Imbalanced Production of Reactive Oxygen Species and Mitochondrial Antioxidant SOD2 in Fabry Disease-Specific Human Induced Pluripotent Stem Cell-Differentiated Vascular Endothelial Cells

Fabry disease (FD) is an X-linked inherited lysosomal storage disease caused by α-galactosidase A (GLA) deficiency. Progressive intracellular accumulation of globotriaosylceramide (Gb3) is considered to be pathogenically responsible for the phenotype variability of FD that causes cardiovascular dysf...

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Udgivet i:Cell Transplant
Main Authors: Tseng, Wei-Lien, Chou, Shih-Jie, Chiang, Huai-Chih, Wang, Mong-Lien, Chien, Chian-Shiu, Chen, Kuan-Hsuan, Leu, Hsin-Bang, Wang, Chien-Ying, Chang, Yuh-Lih, Liu, Yung-Yang, Jong, Yuh-Jyh, Lin, Shinn-Zong, Chiou, Shih-Hwa, Lin, Shing-Jong, Yu, Wen-Chung
Format: Artigo
Sprog:Inglês
Udgivet: SAGE Publications 2017
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Article
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC5657705/
https://ncbi.nlm.nih.gov/pubmed/27938475
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3727/096368916X694265
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