David J. Pagliarini, PhD

Professor of Cell Biology & Physiology

Hugo F. & Ina C. Urbauer Professor and BJC Investigator, Departments of Cell Biology and Physiology; Biochemistry and Molecular Biophysics; and Genetics

Research Interests

We are an interdisciplinary team of scientists driven to understand the biochemical underpinnings of mitochondrial dysfunction in human diseases. Together, we integrate large-scale methodologies with traditional biochemistry to investigate the modulation, adaptation, and basic metabolic function of mitochondria.

Professional Education
  • 1995-1999 BS Biochemistry, University of Notre Dame
  • 1999-2002 MS Biological Chemistry, University of Michigan
  • 2002-2005 PhD Biomolecular Sciences, UC San Diego
  • 2005-2009 Postdoctoral Fellow, Harvard Medical School

Pagliarini Lab

Couch Biomedical Research Building, Room 1127

Mitochondrial Metabolism | Protein Biochemistry | Chemical Biology | Systems Genetics | Rare Diseases

Mitochondria are complex and dynamic organelles that are home to a vast array of essential metabolic pathways and processes and whose dysfunction underlies hundreds of human diseases. Despite this, much of the basic biology of these organelles remains obscure, and therapeutic options to treat mitochondrial dysfunction remain woefully inadequate. By blending classic biochemistry, molecular & cellular biology, and genetics with large-scale proteomics and systems approaches, our lab aims to systematically define the functions of uncharacterized mitochondrial proteins, identify new gene mutations that underlie human disease, and explore new molecular therapeutics to rectify mitochondria-based disorders.


Loss of C2orf59 defines. A fatal autoinflammatory syndrome in humans and zebrafish that evokes a glycogen storage-associated mitochondriopathy (Links to an external site)

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, Ugurlu Ci Men D, Georgiadou D, Sotiropoulou K, Houlden H, Paul F, Pehlivan D, Laine C, Chai G, Ali NA, Choo SC, Keng SS, Boisson B, Yilmaz E, Xue S, Coon JJ, Ly TTN, Gilani N, Hasbini D, Kayserili H, Zaki M, Isfort RJ, Ordonez N, Tripolszki K, Bauer P, Rezaei N, Seyedpour S, Khotaei GT, Bascom CC, Maroofian R, Chaabouni M, Alsubhi A, Eyaid W, Isikay S, Gleeson JG, Lupiski JR, Casanova JL, Pagliarini DJ, Akarsu NA, Maurer-Stroh S, Centinkaya A, Bertoli-Avelli A, Mathuru AS, Ho L, Bard FA, and Reversade B