Quinzii Lab

Location and Contact Information

Columbia University College of Physicians and Surgeons (P&S)
630 West 168th Street
Room: 4-432
New York, NY 10032
United States

Principal Investigator

Catarina Quinzii Lab Electron Transport Chain Agus

The Quinzii lab studies mitochondrial diseases, with a particular focus on Coenzyme Q (CoQ) deficiency, and the role of mitochondrial dysfunction and CoQ deficiency in neurodegenerative diseases.

Publications

For a complete list of publications, please visit PubMed.gov.

Lab Members

  • Alba Pesini, PhD

    • Postdoctoral Research Scientist

  • Lucia Dec-Prat

    • Student

  • Abbigail Ranger

    • Student

Select Publications

  • Ziosi M, Di Meo I, Kleiner G, Gao, XH, Barca E, Sanchez Quintero M, Tadesse S, Jiang H, Qiao C, Rodenburg RJ, Scalais E, Schuelke M, Willard B, Hatzoglou M, Tiranti V and Quinzii CM. Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway. EMBO Mol Med. 2017 Jan;9(1):96-111. PMCID: PMC5210092.

  • Kleiner G, Barca E, Ziosi M, Emmanuele V, Xu Y, Hidalgo-Gutierrez A, Qiao C, Tadesse S, Area- Gomez E, Lopez LC, Quinzii CM. CoQ10 supplementation rescues nephrotic syndrome through normalization of H2S oxidation pathway. Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease. 2018; 6;1864(11):3708-3722. PMCID: PMC6181133.

  • Corral-Sarasa J, Martínez-Gálvez JM, González-García P, Wendling O, Jiménez-Sánchez L, López-Herrador S, Quinzii CM, Díaz-Casado ME, López LC. 4-Hydroxybenzoic acid rescues multisystemic disease and perinatal lethality in a mouse model of mitochondrial disease. Cell Rep. 2024 May 28;43(5):114148. PMID: 38697100

  • Pesini A, Barriocanal-Casado E, Compagnoni GM, Hidalgo-Gutierrez A, Yanez G, Bakkali M, Chhonker YS, Kleiner G, Larrea D, Tadesse S, Lopez LC, Murry DJ, Di Fonzo A, Area-Gomez E, Quinzii CM. Coenzyme Q10 deficiency disrupts lipid metabolism by altering cholesterol homeostasis in neurons. Free Radic Biol Med. 2025 Jan 7:S0891-5849(25)00009-7. doi: 10.1016/j.freeradbiomed.2025.01.009.

  • Pesini A, Sanchez-Quintero MJ, Lopez-Gomez C, Barriocanal-Casado E, Hidalgo-Gutierrez A, Nyvltova E, Rorbach J, Kleiner G, Ting MT, Garcia C, Dec-Prat L, Tanji K, Damoci CB, McFarland R, Taylor RW, Minczuk M, Barrientos A, Quinzii CM. RMND1 dysfunction causes OXPHOS deficiency and alters mitoribosomal protein homeostasis in vivo. EMBO Mol Med. 2026 Oct 14. doi: 10.1038/s44321-026-00534-1. Online ahead of print. PMID: 42875586 

  • Barriocanal-Casado E, Stopka SA, Pesini A, Aristizabal-Henao JJ, Karmacharya S, Van Cura D, Zhang R, Nickerson KR, Grover K, Zavidij O, Wessel SR, Narain NR, Modur V, Gesta S, Kiebish MA, Quinzii CM. Spatial mapping of CoQ10 repletion by BPM31510 in a genetic mouse model (Coq4F147C) of coenzyme Q deficiency. J Lipid Res. 2026 Mar;67(3):100987. doi: 10.1016/j.jlr.2026.100987. Epub 2026 Jan 29. PMID: 41616828 

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