David J. Pagliarini, PhD

PUBLICATIONS (*co-lead author; †co-senior author; # denotes publications on which I am a lead or corresponding author)

2026

102.

Dynamic protein phosphorylation shapes mitochondrial catalysis, import, and architecture

Smith AJ, Forny P, Rogers SW, Forny M, and Pagliarini DJ

bioRxiv, 2026 doi.org/10.64898/2026.09.28.755151

101.

The predicted interactome of the human mitochondrial proteome

Swaminathan AB, Zulkifli M, Guerra RM, Calabrese SM, Kalafatis DT,

Pagliarini DJ, and Gohil VM

Nature Communications, 2026 Aug 25;17(1):10162

doi: 10.1038/s41467-026-77112-z Epub 2026 Aug 25 PMID: 42778582 PMCID: PMC13601606

100.

Mitochondria with (distinct) personality

Lee WD, Pagliarini DJ, Chen Z, Trifunovic A, Chen Q, Santos JH, Filipovska A, Gammage PA

Molecular Cell, 2026 Aug 6;86(15):2918-2923

doi: 10.1016/j.molcel.2026.06.044 Epub 2026 Aug 6 PMID: 42561926

99.

Fmp30p is a mitochondrial phosphatidylinositol hydrolase that modulates CoQ biosynthesis

Baker ZN, Guerra RM, Rogers S, Pagliarini DJ

bioRxiv, 2025.05.01.651778; doi: https://doi.org/10.1101/2025.05.01.651778

Nature Communications, 2026 May 30;17(1):7013

doi: 10.1038/s41467-026-73766-x Epub 2026 May 30 PMID: 42218121 PMCID: PMC13392021

98.

BRD4-mediated ER membrane contact creates functionally distinct mitochondrial subtypes

Chen B, Stark DC, Jadhav PV, Lynn-Nguyen TM, Halligan BS, Rossiter NJ, Sindoni N, Shin M, Paulo JA, Chang M, Koo I, Koshkin S, Eyunni S, Ronchi P, Paulsen MT, Greenbaum HS, Ruckert MT, Morlacchi P, Hanna DA, Lin J, Guerra RM, Liu T, Pagliarini DJ, Banerjee R, Parolia A, Ljungman ME, Patterson AD, Mancias JD, Mosalaganti S, Sexton JZ, Cali T, Lyssiotis CA, and Shah YM

bioRxiv, 2024 doi.org/10.1101/2024.02.02.578646

Molecular Cell, 2026 Mar 5;86(5):917-936.e12

doi: 10.1016/j.molcel.2026.01.012 Epub 2026 Feb 13 PMID: 41690300 PMCID: PMC13147340

97.

Cooperative architecture of mitochondrial proteome homeostasis

Forny P, Forny M, Smith AJ, Sung AY, Liu K, and Pagliarini DJ

medRxiv, 2026 doi.org/10.64898/2026.02.06.26345691

Manuscript Under Revision (Cell)

2025

96.

Targeting TOMM40 and TOMM22 to Rescue Statin-Impaired Mitochondrial Function, Dynamics, and Mitophagy in Skeletal Myotubes

Yang NV, Rogers S, Guerra R, Chao JY, Pagliarini DJ, Theusch, E, and Krauss RM

bioRxiv, 2023 doi: 10.1101/2023.06.24.546411

Int J Mol Sci. 2025 Nov 13;26(22):10977

doi: 10.3390/ijms262210977 Epub 2025 Nov 13 PMID: 41303460 PMCID: PMC12652557

95.

Uridine-sensitized screening identifies demethoxy-coenzyme Q and NUDT5 as regulators of nucleotide synthesis

Strefeler A, Baker ZN, Chollet S, Foged MM, Guerra RM, Ivanisevic J, Gallart-Ayala H, Pagliarini DJ, Jourdain AA

bioRxiv 2025 doi:10.1101/2025.03

Nat Metabolism, 2025 Nov;7(11):2221-2235

doi: 10.1038/s42255-025-01419-2 Epub 2025 Nov 13 PMID: 41233602 PMCID: PMC12638251

94.

Severe Neurological Presentation in Siblings with COQ5-related Primary Coenzyme Q10 deficiency:

Expanding Clinical and Molecular Spectrum

Wongkittichote P, Guerra RM, Wegner DJ, Toy S, Hauer, JA, Pagliarini DJ, and Granadillo JL

JIMD Rep., 2025 Nov 5;66(6):e70038

doi: 10.1002/jmd2.70038 Epub 2025 Nov 5 PMID: 41199775 PMCID: PMC12588166

93.

ACAD10 encodes two orphan enzymes in the ether lipid biosynthetic and salvage pathways

Ye JS, Purlyte E, Lopez VA, Mill J, Tatge L, Kizzar T, Baldwin DA, Rashan EH, Kim J, Pagliarini DJ, Tomchick DR, Pawłowski K, Douglas PM, Simcox JA, and Tagliabracci VS

bioRxiv, 2025 doi.org/10.1101/2025.09.10.675409

Manuscript Under Revision (Molecular Cell)

92.

RTN4IP1 is essential for the final stages of mitochondrial complex I assembly and coenzyme Q biosynthesis

Olahova M, Guerra RM, Collier JJ, Heidler J, Thompson K, White CR, Castaneda-Tamez P, Cabrera-Orefice A, Lightowlers RN, Chrzanowska-Lightowlers ZMA, Galkin A, Wittig I, Pagliarini DJ and Taylor RW

bioRxiv, 2024 doi.org/10.1101/2024.09.04.610987

EMBO J,. 2025 Oct;44(19):5482-5508

doi: 10.1038/s44318-025-00533-x Epub 2025 Aug 26 PMID: 40859035 PMCID: PMC12489013

91.

Mitochondrial dysfunction and lipid dysregulation in yeast lacking phosphatidylserine

Joshi A, Baker ZN, Stanfield RA, Kalafatis DT, Pagliarini DJ, and Gohil VM

Molecular Biology of the Cell, 2025 Oct 1;36(10):ar121

doi: 10.1091/mbc.E25-03-0128 Epub 2025 Aug 13 PMID: 40802260 PMCID: PMC12444905

90.

ACAD10 and ACAD11 enable mammalian 4-hydroxy acid lipid catabolism

Rashan EH, Bartlett AK, Khana DB, Zhang J, Jain R, Wade G, Abriata LA, Smith AJ, Baker ZN, Cook T, Caldwell A, Chevalier AR, Forny P, Pfleger BF, Dal Peraro M, Yuan P, Amador-Noguez D, Simcox JA, and Pagliarini DJ

bioRxiv, 2024 doi: https://doi.org/10.1101/2024.01.09.574893

Nature Structural and Molecular Biology, 2025 Sep;32(9):1622-1632

doi: 10.1038/s41594-025-01596-4 Epub 2025 Jun 19 PMID: 40537578 PMCID: PMC12440821

89.

Multiomics reveals that triacylglycerol mobilization helps drive recovery from mitochondrial stress

Baker ZN and Pagliarini DJ

Research Briefing (related to publication 88)

Nature Cell Biology, 2025 https://doi.org/10.1038/s41556- 024-01586-6

88.

Triacylglycerol mobilization underpins mitochondrial stress recovery

Baker ZN, Zhu Y, Guerra RM, Smith AJ, Arra A, Serrano LR, Mukherji S, Craig EA, Coon JJ and Pagliarini DJ

Nature Cell Biology, 2025 Feb;27(2):298-308

doi: 10.1038/s41556-024-01586-6 Epub 2025 Jan 8 PMID: 39779944 PMCID: PMC11821527

2024

87.

Identification of a new COQ4 spliceogenic variant causing severe primary coenzyme Q deficiency

Alcázar-Fabra M, Østergaard E, Fernández-Ayala DJM, Desbats MA, Morbidoni V, Tomás-Gallado L, García-Corzo L, Blanquer-Roselló MM, Bartlett AK, Sánchez-Cuesta A, Sena L, Cortés-Rodríguez A, Cascajo-Almenara MV, Pagliarini DJ, Trevisson E, Gronborg SW, and Brea-Calvo G

Molecular Genetics and Metabolism Reports, 2025 March; 42, 101176

doi: 10.1016/j.ymgmr.2024.101176 Epub 2024 Dec 14 PMID: 39759098 PMCID: PMC11699292

86.

DFCP1 is a regulator of starvation-driven ATGL-mediated lipid droplet lipolysis

Ismail VA, Schuetz M, Baker ZN, Castillo-Badillo JA, Naismith TV, Pagliarini DJ, Kast DJ

Journal of Lipid Research, 2025 January; 66(1), 100700

doi: 10.1016/j.jlr.2024.100700 Epub 2024 Nov 19 PMID: 39566849 PMCID: PMC11721518

85.

Missense variants in CMS22 patients reveal that PREPL has both enzymatic and non-enzymatic functions

Monnens Y, Theodoropoulou A, Rosier K, Bhalla K, Mahy A, Vanhoutte R, Meulemans S, Cavani E, Antanasijevic A, Lemmens I, Lee JA, Spellicy CJ, Schroer RJ, Maselli RA, Laverty CG, Agostinis P, Pagliarini DJ, Verhelst S, Marcaida MJ, Rochtus A, Dal Peraro M, and Creemers JWM

bioRxiv, 2023 doi.org/10.1101/2023.12.18.572145

JCI Insight, 2024 Jul 30:e179276.

doi: 10.1172/jci.insight.179276 Epub 2024 Sep 10 PMID: 39078710 PMCID: PMC11385081

84.

Fast and Deep Phosphoproteome Analysis with the Orbitrap Astral Mass Spectrometer

Lancaster NM, Sinitcyn P, Forny P, Peters-Clarke TM, Fecher C, Smith AJ, Shishkova E, Arrey TN, Pashkova A, Robinson ML, Arp N, Fan J, Hansen J, Galmozzi A, Serrano LR, Rojas J, Gasch AP, Westphall MS, Stewart H, Hock C, Damoc E, Pagliarini DJ, Zabrouskov V, and Coon JJ

bioRxiv, 2023 doi.org/10.1101/2023.11.21.568149

Nature Communications, 2024 Aug 15;15(1):7016.

doi: 10.1038/s41467-024-51274-0 Epub 2024 Aug 15 PMID: 39147754 PMCID: PMC11327265

83.

A systematic mutational framework for studying oxidative phosphorylation-related proteins

Sung AY and Pagliarini DJ

Research Briefing (related to publication 82)

Nature Metabolism, 2024 Jun;6(6):998–999

82.

Systematic analysis of NDUFAF6 in complex I assembly and mitochondrial disease

Sung AY, Guerra RM, Steenberge LH, Alston CL, Murayama K, Okazaki Y, Shimura M, Prokisch H, Ghezzi D, Torraco A, Carrozzo R, Rötig A, Taylor RW, Keck JL, and Pagliarini DJ

In Review preprint, 2023 doi.org/10.21203/rs.3.rs-3332602/v1

Nature Metabolism, 2024 Jun;6(6):1128-1142

doi: 10.1038/s42255-024-01039-2 Epub 2024 May 8 PMID: 38720117

81.

Coenzyme Q4 is a functional substitute for coenzyme Q10 and can be targeted to the mitochondria

Steenberge LH, Sung AY, Fan J, and Pagliarini DJ

bioRxiv, 2023 doi.org/10.1101/2023.07.20.549963

Journal of Biological Chemistry, 2024 Apr 6:107269.

doi: 10.1016/j.jbc.2024.107269 Epub 2024 Apr 6 PMID: 38588811 PMCID: PMC11087978

80.

Reducing mitochondrial mysteries

Guerra RM, and Pagliarini DJ

Nature Chemical Biology, 2024 Feb;20(2):132-133

doi: 10.1038/s41589-023-01435-x Epub 2024 Feb PMID: 37884804 PMCID: PMC11378938

79.

COQ4 is required for the oxidative decarboxylation of the C1 carbon of Coenzyme Q in eukaryotic cells

Pelosi P, Morbiato L, Burgardt A, Tonello F, Bartlett AK, Guerra RM, Ferizhendi KK, Desbats MA, Rascalou B, Marchi M, Vazquez-Fonseca L, Agosto C, Zanotti G, Roger-Margueritat M, Alcázar-Fabra M, García-Corzo L, Sánchez-Cuesta A, Navas P, Brea-Calvo G, Trevisson E, Wendisch VF, Pagliarini DJ, Salviati L, and Pierrel F

bioRxiv, 2023 https://doi.org/10.1101/2023.11.13.566839

Molecular Cell, 2024 Mar 7;84(5):981-989.e7.

doi: 10.1016/j.molcel.2024.01.003 Epub 2024 Jan 30 PMID: 38295803 PMCID: PMC7616408

2023

78.

Pptc7 maintains mitochondrial protein content by suppressing receptor-mediated mitophagy

Niemi NM, Serrano LR, Muehlbauer LK, Balnis CE, Wei L, Smith AJ, Kozul KL, Forny M, Connor OM, Rashan EH, Shishkova E, Schueler KL, Keller MP,

Attie AD, Friedman JR, Pagan JK, Coon JJ, and Pagliarini DJ

bioRxiv, 2023 doi.org/10.1101/2023.02.28.530351

Nature Communications, 2023 doi: 10.1038/s41467-023-42069-w.

doi: 10.1038/s41467-023-42069-w Epub 2023 Oct 13 PMID: 37833277 PMCID: PMC10575892

77.

Hem25p is required for mitochondrial IPP transport in fungi

Tai JT, Guerra RM, Rogers SW, Fang Z, Muehlbauer LK, Shishkova E, Overmyer KA, Coon JJ, and Pagliarini DJ

bioRxiv, 2023 doi.org/10.1101/2023.03.14.532620

Nature Cell Biology, 2023 Nov;25(11):1616-1624

doi: 10.1038/s41556-023-01250-5 Epub 2023 Oct 9 PMID: 37813972 PMCID: PMC10759932

76.

Mitochondrial proteome research: the road ahead

Baker ZN, Forny P, and Pagliarini DJ

Nature Reviews Molecular Cell Biology, 2024 Jan;25(1):65-82

doi: 10.1038/s41580-023-00650-7 Epub 2023 Sep 29 PMID: 37773518 PMCID: PMC11378943

75.

Aim18p and Aim46p are CHI-domain-containing mitochondrial hemoproteins in Saccharomyces cerevisiae

Schmitz JM, Wolters JF, Murray NH, Guerra RM, Saunders JM, Jochem J, Smith RW, Bingman CA, Hittinger CT and Pagliarini DJ

bioRxiv, 2022 doi.org/10.1101/2022.11.15.516536

Journal of Biological Chemistry, 2023 Mar;299(3):102981

doi: 10.1016/j.jbc.2023.102981 Epub 2023 Feb 4 PMID: 36739946 PMCID: PMC9996372

74.

Coenzyme Q biochemistry and biosynthesis

Guerra RM, and Pagliarini DJ

Trends in Biochemical Sciences, 2023 May;48(5):463-476

doi: 10.1016/j.tibs.2022.12.006 Epub 2023 Jan 24 PMID: 36702698 PMCID: PMC10106368

2022

73.

Small Molecule Inhibition of the Archetypal UbiB protein COQ8

Murray NH, Asquith CRM, Fang Z, East MP, Ptak N, Smith RW, Vasta JD, Zimprich CA, Corona CR, Robers MB, Johnson GL, Bingman CA, and Pagliarini DJ

bioRxiv, 2022 doi.org/10.1101/2022.03.22.485346

Nature Chemical Biology, 2023 Feb;19(2):230-238

doi: 10.1038/s41589-022-01168-3 Epub 2022 Oct 27 PMID: 36302899 PMCID: PMC9898131

72.

Structure and functionality of a multimeric human COQ7:COQ9 complex

Manicki M*, Aydin H*, Abriata LA, Overmyer KA, Guerra RM, Coon JJ, Dal Peraro M, Frost A^† and

Pagliarini DJ^†

bioRxiv, 2021 doi.org/10.1101/2021.11.15.468694

Molecular Cell, 2022 82(22):4307-4323.e10

doi: 10.1016/j.molcel.2022.10.003 Epub 2022 Oct 27 PMID: 36306796 PMCID: PMC10058641

71.

Nitric oxide-driven modifications of lipoic arm inhibit α-ketoacid dehydrogenases

Seim GL, John SV, Arp NL, Fang Z, Pagliarini DJ, and Fan J

bioRxiv, 2022 doi.org/10.1101/2022.01.31.478543

Nature Chemical Biology, 2023 Mar;19(3):265-274

doi: 10.1038/s41589-022-01153-w Epub 2022 Oct 20 PMID: 36266351 PMCID: PMC9974485

70.

2-propylphenol Allosterically Modulates COQ8A to Enhance ATPase Activity

Murray NH*, Lewis A*, Rincon Pabon JP, Gross ML, Henzler-Wildman K and Pagliarini DJ

ACS Chemical Biology, 2022 17(8):2031-2038

doi: 10.1021/acschembio.2c00434 Epub 2022 Jul 29 PMID: 35904798 PMCID: PMC9586199

69.

Defining mitochondrial protein functions through deep multiomic profiling

Rensvold JW*, Shishkova E*, Miller IJ, Sverchkov Y, Cetinkaya A, Pyle A, Manicki M, Brademan DR, Alanay Y, Raiman J, Jochem A, Peters SR, Linke V, Hutchins PD, Overmyer KA, Salome AZ, Hebert AS, Vincent CE, Kwiecien NW, Rush MJ, Westphall MS, Craven M, Akarsu NA, Taylor RW, Coon JJ^†, and Pagliarini DJ^†

Nature, 2022 Jun;606(7913):382-388

doi: 10.1038/s41586-022-04765-3 Epub 2022 May 25 PMID: 35614220 PMCID: PMC9310563

2021

68.

Prolyl Endopeptidase-Like is a (thio)esterase involved in mitochondrial respiratory chain function

Rosier K, McDevitt MT, Smet J, Floyd BJ, Verschoore M, Marcaida MJ, Bingman CA, Lemmens I, Dal Peraro M, Tavernier J, Cravatt BF, Gounko NV, Vints K, Monnens Y, Bhalla K, Aerts L, Rashan EH, Vanlander A, Van Coster R, Régal L, Pagliarini DJ^†, and Creemers JWM^†

iScience, 2021 Nov 14;24(12)

doi: 10.1016/j.isci.2021.103460 Epub 2021 Nov 14 PMID: 34888501 PMCID: PMC8634043

67.

UbiB proteins regulate cellular CoQ distribution in Saccharomyces cerevisiae

Kemmerer ZA, Robinson KP, Schmitz JM, Manicki M, Paulson BR, Jochem A, Hutchins PD, Coon JJ, and Pagliarini DJ

bioRxiv, 2020 doi.org/10.1101/2020.12.09.418202

Nature Communications, 2021 Aug 6;12(1):4769

doi: 10.1038/s41467-021-25084-7 Epub 2021 Aug 6 PMID: 34362905 PMCID: PMC8346625

66.

The extensive and functionally uncharacterized mitochondrial phosphoproteome

Niemi NM and Pagliarini DJ

Journal of Biological Chemistry, 2021 Jul;297(1):100880

doi: 10.1016/j.jbc.2021.100880 Epub 2021 Jun 16 PMID: 34144036 PMCID: PMC8267538

65.

Loss of C2orf69 defines a fatal auto-inflammatory syndrome in Humans and Zebrafish that evokes a Glycogen storage-associated mitochondriopathy

Wong HH, Seet SH, Maier M, Gurel A, Traspas RM, Lee C , Zhang S, Talim B, Loh AYT, Chia CY, Teoh TS, Sng D, Rensvold J, Unal S, Shishkova E, Cepni E, Nathan FM, Sirota FL, Liang C, Yarali N, Simsek-Kiper PO, Mitani T, Ceylaner S, Arman-Bilir O, Mbarek H, Gumruk F, Efthymiou S, Uğurlu Çimen D, Georgiadou D, Sotiropoulou K, Houlden H, Paul F, Pehlivan D, Lainé C, Chai G, Ali NA, Choo SC, Keng SS, Boisson B, Yılmaz E, Xue S, Coon JJ, Ly TTN, Gilani N, Hasbini D, Kayserili H, Zaki MS, Isfort RJ, Ordonez N, Tripolszki K, Bauer P, Rezaei N, Seyedpour S, Khotaei GT, Bascom CC, Maroofian R, Chaabouni M, Alsubhi A, Eyaid W, Işıkay S, Gleeson JG, Lupski JR, Casanova JL, Pagliarini DJ, Akarsu NA, Maurer-Stroh S, Cetinkaya A, Bertoli-Avella A, Mathuru AS, Ho L, Bard FA, Reversade B

American Journal of Human Genetics, 2021 Jul 1;108(7):1301-1317

doi: 10.1016/j.ajhg.2021.05.003 Epub 2021 May 25 PMID: 34038740 PMCID: PMC8322802

64.

Defining intermediates and redundancies in coenzyme Q precursor biosynthesis

Robinson KP, Jochem A, Johnson SE, Reddy TR, Russell JD, Coon JJ, and Pagliarini DJ

Journal of Biological Chemistry, 2021 Apr 14;296:100643

doi: 10.1016/j.jbc.2021.100643 Epub 2021 Apr 14 PMID: 33862086 PMCID: PMC8122105

63.

Multi-omic Single-Shot Technology for Integrated Proteome and Lipidome Analysis

He Y, Rashan EH, Linke V, Shishkova E, Hebert AS, Jochem A, Westphall MS, Pagliarini DJ, Overmyer KA, and Coon JJ

Analytical Chemistry, 2021 93(9):4217-4222

doi: 10.1021/acs.analchem.0c04764 Epub 2021 Feb 22 PMID: 33617230 PMCID: PMC8028036

2020

62.

Quantitative shotgun proteome analysis by direct infusion

Meyer JG, Niemi NM, Pagliarini DJ, and Coon JJ

Nature Methods, 2020 Dec;17(12):1222-1228

doi: 10.1038/s41592-020-00999-z Epub 2020 Nov 23 PMID: 33230323 PMCID: PMC8009190

61.

Argonaut: a Web Platform for Collaborative Multi-Omic Data Visualization and Exploration

Brademan DR, Miller IJ, Kwiecien NW, Pagliarini DJ, Westphall MS, Coon JJ, and Shishkova E

Patterns, 2020 Oct 9;1(7)

doi: 10.1016/j.patter.2020.100122 Epub 2020 Oct 9 PMID: 33154995 PMCID: PMC7641515

60.

Classification of T-cell activation via autofluorescence lifetime imaging

Walsh AJ, Mueller KP, Tweed K, Jones I, Walsh CM, Piscopo NJ, Niemi NM, Pagliarini DJ, Saha K and Skala MC

bioRxiv, 2019 doi.org/10.1101/536813

Nature Biomedical Engineering, 2021 Jan;5(1):77-88

doi: 10.1038/s41551-020-0592-z Epub 2020 Jul 27 PMID: 32719514 PMCID: PMC7854821

59.

Clinico-genetic, imaging, biomarker, and molecular delineation of COQ8A-ataxia: a multicenter study of 59 patients

Traschütz A, Schirinzi T, Laugwitz L, Murray NH, Bingman CA, Reich S, Kern J, Heinzmann A, Vasco G, Bertini E, Zanni G, Durr A, Magri S, Malandrini A, Taroni F, Baets J, de Jonghe P, de Ridder W, Bereau M, Demuth S, Ganos C, Basak AN, Hanagasi H, Kurul SH, Bender B, Schöls L, Grasshoff U, Klopstock T, Horvath R, van de Warrenburg B, Burglen L, Rougeot C, Ewenczyk C, Koenig M, Santorelli FM, Anheim M, Munhoz RP, Haack T, Distelmaier F, Pagliarini DJ, Puccio H, Synofzik M

Annals of Neurology, 2020 Aug;88(2):251-263

doi: 10.1002/ana.25751 Epub 2020 Jun 10 PMID: 32337771 PMCID: PMC7877690

58.

Systems biochemistry approaches to defining mitochondrial protein function

Sung AY, Floyd BJ, and Pagliarini DJ

Cell Metabolism, 2020 31(4):669-678

doi: 10.1016/j.cmet.2020.03.011 Epub 2020 Apr 7 PMID: 32268114 PMCID: PMC7176052

57.

Mass spectrometry proteomics reveals a function for mammalian Calcoco1 in mTOR regulated selective autophagy

Stefely JA, Zhang Y, Freiberger EC, Kwiecien NW, Thomas HE, Davis AM, Lowry ND, Vincent CE, Shishkova E, Clark NA, Medvedovic M, Coon JJ, Pagliarini DJ, and Mercer CA

Autophagy, 2020 Dec;16(12):2219-2237

doi: 10.1080/15548627.2020.1719746 Epub 2020 Feb 2 PMID: 31971854 PMCID: PMC7751563

2019

56.

Pathogenic biallelic mutations in NDUFAF8 cause Leigh syndrome with an isolated complex I deficiency

Alston CL*, Veling MT*, Heidler J, Taylor LS, Alaimo JT, Sung AY, He L, Hopton S, Broomfield A, Pavaine J, Diaz J, Leon E, Wolf P, McFarland R, Prokisch H, Wortmann SB, Bonnen PE, Wittig I, Pagliarini DJ^†, and Taylor RW^†

American Journal of Human Genetics, 2020 106(1):92-101

doi: 10.1016/j.ajhg.2019.12.001 Epub 2019 Dec 19 PMID: 31866046 PMCID: PMC7042492

55.

ADCK3/COQ8A: The choice target of the UbiB protein kinase-like family

Asquith CRM^†, Murray NH, and Pagliarini DJ^†

Nature Reviews Drug Discovery, 2019 Oct;18(11):815

doi: 10.1038/d41573-019-00158-w Epub 2019 Oct PMID: 31673128 PMCID: PMC7062282

54.

Pptc7 is an essential phosphatase for promoting mammalian mitochondrial metabolism and biogenesis

Niemi NM, Wilson GM, Overmyer KA, Vögtle FN, Lohman DC, Schueler KL, Attie AD, Myketin L, Meisinger C, Coon JJ, and Pagliarini DJ

bioRxiv, 2018 doi.org/10.1101/426247

Nature Communications, 2019 10: 3197

doi: 10.1038/s41467-019-11047-6 Epub 2019 Jul 19 PMID: 31324765 PMCID: PMC6642090

53.

Two-stage metabolic remodeling in macrophages in response to LPS and IFN-γ stimulation

Seim GL, Britt EC, John SV, Yeo FJ, Johnson AR, Eisenstein RS, Pagliarini DJ, and Fan J

Nature Metabolism, 2019 1:731–742

doi: 10.1038/s42255-019-0083-2 Epub 2019 Jul 12 PMID: 32259027 PMCID: PMC7108803

52.

Obesity-dependent Cdk1 Signaling Stimulates Mitochondrial Respiration at Complex I in Pancreatic β-cells

Gregg T, Sdao SM, Dhillon RS, Rensvold JW, Lewandowski SL, Pagliarini DJ, Denu JM, and Merrins MJ

Journal of Biological Chemistry, 2019 294(12):4656-4666

doi: 10.1074/jbc.RA118.006085 Epub 2019 Jan 30 PMID: 30700550 PMCID: PMC6433064

51.

Coenzyme Q biosynthetic proteins assemble in a substrate-dependent manner into domains at ER-mitochondria contact sites

Subramanian K, Jochem A, Le Vasseur M, Lewis S, Paulson BR, Reddy TR, Russell JD, Coon JJ, Pagliarini DJ, and Nunnari J

Journal of Cell Biology, 2019 218(4):1353-1369

doi: 10.1083/jcb.201808044 Epub 2019 Jan 23 PMID: 30674579 PMCID: PMC6446851

50.

An isoprene lipid binding protein promotes eukaryotic coenzyme Q biosynthesis

Lohman DC*, Aydin D*, Von Bank HC, Smith RW, Linke V, Weisenhorn EM, McDevitt MT, Hutchins PD, Wilkerson EM, Wancewicz B, Russell JD, Stefely MS, Beebe ET, Jochem A, Coon JJ, Bingman CA, Dal Peraro M^†, and Pagliarini DJ^†

Molecular Cell, 2019 73(4):763-774.e10

doi: 10.1016/j.molcel.2018.11.033 Epub 2019 Jan 17 PMID: 30661980 PMCID: PMC6386619

2018

49.

Identification and Quantification of Murine Mitochondrial Proteoforms Using an Integrated Top-Down and Intact-Mass Strategy

Schaffer LV, Rensvold JW, Shortreed MR, Cesnik AJ, Jochem A, Scalf M, Frey BL, Pagliarini DJ, and Smith LM

Journal of Proteome Research 2018 17(10):3526-3536

doi: 10.1021/acs.jproteome.8b00469 Epub 2018 Sep 18 PMID: 30180576 PMCID: PMC6201694

48.

Systems analyses reveal physiological roles and genetic regulators of liver lipid species

Jha P*, McDevitt MT*, Gupta R*, Quiros PM, Williams EG, Gariani K, Sleiman MB, Diserens L, Jochem A, Ulbrich A, Coon JJ, Auwerx J^†, and Pagliarini DJ^†

Cell Systems, 2018 6(6):722-733.e6

doi: 10.1016/j.cels.2018.05.016 Epub 2018 Jun 13 PMID: 29909277 PMCID: PMC6054463

47.

Genetic regulation of plasma lipid species and their association with metabolic phenotypes

Jha P*, McDevitt MT*, Halilbasic E, Williams EG, Quiros PM, Gariani K, Sleiman MB, Gupta R, Ulbrich A, Jochem A, Coon JJ, Trauner M, Pagliarini DJ^†, and Auwerx J^†

Cell Systems, 2018 6(6):709-721.e6

doi: 10.1016/j.cels.2018.05.009 Epub 2018 Jun 13 PMID: 29909275 PMCID: PMC6397773

46.

A path to the powerhouse: systems-to-structure approaches for studying mitochondrial proteins

Pagliarini DJ

Protein Science, 2018 27(8):1518-1525

doi: 10.1002/pro.3430 Epub 2018 May 18 PMID: 29675961 PMCID: PMC6153412

45.

DNA Polymerase θ Increases Mutational Rates in Mitochondrial DNA

Wisnovsky S, Sack T, Pagliarini DJ, Laposa RR, and Kelley SO

ACS Chemical Biology, 2018 13(4):900-908

doi: 10.1021/acschembio.8b00072 Epub 2018 Mar 15 PMID: 29509408 PMCID: PMC5914477

2017

44.

Multi-omics reveal specific targets of the RNA-binding protein Puf3p and its orchestration of mitochondrial biogenesis

Lapointe CP*, Stefely JA*, Jochem A, Hutchins PD, Wilson GM, Kwiecien NW, Coon JJ, Wickens MP^†, and Pagliarini DJ^†

bioRxiv, 2017 doi.org/10.1101/146985

Cell Systems, 2018 6(1):125-135

doi: 10.1016/j.cels.2017.11.012 Epub 2017 Dec 13 PMID: 29248374 PMCID: PMC5799006

43.

Multi-omic mitoprotease profiling defines a role for Oct1p in coenzyme Q production

Veling MT, Reidenbach AG, Freiberger EC, Kwiecien NW, Hutchins PD, Drahnak MJ, Jochem A, Ulbrich A, Rush MJP, Russell JD, Coon JJ, and Pagliarini DJ

bioRxiv, 2017 doi.org/10.1101/155044

Molecular Cell, 2017 68(5):970-977.e11

doi: 10.1016/j.molcel.2017.11.023 Epub 2017 Dec 7 PMID: 29220658 PMCID: PMC5730362

42.

Conserved lipid and small molecule modulation of COQ8 reveals regulation of the ancient kinase-like UbiB family

Reidenbach AG, Kemmerer ZA, Aydin D, Jochem A, McDevitt MT, Hutchins PD, Stark JL, Stefely JA, Reddy T, Hebert AS, Wilkerson EM, Johnson IE, Bingman CA, Markley JL, Coon JJ, Dal Peraro M, and Pagliarini DJ

bioRxiv, 2017 doi.org/10.1101/149823

Cell Chemical Biology, 2018 25(2):154-165.e11

doi: 10.1016/j.chembiol.2017.11.001 Epub 2017 Nov 30 PMID: 29198567 PMCID: PMC5819996

41.

Biochemistry of Mitochondrial Coenzyme Q Biosynthesis

Stefely JA and Pagliarini DJ

Trends in Biochemical Sciences, 2017 42(10):824-843

doi: 10.1016/j.tibs.2017.06.008 Epub 2017 Sep 17 PMID: 28927698 PMCID: PMC5731490

40.

Erythropoietin signaling regulates heme biosynthesis

Chung J, Wittig JG, Ghamari A, Maeda M, Dailey TA, Bergonia H, Coughlin EE, Minogue CE, Hebert AS, Li L, Kaplan J, Lodish HF, Bauer DE, Orkin SH, Cantor AB, Maeda T, Phillips JD, Coon JJ, Pagliarini DJ, Dailey HA, and Paw BH

eLife, 2017 May 29;6. pii: e24767

doi: 10.7554/eLife.24767 Epub 2017 May 29 PMID: 28553927 PMCID: PMC5478267

39.

Integrative proteomics and biochemical analyses define Ptc6p as the Saccharomyces cerevisiae pyruvate dehydrogenase phosphatase

Guo X, Niemi NM, Coon JJ, and Pagliarini DJ

Journal of Biological Chemistry, 2017 292(28): 11751-11759

doi: 10.1074/jbc.M117.787341 Epub 2017 May 24 PMID: 28539364 PMCID: PMC5512070

38.

Ptc7p dephosphorylates select mitochondrial proteins to enhance metabolic function

Guo X*, Niemi NM*, Hutchins PD, Condon SGF, Jochem A, Ulbrich A, Higbee AJ, Russell JD, Senes A, Coon JJ, and Pagliarini DJ

Cell Reports, 2017 18(2):307-313

doi: 10.1016/j.celrep.2016.12.049 Epub 2017 Jan 10 PMID: 28076776 PMCID: PMC5234840

2016

37.

Powerhouse Multi-Omics—The Y3K Project (summary related to publication #36)

Kwiecien N, Coon JJ, Stefely JA, Pagliarini DJ

Cell Systems, Cell Systems Call, Principles of Systems Biology—No. 10, 2016 3(4):395-403

36.

Mitochondrial protein functions elucidated by multi-omic mass spectrometry profiling

Stefely JA*, Kwiecien NW*, Freiberger E, Richards AL, Jochem A, Rush MJP, Ulbrich A, Robinson KP, Hutchins PD, Veling MT, Guo X, Kemmerer ZA, Connors KJ, Trujillo EA, Sokol J, Marx H, Westphall MS, Hebert AS, Pagliarini DJ^†, and Coon JJ^†

Nature Biotechnology, 2016 34(11):1191-1197

doi: 10.1038/nbt.3683 Epub 2016 Sep 26 PMID: 27669165 PMCID: PMC5101133

35.

Iron Deprivation Induces Transcriptional Regulation of Mitochondrial Biogenesis

Rensvold JW, Krautkramer KA, Dowell JA, Denu JM, and Pagliarini DJ

Journal of Biological Chemistry, 2016 291(40):20827-20837

doi: 10.1074/jbc.M116.727701 Epub 2016 Aug 5 PMID: 27497435 PMCID: PMC5076495

34.

Mitochondrial protein interaction mapping identifies regulators of respiratory chain function

Floyd BJ*, Wilkerson EM*, Veling MT*, Minogue CE*, Xia C, Beebe ET, Wrobel, RL, Cho H, Kremer LS, Alston CL, Gromek KA, Dolan BK, Ulbrich A, Stefely JA, Bohl SL, Werner KM, Jochem A, Westphall MS, Rensvold JW, Taylor RW, Prokisch H, Kim JP, Coon JJ, and Pagliarini DJ

Molecular Cell, 2016 63(4): 621-32

doi: 10.1016/j.molcel.2016.06.033 Epub 2016 Aug 4 PMID: 27499296 PMCID: PMC4992456

33.

Cerebellar Ataxia and Coenzyme Q Deficiency Through Loss of Unorthodox Kinase Activity

Stefely JA*, Licitra F*, Laredj L, Reidenbach AG, Kemmerer ZA, Grangeray A, Jaeg-Ehret T, Minogue CE, Ulbrich A, Hutchins PD, Wilkerson EM, Ruan Z, Aydin D, Hebert AS, Guo X, Freiberger EC, Reutenauer L, Jochem A, Chergova M, Johnson IE, Lohman DC, Rush MJP, Kwiecien NW, Singh PK, Schlagowski AI, Floyd BJ, Forsman U, Sindelar P, Westphall MS, Pierrel F, Zoll J, Dal Peraro M, Kannan N, Bingman CA, Coon JJ, Isope P, Puccio H^†, and Pagliarini DJ^†

Molecular Cell, 2016 63(4):608-20

doi: 10.1016/j.molcel.2016.06.030 Epub 2016 Aug 4 PMID: 27499294 PMCID: PMC5012427

32.

Transomics: Mitochondrial systems analyses get supercomplex

Pagliarini DJ

Cell Metabolism, 2016 24(1):13-4

doi: 10.1016/j.cmet.2016.06.018 Epub 2016 Jul 12 PMID: 27411007

31.

Mitochondrial Protein Hyperacetylation in the Failing Heart

Horton JL, Martin OJ, Lai L, Riley NM, Richards AL, Vega RB, Leone TC, Pagliarini DJ, Muoio DM, Bedi KC, Margulies KB, Coon JJ, and Kelly DP

JCI Insight, 2016 2(1) pii: e84897

doi: 10.1172/jci.insight.84897 Epub 2016 Feb 25 PMID: 26998524 PMCID: PMC4795836

2015

30.

A single kinase generates the majority of the secreted phosphoproteome

Tagliabracci VS*, Wiley SE*, Guo X, Kinch LN, Durrant E, Wen J, Xiao J, Cui J, Nguyen KB, Engel JL, Coon JJ, Grishin N, Pinna LA, Pagliarini DJ, and Dixon JE

Cell, 2015 161(7):1619-32

doi: 10.1016/j.cell.2015.05.028 Epub 2015 Jun 18 PMID: 26091039 PMCID: PMC4963185

29.

Maximal oxidative capacity during exercise is associated with skeletal muscle fuel selection and dynamic changes in mitochondrial protein acetylation

Overmyer KA, Evans CR, Qi NR, Minogue CE, Chermside-Scabbo CJ, Koch LG, Britton SL, Pagliarini DJ, Coon JJ, and Burant CF

Cell Metabolism, 2015 21(3):468-78

doi: 10.1016/j.cmet.2015.02.007 Epub 2015 Mar 3 PMID: 25738461 PMCID: PMC4350023

28.

Multiplexed quantification for data-independent acquisition

Minogue CE, Hebert AS, Rensvold JW, Westphall MS, Pagliarini DJ, and Coon JJ

Analytical Chemistry, 2015 87(5):2570-5

doi: 10.1021/ac503593d Epub 2015 Feb 11 PMID: 25621425 PMCID: PMC4472008

2014

27.

Mitochondrial ADCK3 employs an atypical protein kinase-like fold to enable coenzyme Q biosynthesis

Stefely JA*, Reidenbach AG*, Ulbrich A, Oruganty K, Floyd BJ, Jochem A, Saunders JM, Johnson IE, Minogue CE, Wrobel RL, Barber GE, Lee D, Li S, Kannan N, Coon JJ, Bingman CA, and Pagliarini DJ,

Molecular Cell, 2015 57(1): 83-94

doi: 10.1016/j.molcel.2014.11.002 Epub 2014 Dec 11 PMID: 25498144 PMCID: PMC4289473

26.

Mitochondrial COQ9 is a lipid binding protein that associates with COQ7 to enable coenzyme Q biosynthesis

Lohman DC*, Forouhar F*, Beebe ET, Stefely MS, Minogue CE, Ulbrich A, Stefely JA, Sukumar S, Luna-Sanchez M, Lew S, Seetharaman J, Xiao R, Wang H, Wrobel RL, Everett JK, Mitchell JC, Lopez LC, Coon JJ, Tong L^†, and Pagliarini DJ^†

Proceedings of the National Academy of Sciences, 2014 111(44):E4697-705 (Direct submission)

doi: 10.1073/pnas.1413128111 Epub 2014 Oct 22 PMID: 25339443 PMCID: PMC4226113

25.

A Gly-zipper motif mediates homo-dimerization of the transmembrane domain of the mitochondrial kinase ADCK3

Khadria AS, Mueller BK, Stefely JA, Tan CH, Pagliarini DJ^†, and Senes A^†

Journal of the American Chemical Society, 2014 136(40): 14068-77

doi: 10.1021/ja505017f Epub 2014 Sep 24 PMID: 25216398 PMCID: PMC4195374

24.

Intelligent data acquisition blends targeted and discovery methods

Bailey DJ, McDevitt MT, Westphall MS, Pagliarini DJ, and Coon JJ

Analytical Chemistry, 201, 13(4):2152-61

doi: 10.1021/pr401278j Epub 2014 Mar 18 PMID: 24611583 PMCID: PMC3983381

23.

Neutron-encoded mass signatures for quantitative top-down proteomics

Rhoads TW, Rose CM, Bailey DJ, Molden RC, Riley NM, Nestler AJ, Merrill AE, Smith LM, Hebert AS, Westphall MS, Pagliarini DJ, Garcia BA, and Coon JJ

Analytical Chemistry, 2014, 86(5):2314-9

doi: 10.1021/ac403579s Epub 2014 Feb 19 PMID: 24475910 PMCID: PMC3983007

22.

Mitochondrial DNA variant in COX1 subunit significantly alters energy metabolism of geographically divergent wild isolates in Caenorhabditis elegans

Dingley SD, Polyak E, Ostrovsky J, Srinivasan S, Lee I, Rosenfeld A, Tsukikawa M, Xiao R, Selak M, Coon JJ, Hebert AS, Grimsrud PA, Kwon YJ, Pagliarini DJ, Gai X, Schurr TG, Huttemann M, Nakamaru-Ogiso E, and Falk MJ

Journal of Molecular Biology, 2014 426(11):2199-216

doi: 10.1016/j.jmb.2014.02.009 Epub 2014 Feb 14 PMID: 24534730 PMCID: PMC4067970

2013

21.

Hallmarks of a new era in mitochondrial biochemistry

Pagliarini DJ^† and Rutter J^†

Genes & Development, 2013 27(24): 2615-27

doi: 10.1101/gad.229724.113 Epub 2013 Dec 15 PMID: 24352419 PMCID: PMC3877752

20.

Amine-reactive neutron-encoded labels for highly-plexed proteomic quantitation

Hebert AS, Merrill AE, Stefely JA, Bailey DJ, Wenger CD, Westphall MS, Pagliarini DJ, and Coon JJ

Molecular and Cellular Proteomics, 2013 12(11):3360-9

doi: 10.1074/mcp.M113.032011 Epub 2013 Jul 23 PMID: 23882030 PMCID: PMC3820946

19.

Quantification of mitochondrial acetylation dynamics highlights prominent sites of metabolic regulation

Still AJ*, Floyd BJ*, Hebert AS*, Bingman CA, Gunderson DR, Dolan BK, Carson JJ, Dittenhafer-Reed KE, Stapleton DS, Keller MP, Westphall MS, Denu JM, Attie AD, Coon JJ, and Pagliarini DJ

Journal of Biological Chemistry, 2013 288(36):26209-19

doi: 10.1074/jbc.M113.483396 Epub 2013 Jul 17 PMID: 23864654 PMCID: PMC3764825

18.

Neutron-encoded mass signatures for multiplexed proteome quantification

Hebert AS, Merrill AE, Bailey DJ, Still AJ, Westphall MS, Strieter ER, Pagliarini DJ, and Coon JJ

Nature Methods, 2013 10(4):332-4

doi: 10.1038/nmeth.2378 Epub 2013 Feb 24 PMID: 23435260 PMCID: PMC3612390

17.

Complementary RNA and Protein Profiling Identifies Iron as a Key Regulator of Mitochondrial Biogenesis

Rensvold JW, Ong SE, Jeevananthan A, Carr SA, Mootha VK, and Pagliarini DJ

Cell Reports, 2013 3(1): 237-245

doi: 10.1016/j.celrep.2012.11.029 Epub 2013 Jan 10 PMID: 23318259 PMCID: PMC3812070

2012

16.

Calorie Restriction and SIRT3 Trigger Global Reprogramming of the Mitochondrial Protein Acetylome

Hebert AS, Dittenhafer-Reed KE, Yu W, Bailey DJ, Selen ES, Boersma MD, Carson JJ, Tonelli M, Balloon AJ, Higbee AJ, Westphall MS, Pagliarini DJ, Prolla TA, Assadi-Porter F, Roy S, Denu JM, and Coon JJ

Molecular Cell 2013, 49(1):186-199

doi: 10.1016/j.molcel.2012.10.024 Epub 2012 Nov 29 PMID: 23201123 PMCID: PMC3704155

15.

A quantitative map of the liver mitochondrial phosphoproteome reveals post-translational control of ketogenesis

Grimsrud PA*, Carson JJ*, Hebert AS, Hubler SL, Niemi NM, Bailey DJ, Jochem A, Stapleton DS, Keller MP, Westphall MS, Yandell BS, Attie AD, Coon JJ, and Pagliarini DJ

Cell Metabolism, 2012 16(5):672-83

doi: 10.1016/j.cmet.2012.10.004 Epub 2012 Nov 7 PMID: 23140645 PMCID: PMC3506251

14.

Automated Gas-Phase Purification for Accurate, Multiplexed Quantification on a Stand-Alone Ion-Trap Mass Spectrometer

Vincent CE, Rensvold JW, Westphall MS, Pagliarini DJ, and Coon JJ

Analytical Chemistry, 2013 85(4):2079-86

doi: 10.1021/ac302156t Epub 2012 Oct 24 PMID: 23046161 PMCID: PMC3568235

13.

Solution NMR Structure of Yeast Succinate Dehydrogenase Flavinylation Factor Sdh5 Reveals a Putative Sdh1 Binding Site

Eletsky A, Jeong MY, Kim H, Lee HW, Xiao R, Pagliarini DJ, Prestegard JH, Winge DR, Montelione GT, and Szyperski T

Biochemistry, 2012 51(43): 8475-7

doi: 10.1021/bi301171u Epub 2012 Oct 19 PMID: 23062074 PMCID: PMC3667956

12.

Inhibitors of bacterial tubulin target bacterial membranes in vivo

Foss MH, Eun YJ, Grove CI, Pauw DA, Sorto NA, Rensvold JW, Pagliarini DJ, Shaw JT, and Weibel DB

Med Chem Comm, 2013 4(1):112-119

doi: 10.1039/C2MD20127E Epub 2012 Jul 18 PMID: 23539337 PMCID: PMC3607388

11.

Analysis of the acidic proteome with negative electron-transfer dissociation mass spectrometry

McAlister GC, Russell JD, Rumachik NG, Hebert AS, Syka JEP, Geer LY, Westphall MS, Pagliarini DJ, and Coon JJ

Analytical Chemistry, 2012 84(6): 2875-82

doi: 10.1021/ac203430u Epub 2012 Mar 1 PMID: 22335612 PMCID: PMC3310326

2010

Pre-2012 (publications from postdoctoral and graduate work)

10.

Pharmacological Targeting of the Mitochondrial Phosphatase PTPMT1

Doughty-Shenton D, Joseph JD, Zhang J, Pagliarini DJ, Kim Y, Lu D, Dixon JE, and Casey PJ

The Journal of Pharmacology and Experimental Therapeutics, 2010 332(2): 584-92

doi: 10.1124/jpet.109.163329 Epub 2010 Feb 18 PMID: 20167843 PMCID: PMC2872949

2009

Upstream open reading frames cause widespread reduction of protein expression and are polymorphic among humans

Calvo SE*, Pagliarini DJ*, and Mootha VK

Proceedings of the National Academy of Sciences, 2009 106(18):7507-12 (Direct submission)

doi: 10.1073/pnas.0810916106 Epub 2009 Apr 16 PMID: 19372376 PMCID: PMC2669787

2008

Mutation of C20orf7 disrupts Complex I assembly and causes lethal neonatal mitochondrial disease

Sugiana C*, Pagliarini DJ*, McKenzie M, Kirby DM, Salemi R, Abu-Amero KK, Dahl HHM, Hutchison WM, Vascotto KA, Smith SM, Newbold RF, Christodoulou J, Calvo SE, Mootha VK, Ryan MT, and Thorburn DR

American Journal of Human Genetics, 2008 83(4):468-478

doi: 10.1016/j.ajhg.2008.09.009 Epub 2008 Oct PMID: 18940309 PMCID: PMC2561934

A mitochondrial protein compendium elucidates complex I disease biology

Pagliarini DJ*, Calvo SE*, Chang B, Sheth SA, Vafai SB, Ong SE, Walford GA, Sugiana C, Boneh A, Chen WK, Hill DE, Vidal M, Evans JG, Thorburn DR, Carr SA, and Mootha VK

Cell, 2008 134(1):112-123

doi: 10.1016/j.cell.2008.06.016 Epub 2008 Jul 11 PMID: 18614015 PMCID: PMC2778844

Dual Specific Phosphatases 18 and 21 Target to Opposing Sides of the Mitochondrial Inner Membrane

Rardin MJ, Wiley SE, Murphy AN, Pagliarini DJ, and Dixon JE

Journal of Biological Chemistry, 2008 283(22):15440-50

doi: 10.1074/jbc.M709547200 Epub 2008 Apr 2 PMID: 18385140 PMCID: PMC2397459

2005

Mitochondrial Modulation: Reversible Phosphorylation Takes Center Stage?

Pagliarini DJ and Dixon JE

Trends in Biochemical Sciences, 2006 31(1):26-34

doi: 10.1016/j.tibs.2005.11.005 Epub 2005 Dec 5 PMID: 16337125

Involvement of a Mitochondrial Phosphatase in the Regulation of ATP Production and Insulin Secretion in Pancreatic Beta Cells

Pagliarini DJ, Wiley SE, Kimple ME, Dixon JR, Kelly P, Worby CA, Casey PJ, and Dixon JE

Molecular Cell, 2005 19(2):197-207

doi: 10.1016/j.molcel.2005.06.008 Epub 2005 Jul 22 PMID: 16039589

2004

A PTEN-like phosphatase with a novel substrate specificity

Pagliarini DJ, Worby CA, and Dixon JE

Journal of Biological Chemistry, 2004 279(37):38590-96.

doi: 10.1074/jbc.M404959200 Epub 2004 Jul 9 PMID: 15247229

Protein Tyrosine Phosphatases

Pagliarini DJ*, Robinson, FL*, and Dixon, J.E.

Encyclopedia of Biochemistry, 2004 Vol. 3, 536-542

2003

A PTEN-related 5-Phosphatidylinositol Phosphatase Localized in the Golgi

Merlot S, Meili R, Pagliarini DJ, Maehama T, Dixon JE, and Firtel RA

Journal of Biological Chemistry, 2003 278(41):39866-73

PATENTS and INVENTION DISCLOSURES

doi: 10.1074/jbc.M306318200 Epub 2003 Jul 23 PMID: 12878591

Dixon JE and Pagliarini DJ, US 7,749,976 B2 (2010)

Isolated PTPMT1 protein which mediates insulin production and uses thereof

Coon JJ, Stefely JA, Kwiecien NW, Pagliarini DJ, Hebert AS, Veling MT. Biomarkers of Mitochondrial Dysfunction. Wisconsin Alumni Research Foundation Ref. No. P160312US01(2016)

Pagliarini DJ, Kemmerer ZA, and Reidenbach AG. Acute chemical-genetic inhibition of coenzyme Q biosynthesis. Wisconsin Alumni Research Foundation Ref. No. P180071 (2017)

Pagliarini DJ, Murray NH, Asquith C. Development of a specific inhibitor for coenzyme Q biosynthesis. Wisconsin Alumni Research Foundation Ref. No. P220068 (2021)

Pagliarini DJ, Steenberge L. Mitochondria-targeted CoQ delivery system. Wisconsin Alumni Research Foundation Ref. No. P220119 (2021)

INVITED RESEARCH PRESENTATIONS (2011 – present. Future presentations are in italics)

2027

Mar 8

Broad-MIT Seminars in Chemical Biology

Feb 17

University of Arkansas Integrative Metabolic Research Center

Jan 25

UF Scripps Graduate Student–Invited Seminar Series

Jan 7

Anderson Lecture, Rockefeller University

2026

Dec 3

UC Berkeley, Molecular Therapeutics Seminar Series, Department of Molecular and Cell Biology

Nov 2

Distinguished Alumni Award Lecture, UC San Diego Department of Pharmacology

Sep 1

University of Nebraska–Lincoln, Department of Biochemistry

May 13

UC Berkeley, Metabolic Biology Seminar Series, Department of Metabolic Biology and Nutrition

May 11

Arc Institute

Apr 16

Memorial Sloan Kettering Cancer Center

Apr 2

Vagelos Grand Rounds, WashU

Mar 23

INFORM (International Network for Fatty Acid Oxidation Research and Management) (virtual)

Mar 3

Yale, Department of Cell Biology

Feb 14

Keystone Symposium on Mitochondria Signaling in Physiology and Disease, Keystone, CO

Jan 7

29th Enzyme Mechanisms Conference, San Diego, CA

2025

Nov 20

UMass Medical School, Metabolism Network seminar series

Nov 10

Keynote speaker—Joint Midwest Metabolism/TRiMAD Conference

Sept 15

Frontiers in Metabolism — Mechanisms of Metabolic Disease, Morgridge Institute, Madison, WI

Sept 4

International Congress of Inborn Errors of Metabolism (ICIEM), Tokyo, Japan

July 13

Gordon Research Conference on Molecular Membrane Biology, New Hampshire

June 19

Mitochondrial Medicine 2025, United Mitochondrial Disease Foundation, St. Louis, MO

June 16

10^th Conference of the International CoQ10 Association, Copenhagen, Denmark

Mar 6

University of Utah, Department of Biochemistry

Feb 24

3^rdMitochondria Conference: From Basic Biology to Mechanisms of Disease, Dominican Republic

Jan 24

Emory University, Department of Pharmacology

2024

Nov 5

Brandeis University, Department of Biology

July 10

Gordon Research Conference on Mitochondria and Chloroplasts, Barcelona, Spain

Apr 4

University of Saskatchewan, Department of Biochemistry, Microbiology and Immunology

Mar 26

ASBMB annual meeting, San Antonio, TX

Mar 7

Harvard Medical School, Department of Cell Biology

Feb 8

Monash University, Melbourne, Australia

Feb 5

Lorne Conference on Protein Structure and Function, Lorne, Australia

2023

Oct 12

Joslin Longwood Area Diabetes and Metabolism series, Harvard Medical School

Sep 6

University of Colorado, Boulder, Department of Biochemistry

Apr 17

Distinguished Alumni Lectureship, University of Michigan Department of Biological Chemistry

Feb 23

Washington University School of Medicine Metabolism Obesity and Diabetes (MOD) seminar

2022

Nov 17

AussieMit, Sydney, Australia

Sept 30

EMBO/FEBS Advanced Course on Mitochondria in life, death and disease, Budva, Montenegro

Aug 15

ASBMB Symposium on Mass Spectrometry in the Health and Life Sciences, Broad Institute

July 22

Altos Laboratories, San Francisco

May 25

University of Freiburg, Collaborative Research Center (SFB), Freiburg, Germany

May 17

Helmholtz Munich, Munich, Germany

May 13

10^th Conference of the International CoQ10 Association, Hamburg, Germany

Apr 27

Case Western Reserve Undergraduate Biochemistry Gala — Keynote speaker

Apr 27

International Centre for Genomic Medicine in Neuromuscular Diseases, Cambridge, UK

Apr 4

Annual Congress of the European Proteomic Association, Leipzig, Germany

Mar 27

Keystone Symposium on Mitochondrial Biochemistry, Breckenridge, CO (Lead organizer)

Mar 2

Deuel Conference on Lipids, Monterey, California

Jan 11

Broad Institute Metabolism Program

2021

Dec 3

Karolinska Institute, MolMet seminar

Dec 2

Center for Cardiovascular Research, Washington University School of Medicine

Nov 9

University of Pennsylvania School of Medicine, Institute for Diabetes, Obesity, and Metabolism

Sep 23

EMBO Workshop on Mitochondrial Homeostasis and Human Disease, Catalonia, Spain

June 2

University of Washington, Department of Genome Sciences

May 7

Mitochondrial Medicine 2020, United Mitochondrial Disease Foundation

Apr 28

ASBMB annual meeting, San Diego, CA (award lecture)

Mar 10

Mitochondrial Collaborative Research Center, Cologne, Germany

Feb 11

Weill Cornell Medical College, Department of Biochemistry

2020

Apr 17

Cancer Metabolism and Signaling Symposium, New York Academy of Sciences

Mar 3

UT Southwestern, Distinguished Lecturer for the Cell Biology at the Leading Edge seminar series

Jan 31

Keystone Symposium on Diabetes: Glucose Control and Beyond, Santa Fe, NM

2019

Dec 9

Washington University School of Medicine, BJC Investigator Program Seminar

Nov 26

MIT, Department of Biology

Nov 4

EPFL, Lausanne, Switzerland

Oct 9

UW-Madison, Department of Neuroscience

Sept 27

NHLBI Symposium on Mitochondrial Networks and Energetics, Bethesda, MD

Apr 16

University of Notre Dame, Department of Biological Sciences

Apr 6

ASBMB annual meeting, Orlando, FL

Mar 20

Gordon Research Conference on Mitochondria in Health and Disease, Ventura, CA

Mar 4

Annual meeting of the US Human Proteome Organization (HUPO), Bethesda, MD

Feb 19

Duke University, Molecular Physiology Institute Integrative Biology seminar series

2018

Oct 26

Mayo Clinic Metabolism and Molecular Nutrition Symposium, Rochester, MN

Oct 11

Mitochondrial Plasticity in Metabolism and Signaling, Max Planck Institute, Cologne, Germany

Aug 24

Symposium honoring the careers of Jack Dixon, Tony Hunter, and Susan Taylor, La Jolla, CA

Aug 9

North American Mass Spectrometry Summer School, Madison, WI

June 27

Mitochondrial Medicine 2018, United Mitochondrial Disease Foundation, Nashville, TN

June 24

9^th Conference of the International CoQ10 Association, New York, NY

June 5

Cell Symposium on Mitochondria, San Diego, CA

May 9

Mitochondrial Medicine 2018, Wellcome Genome Campus, Cambridge, UK

Mar 13

Annual meeting of the US Human Proteome Organization (HUPO), Minneapolis, MN

2017

Nov 21

Harvard Medical School, Department of Cell Biology

Oct 25

EPFL Life Sciences Symposium, Frontiers in Metabolism, Lausanne, Switzerland

Oct 23

University of Freiburg, Institute for Biochemistry and Molecular Biology

Oct 18

UW-Madison, Department of Genetics

Oct 13

Mayo Clinic, Metabolomics Symposium

Aug 22

254^th American Chemical Society National Meeting, Washington D.C.

July 26

31st Annual Symposium of the Protein Society (Award Lecture), Montreal, Canada

May 25

FASEB Conference on Mitochondrial Biogenesis and Dynamics, West Palm Beach, Florida

Mar 9

Deuel Conference on Lipids, Monterey, California

Feb 23

Emory School of Medicine, Department of Biochemistry

Jan 18

Keystone Symposium on Mitochondrial Communication, Taos, New Mexico

2016

Dec 8

Michigan State University, Department of Biochemistry and Molecular Biology

Oct 26

UW-Madison, Institute on Aging

May 6

National Institutes of Health, NIDDK Proteomics group

Apr 2

ASBMB annual meeting, San Diego, CA

Mar 31

Baylor College of Medicine, Department of Biochemistry and Molecular Biology

Mar 22

University of Washington, Departments of Biochemistry and Pharmacology

Mar 9

University of California, Berkeley, Department of Nutritional Sciences and Toxicology

Feb 15

University of Iowa, Department of Biochemistry

2015

Nov 30

University of Kentucky, Department of Molecular and Cellular Biochemistry

Nov 11

Van Andel Research Institute, Grand Rapids, MI

Oct 9

8^th Conference of the International CoQ10 Association, Bologna, Italy

Oct 7

École Polytechnique Fédérale de Lausanne, Laboratory of Integrative & Systems Physiology

Sep 28

Morgridge Institute for Research Metabolism Workshop

June 26

UW-Madison Medical School, Endocrinology Grand Rounds

May 27

Johns Hopkins University, Department of Physiology

May 12

UW-Madison, Institute on Aging

Apr 29

University of Minnesota, Department of Biochemistry

Apr 10

University of Cincinnati Medical School, Hematology-Oncology Grand Rounds

Feb 18

Texas A&M University, Department of Biochemistry and Biophysics

Jan 13

Purdue University, Department of Biochemistry

2014

Dec 11

Yale University, Department of Pharmacology

Nov 5

Medical College of Wisconsin, Department of Biochemistry

Oct 21

Harvard Medical School, MGH Cardiovascular Research Center

Sept 24

Mechanisms of cellular compartmentalization, Philipps Universität Marburg, Germany

June 14

American Diabetes Association's Scientific Sessions Meeting, San Francisco, CA

June 6

Mitochondrial Medicine 2014, United Mitochondrial Disease Foundation Pittsburgh, PA

May 7

UW-Madison, McArdle Seminar in Cancer Biology

Apr 27

ASBMB annual meeting, San Diego, CA

Feb 17

Genentech, San Francisco, CA

Feb 15

Biophysical Society 58th Annual Meeting, San Francisco, CA

2013

Oct 3

Cell and Regenerative Biology Seminar Series, UW-Madison

Aug 1

2013 Wisconsin Human Proteomics Symposium

June 13

Howard Hughes Medical Institute Symposium in honor of Jack Dixon

May 13

University of Pennsylvania, The Children's Hospital of Philadelphia

Apr 29

UW-Madison Molecular and Cellular Pharmacology Seminar Series

Apr 10

Northeast Structural Genomics Consortium (NESG) Annual Meeting

2012

Oct 25

Wayne State University, Center for Molecular Medicine and Genetics

Oct 2

Chaos and Complex Systems Seminar, UW-Madison

June 13

Mitochondrial Medicine 2012, United Mitochondrial Disease Foundation, Bethesda, MD

Mar 15

Pittcon 2012, Orlando, FL

2011

Dec 6

Caltech, Division of Biology

Dec 2

Salk Institute, Glenn Symposium

May 11

HHMI, Janelia Farms, Expanding Roles of Mitochondria in Cell Biology and Disease

May 5

UW-Madison, Department of Nutritional Sciences

Mar 25

UW-Madison, Carbone Comprehensive Cancer Center

Mar 28

Northeast Structural Genomics Consortium (NESG) Annual Meeting

DAVID J. PAGLIARINI, PHD

PROFESSIONAL EXPERIENCE

EDUCATION & TRAINING

SELECTED HONORS AND AWARDS

PUBLICATIONS (*co-lead author; †co-senior author; # denotes publications on which I am a lead or corresponding author)

Pre-2012 (publications from postdoctoral and graduate work)

INVITED RESEARCH PRESENTATIONS (2011 – present. Future presentations are in italics)

FUNDING

Current

Completed

SERVICE

Washington University Service (2020 –)

Departmental service

Morgridge Institute Service (2015-2020)

Director, Metabolism Research Theme

Additional Morgridge Service

UW-Madison Service (2009-2020)

Biochemistry Department

College and University

Professional Service

National and International Grant reviews

Meeting planning

Advisory board membership

Editorial service

TEACHING and MENTORING

Classroom teaching

SOCIETY MEMBERSHIPS

National and international associations