Genetic and hypoxic alterations of the microRNA-210-ISCU1/2 axis promote iron–sulfur deficiency and pulmonary hypertension
Kevin White, Yu Lu, Sofia Annis, Andrew E Hale, B Nelson Chau, James E Dahlman, Craig Hemann, Alexander R Opotowsky, Sara O Vargas, Ivan Rosas, Mark A Perrella, Juan C Osorio, Kathleen J Haley, Brian B Graham, Rahul Kumar, Rajan Saggar, Rajeev Saggar, W Dean Wallace, David J Ross, Omar F Khan, Andrew Bader, Bernadette R Gochuico, Majed Matar, Kevin Polach, Nicolai M Johannessen, Haydn M Prosser, Daniel G Anderson, Robert Langer, Jay L Zweier, Laurence A Bindoff, David Systrom, Aaron B Waxman, Richard C Jin and Stephen Y Chan
Article first published online: 30 MAR 2015 | DOI: 10.15252/emmm.201404511
Hypoxia-inducible miR-210 down-regulates its targets ISCU1/2 to regulate pulmonary vascular and endothelial levels of Fe-S clusters upon acquired injury or mutations. A patient with ISCU loss-of-function mutation presents with pulmonary vasculopathy.