Frataxin-deficient cardiomyocytes present an altered thiol-redox state which targets actin and pyruvate dehydrogenase
Friedreich ataxia (FA) is a cardioneurodegenerative disease caused by deficient frataxin expression. This mitochondrial protein has been related to iron homeostasis, energy metabolism, and oxidative stress. Previously, we set up a cardiac cellular model of FA based on neonatal rat cardiac myocytes (...
Main Authors: | , , , |
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Format: | Article |
Language: | English |
Published: |
Elsevier
2020-05-01
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Series: | Redox Biology |
Online Access: | http://www.sciencedirect.com/science/article/pii/S2213231719315150 |