Please use this identifier to cite or link to this item: https://doi.org/10.1038/s41419-019-2036-9
Title: Mitochondrial 3243A > G mutation confers pro-atherogenic and pro-inflammatory properties in MELAS iPS derived endothelial cells
Authors: Pek, N.M.Q.
Phua, Q.H.
Ho, B.X.
Pang, J.K.S.
Hor, J.-H.
An, O. 
Yang, H.H. 
Yu, Y.
Fan, Y.
Ng, S.-Y. 
Soh, B.-S. 
Issue Date: 2019
Publisher: Nature Publishing Group
Citation: Pek, N.M.Q., Phua, Q.H., Ho, B.X., Pang, J.K.S., Hor, J.-H., An, O., Yang, H.H., Yu, Y., Fan, Y., Ng, S.-Y., Soh, B.-S. (2019). Mitochondrial 3243A > G mutation confers pro-atherogenic and pro-inflammatory properties in MELAS iPS derived endothelial cells. Cell Death and Disease 10 (11) : 802. ScholarBank@NUS Repository. https://doi.org/10.1038/s41419-019-2036-9
Rights: Attribution 4.0 International
Abstract: Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a mitochondrial disorder that is commonly caused by the m.3243A > G mutation in the MT-TL1 gene encoding for mitochondrial tRNA(Leu(UUR)). While clinical studies reported cerebral infarcts, atherosclerotic lesions, and altered vasculature and stroke-like episodes (SLE) in MELAS patients, it remains unclear how this mutation causes the onset and subsequent progression of the disease. Here, we report that in addition to endothelial dysfunction, diseased endothelial cells (ECs) were found to be pro-atherogenic and pro-inflammation due to high levels of ROS and Ox-LDLs, and high basal expressions of VCAM-1, in particular isoform b, respectively. Consistently, more monocytes were found to adhere to MELAS ECs as compared to the isogenic control, suggesting the presence of an atherosclerosis-like pathology in MELAS. Notably, these disease phenotypes in endothelial cells can be effectively reversed by anti-oxidant treatment suggesting that the lowering of ROS is critical for treating patients with MELAS syndrome. � 2019, The Author(s).
Source Title: Cell Death and Disease
URI: https://scholarbank.nus.edu.sg/handle/10635/212919
ISSN: 20414889
DOI: 10.1038/s41419-019-2036-9
Rights: Attribution 4.0 International
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