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 (...

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Bibliographic Details
Main Authors: Rosa Purroy, Marta Medina-Carbonero, Joaquim Ros, Jordi Tamarit
Format: Article
Language:English
Published: Elsevier 2020-05-01
Series:Redox Biology
Online Access:http://www.sciencedirect.com/science/article/pii/S2213231719315150