Featured Publications

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• ACAD10 and ACAD11 enable mammalian 4-hydroxy acid lipid catabolism  
  Rashan EH, Bartlett AK, Khana DB, Zhang J, Jain R, Smith AJ, Baker ZN, Cook T, Caldwell A, Chevalier AR, Pfleger BF, Yuan P, Amador-Noguez D, Simcox JA, and Pagliarini DJ
  Nature Molecular & Structural Biology, 2025
• Triacylglycerol mobilization underpins mitochondrial stress recovery   
  Baker ZN, Zhu Y, Guerra RM, Smith AJ, Arra A, Serrano LR, Overmyer KA, Mukherji S, Craig EA, Coon JJ, Pagliarini DJ.  
  Nature Cell Biology, 2025
• 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  
  Nature Metabolism, 2024
• Pptc7 maintains mitochondrial protein content by suppressing receptor-mediated mitophagy
  Niemi NM†, Serrano L, Muehlbauer LK, Balnis C, Kozul K, Rashan EH, Shishkova E, Schueler K, Keller MP, Attie AD, Pagan J, Coon JJ, and Pagliarini DJ†
  Nature Communications, 2023
• Hem25p is required for mitochondrial IPP transport in fungi
  Tai JT, Guerra RM, Rogers SW, Fang Z, Muehlbauer KL, Shishkova E, 

              Overmyer KA, Coon JJ, and Pagliarini DJ
             Nature Cell Biology, 2023

• 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  
• 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