Omaveloxolone, But Not Dimethyl Fumarate, Improves Cardiac Function in Friedreich's Ataxia Mice With Severe Cardiomyopathy

Omaveloxolone, But Not Dimethyl Fumarate, Improves Cardiac Function in Friedreich's Ataxia Mice With Severe Cardiomyopathy

Background Friedreich's ataxia (FA) is a genetic disorder caused by a severe decrease in FXN (frataxin) protein expression in mitochondria. The clinical manifestation of this disorder is a cerebellar ataxia; however, the common lethal component in FA is cardiomyopathy. Methods A conditional Fxn...

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Bibliographic Details
Main Authors: Lili Salinas, Francisco Figueroa, Claire B. Montgomery, Phung N. Thai, Nipavan Chiamvimonvat, Gino Cortopassi, Elena N. Dedkova
Format: Article
Language:English
Published: Wiley 2025-06-01
Series:Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
Subjects:
animal models of human disease
calcium signaling
cardiomyopathy
dimethyl fumarate
Friedreich's ataxia
omaveloxolone
Online Access:https://www.ahajournals.org/doi/10.1161/JAHA.124.038505
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https://www.ahajournals.org/doi/10.1161/JAHA.124.038505

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