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Mugoni, V, Postel, R, Catanzaro, V, De Luca, E, Turco, E, Digilio, G, Silengo, L, Murphy, MP, Medana, C, Stainier, DY, Bakkers, J and Santoro, MM (2013) Ubiad1 is an antioxidant enzyme that regulates eNOS activity by CoQ10 synthesis. Cell 152:504-18


Protection against oxidative damage caused by excessive reactive oxygen species (ROS) by an antioxidant network is essential for the health of tissues, especially in the cardiovascular system. Here, we identified a gene with important antioxidant features by analyzing a null allele of zebrafish ubiad1, called barolo (bar). bar mutants show specific cardiovascular failure due to oxidative stress and ROS-mediated cellular damage. Human UBIAD1 is a nonmitochondrial prenyltransferase that synthesizes CoQ10 in the Golgi membrane compartment. Loss of UBIAD1 reduces the cytosolic pool of the antioxidant CoQ10 and leads to ROS-mediated lipid peroxidation in vascular cells. Surprisingly, inhibition of eNOS prevents Ubiad1-dependent cardiovascular oxidative damage, suggesting a crucial role for this enzyme and nonmitochondrial CoQ10 in NO signaling. These findings identify UBIAD1 as a nonmitochondrial CoQ10-forming enzyme with specific cardiovascular protective function via the modulation of eNOS activity.


PubMed PMC3574195 Online version:10.1016/j.cell.2013.01.013


Animals; Dimethylallyltranstransferase/genetics; Dimethylallyltranstransferase/metabolism; Endothelial Cells/metabolism; Golgi Apparatus/metabolism; Heart/embryology; Humans; Myocardium/cytology; Nitric Oxide Synthase Type III/metabolism; Reactive Oxygen Species/metabolism; Ubiquinone/analogs & derivatives; Ubiquinone/genetics; Zebrafish/embryology; Zebrafish/metabolism; Zebrafish Proteins/genetics; Zebrafish Proteins/metabolism