Quinzii Lab
Location and Contact Information
Principal Investigator
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
Eliana Barriocanal-Casado, PhD
- Postdoctoral Research Scientist
Alba Pesini, PhD
- Postdoctoral Research Scientist
Lucia Dec-Prat
- 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.
Sidhom EH, Kim C, Kost-Alimova M, Ting MT, Keller K, Avila-Pacheco J, Watts AJ, Vernon KA, Marshall JL, Reyes-Bricio E, Racette M, Wieder N, Kleiner G, Grinkevich EJ, Chen F, Weins A, Clish CB, Shaw JL, Quinzii CM, Greka A. Targeting a Braf/Mapk pathway rescues podocyte lipid peroxidation in CoQ deficiency kidney disease. J Clin Invest. 2021 Mar 1;131(5) :e141380. PMCID: 7919729.
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.