET, Kelly JW. Sequence-dependent denaturation energetics: a major determinant in amyloid disease diversity. Proc. Natl. Acad. Sci. USA 2002; 99:16427-16432.

  • Liu K, Kelly JW, Wemmer DE. Native state hydrogen exchange study of suppressor and pathogenic variants of transthyretin. J. Mol. Biol. 2002; 320:821-832.

  • McCammon MG, Scott DJ, Keetch CA, Greene LH, Purkey HE, Petrassi HM, Kelly JW, Robinson CV. Screening transthyretin amyloid fibril inhibitors: characterization of novel multiprotein, multiligand complexes by mass spectrometry. Structure 2002; 10:851-863.

  • Deechongkit S, Kelly JW. The effect of backbone cyclization on the thermodynamics of β-sheet unfolding: stability optimization of the PIN WW domain. J. Am. Chem. Soc. 2002; 124:4980-4986.

  • Kowalski JA, Liu K, Kelly JW. NMR solution structure of the isolated Apo Pin1 WW domain: comparison to the X-ray crystal structures of Pin1. Biopolymers 2002; 63:111-121.

  • Sacchettini JC, Kelly JW. Therapeutic strategies for human amyloid diseases. Nat. Rev. Drug Discov. 2002; 1:267-275.

  • Oza VB, Smith C, Raman P, Koepf EK, Lashuel HA, Petrassi HM, Chiang KP, Powers ET, Sacchettini J, Kelly JW. Synthesis, structure, and activity of diclofenac analogues as transthyretin amyloid fibril formation inhibitors. J. Med. Chem. 2002; 45:321-332.

  • Powers ET, Yang SI, Lieber CM, Kelly JW. Ordered Langmuir-Blodgett films of amphiphilic β-hairpin peptides imaged by atomic force microscopy. Angew. Chem. Int. Ed. 2002; 41:127-130.

2001

  • Jiang X, Buxbaum JN, Kelly JW. The V122I cardiomyopathy variant of transthyretin increases the velocity of rate-limiting tetramer dissociation resulting in accelerated amyloidosis. Proc. Natl. Acad. Sci. USA 2001; 98:14943-14948.

  • White JT, Kelly JW. Support for the multigenic hypothesis of amyloidosis: The binding stoichiometry of retinol-binding protein, vitamin A, and thyroid hormone influences transthyretin amyloidogenicity in vitroProc. Natl. Acad. Sci. USA 2001; 98:13019-13024.

  • Chen C-D, Huff ME, Matteson J, Page L, Phillips R, Kelly JW, Balch WE. Furin initiates gelsolin familial amyloidosis in the Golgi through a defect in Ca2+ stabilization. EMBO J. 2001; 20:6277-6287.

  • Hammarstrom P, Schneider F, Kelly JW. Trans-suppression of misfolding in an amyloid disiease. Science 2001; 293:2459-2461.

  • Jager M, Nguyen H, Crane JC, Kelly JW, Gruebele M. The folding mechanism of β-sheet: the WW domain. J. Mol. Biol. 2001; 311:373-393.

  • Hammarstrom P, Jiang X, Deechongkit S, Kelly JW. Anion shielding of electrostatic repulsions in transthyretin modulates stability and amyloidosis: insight into the chaotrope unfolding dichotomy. Biochemistry 2001; 40:11453-11459.

  • Jiang X, Smith CS, Petrassi HM, Hammarstrom P, White JT, Sacchettini JC, Kelly JW. An engineered transthyretin monomer that is nonamyloidogenic, unless it is partially denatured. Biochemistry 2001; 40:11442-11452.

  • Schneider F, Hammarstrm P, Kelly JW. Transthyretin slowly exchanges subunits under physiological conditions: a convenient chromatographic method to study subunit exchange in oligomeric proteins. Protein Sci. 2001; 10:1606-1613.

  • Jiang, X, Kowalski J, Kelly JW. Increasing protein stability using a rational approach combining sequence homology and structural alignment: Stabilizing the WW domain. Prot. Science 2001; 10:1454-1465.

  • Kaul R, Angeles AR, Jger M, Powers ET, Kelly JW. Incorporating β-turns and a turn mimetic out of context in loop 1 of the WW domain affords cooperatively folded β-sheets. J. Am. Chem. Soc. 2001; 123:5206-5212.

  • Purkey HE, Dorrell MI, Kelly JW. Evaluating the binding selectivity of transthyretin amyloid fibril inhibitors in blood plasma. Proc. Natl. Acad. Sci. USA 2001; 98:5566-5571.

  • Ratnaswamy G, Huff ME, Su AI, Rion S, Kelly JW. Destabilization of Ca2+-free gelsolin may not be responsible for proteolysis in Familial Amyloidosis of Finnish Type. Proc. Natl. Acad. Sci. USA 2001; 98:2334-2339.

  • Petrassi HM, Sharpless KB, Kelly JW. The copper-mediated cross-coupling of phenylboronic acids and N-hydroxyphthalimide at room temperature: synthesis of aryloxyamines. Org. Lett. 2001; 3:139-142.

  • Powers ET, Kelly JW. Medium-dependent self-assembly of an amphiphilic peptide: direct observation of peptide phase domains at the air-water interface. J. Am. Chem. Soc. 2001; 123:775-776.

2000


  • Liu K, Cho HS, Hoyt DW, Nguyen TN, Olds P, Kelly JW, Wemmer DE. Deuterium-proton exchange on the native wild-type transthyretin tetramer identifies the stable core of the individual subunits and indicates mobility at the subunit interface. J. Mol. Biol. 2000; 303:555-565.

  • Raman P, Razavi H, Kelly JW. Titanium(IV)-mediated tandem deprotection-cyclodehydration of protected cysteine N-amides: biomimetic syntheses of thiazoline- and thiazole-containing heterocycles. Org. Lett. 2000; 2:3289-3292.

  • Liu K, Cho HS, Lashuel HA, Kelly JW, Wemmer DE. A glimpse of a possible amyloidogenic intermediate of transthyretin. Nat. Struc. Biol. 2000; 7:754-757.

  • Lashuel HA, LaBrenz SR, Woo L, Serpell LC, Kelly JW. Protofilaments, filaments, ribbons, and fibrils from peptidomimetic self-assembly: implications for amyloid fibril formation and materials science. J. Am. Chem. Soc. 2000; 122:5262-5277.

  • Crane JC, Koepf EK, Kelly JW, Gruebele M. Mapping the transition state of the WW domain β-sheet. J. Mol. Biol. 2000; 298:283-292.

  • Petrassi HM, Klabunde T, Sacchettini J, Kelly JW. Structure-based design of N-phenyl phenoxazine transthyretin amyloid fibril inhibitors. J. Am. Chem. Soc. 2000; 122:2178-2192.

  • Klabunde T, Petrassi HM, Oza VB, Raman P, Kelly JW, Sacchettini JC. Rational design of potent human transthyretin amyloid disease inhibitors. Nat. Struct. Biol. 2000; 7:312-321.

1999

  • Koepf EK, Petrassi HM, Ratnaswamy G, Huff ME, Sudol M, Kelly JW. Characterization of the structure and function of W --> F WW domain variants: identification of a natively unfolded protein that folds upon ligand binding. Biochemistry 1999; 38:14338-14351.

  • Lashuel HA, Wurth C, Woo L, Kelly JW. The most pathogenic transthyretin variant, L55P, forms amyloid fibrils under acidic conditions and protofilaments under physiological conditions. Biochemistry 1999; 38:13560-13573.

  • Koo EH, Lansbury PT, Kelly JW. Amyloid diseases: abnormal protein aggregation in neurodegeneration. Proc. Natl. Acad. Sci. USA 1999; 96:9989-9990.

  • Bekele H, Fendler JH, Kelly JW. Self-assembling peptidomimetic monolayer nucleates oriented CdS nanocrystals. J. Am. Chem. Soc. 1999; 121:7266-7267.

  • Oza VB, Petrassi HM, Purkey HE, Kelly JW. Synthesis and evaluation of anthranilic acid-based transthyretin amyloid fibril inhibitors. Bioorg. Med. Chem. Lett. 1999; 9:1-6.

  • Ratnaswamy G, Koepf E, Bekele H, Yin H, Kelly JW. The amyloidogenicity of gelsolin is controlled by proteolysis and pH. Chemistry & Biology 1999; 6:293-304.

  • Baures PW, Oza VB, Peterson SA, Kelly JW. Synthesis and evaluation of inhibitors of transthyretin amyloid formation based on the non-steroidal anti-inflammatory drug, flufenamic acid. Bioorg. Med. Chem. 1999; 7:1339-1347.

  • Chitnumsub P, Fiori WR, Lashuel HA, Diaz H, Kelly JW. The nucleation of monomeric parallel β-sheet-like structures and their self-assembly in aqueous solution. Bioorg. Med. Chem. 1999; 7:39-59.

  • Koepf EK, Petrassi HM, Sudol M, Kelly JW. WW: an isolated three-stranded antiparallel β-sheet domain that unfolds and refolds reversibly; evidence for a structured hydrophobic cluster in urea and GdnHCl and a disordered thermal unfolded state. Prot. Science 1999; 8:841-853.

1998

  • Lashuel HA, Lai Z, Kelly JW. Characterization of the transthyretin acid denaturation pathways by analytical ultracentrifugation: implications for wild-type, V30M, and L55P amyloid fibril formation. Biochemistry 1998; 37:17851-17864.

  • Kelly JW. Overview of the biosynthesis of amino acids, peptides, porphyrins and alkaloids with a focus on the biosynthesis of aromatic amino acids. In Comprehensive Natural Products Chemistry, Vol 4, Amino Acids, Peptides, Porphyrins and Alkaloids. Kelly, J.W. (Ed.), Elsevier Science Ltd, Oxford, 1998; 1-11.

  • Peterson SA, Klabunde T, Lashuel HA, Purkey H, Sacchettini JC, Kelly JW. Inhibiting transthyretin conformational changes that lead to amyloid fibril formation. Proc. Natl. Acad. Sci USA 1998; 95:12956-12960.

  • Nettleton EJ, Sunde M, Lai Z, Kelly JW, Dobson CM, Robinson CV. Protein subunit interactions and structural integrity of amyloidogenic transthyretins: evidence from electrospray mass spectrometry. J. Mol. Biol. 1998; 281:553-564.

  • Xie Y, Lashuel HA, Miroy GJ, Dikler S, Kelly JW. Recombinant human retinol-binding protein refolding, native disulfide formation, and characterization. Prot. Exp. Purif. 1998; 14:31-37.

  • LaBrenz SR, Bekele H, Kelly JW. Enhancement of the water solubility of aromatic molecules via the Heck reaction. A comparison of ethano-ammonium, -carboxylate, and -phosphonate functional groups. Tetrahedron 1998; 54:8671-8678.

  • Patricelli MP, Lashuel HA, Giang DK, Kelly JW, Cravatt BF. Comparative characterization of a wild type and transmembrane domain-deleted fatty acid amide hydrolase: identification of the transmembrane domain as a site for oligomerization. Biochemistry 1998; 37:15177-15187.

  • Baures PW, Peterson SA, Kelly JW. Discovering transthyretin amyloid fibril inhibitors by limited screening. Bioorg. Med. Chem. 1998; 6:1389-1401.

  • Kelly JW. The alternative conformations of amyloidogenic proteins and their multi-step assembly pathways. Curr. Opin. Struct. Biol. 1998; 8:101-106.

  • Kelly JW. The environmental dependency of protein folding best explains prion and amyloid disease. Proc. Natl. Acad. Sci. USA 1998; 95:930-932.

1997

  • Dikler S, Kelly JW, Russell DH. Improving mass spectrometric sequencing of arginine-containing peptides by derivatization with acetylacetone. J. Mass. Spectrom. 1997; 32:1337-1349.

  • Kelly JW. Amyloid fibril formation and protein misassembly: a structural quest for insights into amyloid and prion diseases. Structure 1997; 5:595-600.

  • Lai Z, McCulloch J, Lashuel HA, Kelly JW. Guanidine hydrochloride-induced denaturation and refolding of transthyretin exhibits a marked hysteresis: equilibria with high kinetic barriers. Biochemistry 1997; 36:10230-10239.

  • Colon W, Lai V, Lashuel H, McVulloch J, McCutchen S, Miroy G, Peterson S, Kelly JW. Transthyretin quaternary and tertiary structural changes facilitate misassembly into amyloid. Adv. Prot. Chem. 1997; 50:161-181.

  • Bekele H, Nesloney CL, McWilliams KW, Zacharias NM, Chitnumsub P, Kelly JW. Improved synthesis of the Boc and Fmoc derivatives of 4-(2'-aminoethyl)-6-dibenzofuranpropionic acid: an unnatural amino acid that nucleates β-sheet folding. J. Org. Chem. 1997; 62:2259-2262.

  • Chen HI, Einbond A, Kwak S-J, Linn H, Koepf E, Peterson S, Kelly JW, Sudol M. Characterization of the WW domain of human Yes-associated protein and its polyproline-containing ligands. J. Biol. Chem. 1997; 272:17070-17077.

1996

  • Zhang W, Diaz H, Kelly JW, Taylor JW. Examining the structural and functiona roles of the β-turn region in neuropeptide Y. Lett. Pep. Sci. 1996; 3:133-142.

  • Miroy GJ, Lai Z, Lashuel HA, Peterson SA, Strang C, Kelly JW. Inhibiting transthyretin amyloid fibril formation via protein stabilization. Proc. Natl. Acad. Sci. USA 1996; 93:15051-15056.

  • McWilliams K, Kelly JW. Synthesis and conformational preferences of a potential β-sheet nucleator based on the 9,9-dimethylxanthene skeleton. J. Org. Chem. 1996; 61:7408-7414.

  • Colon W, Lai Z, McCutchen SL, Miroy GJ, Strang CJ, Kelly JW. FAP mutations destabilize transthyretin facilitating conformational changes required for amyloid formation. In The Nature and Origin of Amyloid Fibrils (CIBA Foundation Symposium 199) Wiley, Chichester, 1996; 228-242.

  • Nesloney CL, Kelly JW. Progress towards understanding β-sheet structure. Bioorg. Med. Chem. 1996; 4:739-766.

  • Nesloney CN, Kelly JW. A 2,3'-substituted biphenyl-based amino acid facilitates the formation of a monomeric β-hairpin-like structure in aqueous solution at elevated temperature. J. Am. Chem. Soc. 1996; 118:5836-5845.

  • Nesloney CL, Kelly JW. Synthesis and hydrogen bonding capabilities of biphenyl-based amino acids designed to nucleate β-sheet structure. J. Org. Chem. 1996; 61:3127-3137.

  • Lai Z, Colon W, Kelly JW. The acid-mediated denaturation pathway of transthyretin yields a conformational intermediate that can self-assemble into amyloid. Biochemistry 1996; 35:6470-6482.

  • Kelly JW. Alternative conformations of amyloidogenic proteins govern their behavior. Curr. Opin. Struct. Biol. 1996; 6:11-17.

  • Choo DW, Schneider JP, Graciani NR, Kelly JW. A nucleated antiparallel β-sheet that folds and undergoes self-assembly: a template promoted folding strategy toward controlled molecular architectures. Macromolecules 1996; 29:355-366.

1995

  • McCutchen SL, Lai Z, Miroy G, Kelly JW, Colon W. Comparison of lethal and nonlethal transthyretin variants and their relationship to amyloid disease. Biochemistry 1995; 34:13527-13536.

  • Schneider JP, Kelly JW. Templates that induce α-helical, β-sheet and loop conformations. Chem. Rev. 1995; 95:2169-2187.

  • Schneider JP, Kelly JW. Synthesis and efficacy of square planar copper complexes designed to nucleate β-sheet structure. J. Am. Chem. Soc. 1995; 117:2533-2546.

  • Graciani NR, Swanson DS, Kelly JW. Phosphomonoesters and phosphodiesters derived from the photohydrolysis of 2-methoxy-5-nitrophenyl substituted phosphotriesters. Tetrahedron 1995; 51:1077-1086.

  • LaBrenz SR, Kelly JW. Peptidomimetic host that binds a peptide guest affording a β-sheet structure that subsequently self-assembles. A simple receptor mimic. J. Am. Chem. Soc. 1995; 117:1655-1656.

1994

  • Graciani NR, Tsang KY, McCutchen SL, Kelly JW. Amino acids that specify secondary structure through hydrophobic clustering and histidine-aromatic interactions lead to biologically active peptidomimetics. Bioorg. Med. Chem. 1994; 2:999-1006.

  • Kelly JW, Lansbury PT. A chemical approach to elucidate the mechanism of transthyretin and β-protein amyloid fibril formation. Amyloid 1994; 1:186-205.

  • Tsang K-Y, Diaz H, Graciani N, Kelly JW. Hydrophobic cluster formation is necessary for dibenzofuran-based amino acids to function as β-sheet nucleators. J. Am. Chem. Soc. 1994; 116:3988-4005.

1993

  • McCutchen SL, Kelly JW. Intermolecular disulfide linkages are not required for transthyretin amyloid fibril formation in vitroBiochem. Biophys. Res. Comm. 1993; 197:415-421.

  • McCutchen SL, Colon W, Kelly JW. The transthyretin mutation Leu-55-Pro significantly alters tetramer stability and Amyloidogenicity. Biochemistry 1993; 32:12119-12127.

  • Choo D, Diaz H, Tsang KY, Kelly JW. The design of water soluble β-sheet structure based on a nucleation strategy. Tetrahedron 1993; 49:3533-3545.

  • Diaz H, Tsang KY, Choo D, Kelly JW. The design, synthesis and partial characterization of small water soluble β-sheets mediated by a dibenzofuran based template. J. Am. Chem. Soc. 1993; 115:3790-3791.

1992

  • Diaz H, Espina JR, Kelly JW. A dibenzofuran based amino acid designed to nucleate antiparallel β-sheet structure: evidence for intramolecular hydrogen bond formation. J. Am. Chem. Soc. 1992;114:8316-8318.

  • Colon W, Kelly JW. Partial denaturation of transthyretin is sufficient for amyloid fibril formation in vitroBiochemistry 1992; 31:8654-8660.

  • Banzon JA, Kelly JW. β-Sheet rearrangements, serpins and beyond. Prot. Engineering 1992; 5:113-115.

  • Schneider JP, Topgi RS, Kelly JW. A convenient synthesis of 6,6'-diamino-2,2'-bipyridine. Synthetic Comm. 1992; 22:1033-1037.

  • Kelly JW, Robinson PL, Murray WT, Eskew NA, Pautard Cooper A, Mathieu P, Evans SA. New synthetic methodologies employing substituted 1,3,2,5-dioxaphospholanes. ACS Symposium Series 1992; 486:186-201.

1991

  • Diaz H, Kelly JW. The synthesis of dibenzofuran based diacids and amino acids designed to nucleate parallel and anti-parallel β-sheet formation. Tetrahedron Lett. 1991; 32:5725-5728.

  • Colon W, Kelly JW. Transthyretin acid induced denaturation is sufficient for amyloid fibril formation in vitro. In "Applications of Enzyme Biotechnology" Kelly JW & Balwin TO (Eds). Plenum, New York, 1991, pp 99-108

1990

No publication available.

1989

  • Kaiser ET, Mihara H, Laforet GA, Kelly JW, Walters L, Findeis MA, Sasaki T. Peptide and protein synthesis by segment synthesis-condensation. Science 1989; 243:187-192.

1988

  • Kelly JW. New tools for probing protein structure. Bio/Technology 1988; 6:125-129.

  • Kelly JW, Walters L, Kaiser ET. The oxime based segment synthesis of Cro in "The Analysis of Peptides and Proteins by Mass Spectrometry", C.J. McNeal (editor). Wiley, 1988:15-19.

  • Ruhlmann K, Scheim U, Evans SA, Kelly JW, Bassindale AR. Synthesis of siloxanes: oxygen-17 NMR spectroscopy. J. Organometallic Chem. 1988; 340:19-25.

1987

  • Kelly JW, Evans SA. Oxygen-17 NMR spectral studies of selected aromatic sulfones. Magn. Res. Chem. 1987; 25:305-308.

  • Kelly JW, Robinson PL, Evans SA. Dioxyphosphoranes: an emerging class of versatile reagents for organic synthesis. Phosphorus and Sulfur 1987; 30:147-150.

  • Murray WT, Kelly JW, Evans SA. Synthesis of substituted 1,4-oxathianes mechanistic details of diethoxytriphenylphosphorane- and triphenylphosphine/tetrachloromethane-promoted cyclodehydrations and 13C NMR spectroscopy. J. Org. Chem. 1987; 52:525-529.

  • Robinson PL, Kelly JW, Evans SA. The chemistry of α,Ω-mercaptoalcohols in the presence of diethoxytriphenylphosphorane: temperature dependence of cyclodehydrations and S-ethylations. Phosphorus and Sulfur 1987; 31:59-70.

1986

  • Kelly JW, Eskew NL, Evans SA. Cyclohydration of N- and C-substituted α-amino alcohols to the corresponding aziridines with diethoxytriphenylphosphorane. J. Org. Chem. 1986; 51:95-97.

  • Kelly JW, Evans SA. Bis(neopentyloxy)triphenylphosphorane: a versatile, nonalkylating cyclodehydration reagent. J. Org. Chem. 1986; 51:5490-5492.

  • Kelly JW, Evans SA. Regioselective phosphoranylation and cyclodehydration of triols with diethoxytriphenylphosphorane. J. Am. Chem. Soc. 1986; 108:7681-7685.

  • Kelly JW, Robinson PL, Evans SA. 2,2,2-Triphenyl-4,5-(2',2''-biphenylene)-1,3,2-dioxaphosphane: an effective reagent for converting diols, amino alcohols and especially mercapto alcohol to the corresponding heterocycles. J. Org. Chem. 1986; 51:4473-4475.

  • Robinson PL, Kelly JW, Evans SA. The synthetic utility of dioxyphosphoranes in organic synthesis. Phosphorus and Sulfur 1986; 26:15-24.

1985

  • Robinson PL, Barry CN, Kelly JW, Evans SA. Diethoxytriphenylphosphorane: a mild regioselective cyclodehydrating reagent for conversion of diols to cyclic ethers. Stereochemistry, synthetic utility and scope. J. Am. Chem. Soc. 1985; 107:5210-5219.

  • Kelly JW, Robinson PL, Evans SA. Diethoxydiphenylpolystyrylphosphorane: a new polymeric reagent for the efficient cyclodehydration of simple diols. J. Org. Chem. 1985; 50:5007-5009.