Abstract 11811: Endothelial Deficiency of Iron-sulfur Scaffolding Protein Nfu1 Drives Metabolic Dysfunction to Promote Pulmonary Hypertension

BackgroundDeficient iron-sulfur (Fe-S) cluster assembly reprograms endothelial metabolism leading to pulmonary hypertension (PH). NFU1 is an Fe-S scaffolding protein where genetic deficiency leads to infantile multiple mitochondrial dysfunction syndrome (MMDS) and PH. However, the role of NFU1 in th...

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Published in:Circulation (New York, N.Y.) N.Y.), 2019-11, Vol.140 (Suppl_1 Suppl 1), p.A11811-A11811
Main Authors: Perk, Dror, Culley, Miranda K, Yu, Qiujun, Tai, Yi Yin, Woodcock, Chen-Shan J, Chan, Stephen Y
Format: Article
Language:English
Online Access:Get full text
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Summary:BackgroundDeficient iron-sulfur (Fe-S) cluster assembly reprograms endothelial metabolism leading to pulmonary hypertension (PH). NFU1 is an Fe-S scaffolding protein where genetic deficiency leads to infantile multiple mitochondrial dysfunction syndrome (MMDS) and PH. However, the role of NFU1 in the pulmonary endothelium remains undefined.HypothesisGenetic and acquired NFU1 deficiency promotes metabolic rewiring and endothelial pathophenotypic changes consistent with PH.MethodsIn cultured human pulmonary arterial endothelial cells (PAECs), gene expression was modulated with hypoxia (
ISSN:0009-7322
1524-4539
DOI:10.1161/circ.140.suppl_1.11811