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Open AccessFabry Disease: Cardiac Implications and Molecular Mechanisms
This review explores the interplay among metabolic dysfunction, oxidative stress, inflammation, and fibrosis in Fabry disease, focusing on their potential implications for cardiac involvement. We aim to discus...
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Article
Open AccessActivation of the integrated stress response rewires cardiac metabolism in Barth syndrome
Barth Syndrome (BTHS) is an inherited cardiomyopathy caused by defects in the mitochondrial transacylase TAFAZZIN (Taz), required for the synthesis of the phospholipid cardiolipin. BTHS is characterized by heart ...
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Article
Open AccessCardiac Involvement in Mitochondrial Disorders
We review pathophysiology and clinical features of mitochondrial disorders manifesting with cardiomyopathy.