Primary research articles
- Small Molecule Inhibition of the Archetypal UbiB protein COQ8
Murray NH, Asquith CR, Fang Z, Smith RW, Vasta JD, Zimprich CA, Corona CR, Robers MB, Bingman CA, and Pagliarini DJ
Nature Chemical Biology, 2022 - 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†
Molecular Cell, 2022 - 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 J, Peters SR, Linke V, Hutchins PD, Overmyer KA, Salome AZ, Hebert AS, Balnis CV, Kwiecien NW, Rush MJ, Westphall MS, Craven M, Akarsu NA, Taylor RW, Coon JJ†, and Pagliarini DJ†
Nature, 2022 doi: 10.1038/s41586-022-04765-3 - 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
Nature Communications, 2021 12(1):4769 - Pathogenic biallelic mutations in NDUFAF8 cause Leigh syndrome with an isolated complex I deficiency
Alston CL*, Veling MT*, Heidler J, Taylor LC, Alaimo JT, Sung AY, He L, Hopton S, Broomfield A, Pavaine J, Pavaine J, Diaz J, 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 - Pptc7 is an essential phosphatase that promotes mitochondrial metabolism by enabling protein import and processingNiemi NM, Wilson GM, Overmyer KA, Vögtle FN, Lohman DC, Schueler KL, Attie AD, Meisinger C, Coon JJ, and Pagliarini DJ
Nature Communications, 2019 10: 3197 - An isoprene lipid binding protein promotes eukaryotic coenzyme Q biosynthesis
Lohman DC*, Aydin D*, Von Bank H, Smith R, 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 - 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 - 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, Ulbrich A, Coon JJ, Trauner M, Pagliarini DJ†, and Auwerx J†
Cell Systems, 2018 6(6):709-721 - 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†
Cell Systems, 2018 6(1):125-135 - 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, JA, Johnson IE, Bingman CA, Markley JL, Coon JJ, Dal Peraro M, and Pagliarini DJ
Cell Chemical Biology, 2018 25(2):154-165 PREVIEW - 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 JPR, Russell JD, Coon JJ, and Pagliarini DJ
Molecular Cell, 2017 68(5):970-977 - Ptc7p Dephosphorylates Select Mitochondrial Proteins to Enhance Metabolic Function
Guo X*, Niemi NM*, Hutchins PD, Condon SG, Jochem A, Ulbrich A, Higbee AJ, Russell JD, Senes A, Coon JJ, Pagliarini DJ
Cell Reports, 2017 18(2):307-313 - Mitochondrial Protein Functions Elucidated by Multi-Omic Mass Spectrometry Profiling
Stefely JA*, Kwiecien NW*, Freiberger EC, Richards AL, Jochem A, Rush MJ, Ulbrich A, Robinson KP, Hutchins PD, Veiling MT, Guo X, Kemmerer ZA, Connors KJ, Trujillo EA, Sokol J, Marx H, Westphall MS, Hebert AS, Pagliarini DJ†, Coon JJ†
Nature Biotechnology, 2016 Sep 26 PMID: 27669165 PREVIEW - 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 JJ, Coon JJ, Pagliarini DJ
Molecular Cell, 2016 Aug 18;63(4):621-32 - 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 MJ, Kwiecien NW, Singh PK, Schlagowski AI, Floyd BJ, Forsman U, Sindelar PJ, Westphall MS, Pierrel F, Zoll J, Dal Peraro M, Kannan N, Bingman CA, Coon JJ, Isope P, Puccio H†, Pagliarini DJ†
Molecular Cell, 2016 Aug 18;63(4):608-2. - 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 - 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 CV, Chermside-Scaboo CJ, Kock LG, Britton SL, Pagliarini DJ, Coon JJ and Burant CF
Cell Metabolism, 2015, 3;21(3):468-78 - Mitochondrial COQ9 is a lipid binding protein that associates with COQ7 to enable coenzyme Q biosynthesis
Lohman DC*, Forouhar F*, Beebe ET, Stefeley MS, Minogue CE, Ulbrich A, Stefely JA, Sukumar S, Luna-Sánchez M, Lew S, Seetharaman J, Xiao R, Wang H, Wrobel RL, Everett JK, Mitchell JC, López LC, Coon JJ, Tong L† and Pagliarini DJ†
Proceedings of the National Academy of Sciences, 2014 111(44):E4697-705 - 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, 14068-77 - 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 - A quantitative map of the liver mitochondrial phosphoproteome reveals posttranslational 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: 672-83 - 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 USA 2009, 106: 7507-12 - Mutation of C20orf7 disrupts complex I assembly and causes lethal infantile mitochondrial disease
Sugiana C*, Pagliarini DJ*, McKenzie M, Kirby DM, Salemi R, Dahl HHM, Hutchison WM, Vascotto KA, Smith SM, Christodoulou J, Calvo SE, Mootha VK, Ryan MT and Thorburn DR
American Journal of Human Genetics 2008, 83(4): 468-78. - 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): 112-23. - 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, Casey PJ, and Dixon JE
Molecular Cell 2005, 19(2): 197-207 PREVIEW
Accepted cover for Floyd et al. 2016
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Reviews and commentaries
- The extensive and functionally uncharacterized mitochondrial phosphoproteome
Niemi NM and Pagliarini DJ
Journal of Biological Chemistry, 2021 Jun 15:100880 - Systems biochemistry approaches to defining mitochondrial protein function
Sung AY, Floyd BJ, and Pagliarini DJ
Cell Metabolism, 2020, 31 (4): 669-678 - 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 - A path to the powerhouse: systems-to-structure approaches for studying mitochondrial proteins
Pagliarini DJ
Protein Science, 2018 27(8):1518-1525 - Biochemistry of Mitochondrial Coenzyme Q Biosynthesis
Stefely JA and Pagliarini DJ
Trends in Biochemical Sciences, 2017 42(10):824-843 - Transomics: Mitochondrial Systems Analyses Get Supercomplex
Pagliarini DJ
Cell Metabolism, 2016 Jul 12;24(1):13-4 - Hallmarks of a new era in mitochondrial biochemistry
Pagliarini DJ† and Rutter J†
Genes and Development 2013, 27(24): 2615-27 - Mitochondrial modulation: reversible phosphorylation takes center stage?
Pagliarini DJ and Dixon JE
Trends in Biochemical Sciences 2006, 31(1): 26-34
Accepted cover for Rensvold et al. 2013
Created by Jarred Rensvold