Pagliarini Lab
Pagliarini Lab
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    • Home
    • Research
      • Overview
      • Systems Biochemistry
      • CoQ Biosynthesis
      • Mitochondrial Regulation
    • Publications
      • Featured
      • All by year
    • News
    • Meet Our Team
      • Current Lab
      • Lab Alumni
    • Contact
  • Home
  • Research
    • Overview
    • Systems Biochemistry
    • CoQ Biosynthesis
    • Mitochondrial Regulation
  • Publications
    • Featured
    • All by year
  • News
  • Meet Our Team
    • Current Lab
    • Lab Alumni
  • Contact

Regulation of mitochondrial function and biogenesis

Nearly all cells rely on mitochondria for their core metabolic needs. As such, it is essential that cells are able to appropriately maintain the content, function, and activity of these organelles. Mitochondrial adaptation can involve transcriptional reprogramming, protease-based proteome remodeling, altered mitochondrial dynamics, and the use of post-translational modifications (PTMs) to regulate protein activity. We are exploring the various ways in which cells modulate mitochondrial protein content and function to meet their changing metabolic needs.

Selected Publications

  • 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) 
  • 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
  • Pptc7 is an essential phosphatase that promotes mitochondrial metabolism by enabling protein import and processing
    Niemi 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
  • 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
  • Integrative proteomics and biochemical analyses define Ptc6p as the Saccharomyces cerevisiae pyruvate dehydrogenase phosphatase
    Guo X, Niemi NM, Coon JJ, Pagliarini DJ
    Journal of Biological Chemistry, 2017 292(28): 11751-11759
  • 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
  • Quantification of mitochondrial acetylation dynamics highlights prominent sites of metabolic regulation
    Still AJ*, Floyd BJ*, Hebert AS*, Bingman CA, Carson JJ, Gunderson DR, Dolan BK, Grimsrud PA, 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 
  • Iron Deprivation Induces Transcriptional Regulation of Mitochondrial Biogenesis
    Rensvold JW, Krautkramer KA, Dowell JA, Denu JM, Pagliarini DJ
    Journal of Biological Chemistry, 2016 291(40):20827-20837
  • 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
    Cell Metabolism 2012, 16: 672-83 

Systems biochemistry approaches to defining mitochondrial protein function

Elucidating the mechanisms of coenzyme Q biosynthesis

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