Yvonne Wohlfarter

This study shows that TAFAZZIN deficiency disrupts mitochondrial maturation in cardiomyocytes, leading to structural and functional impairments associated with Barth syndrome cardiomyopathy.

This study uses stem cell models to investigate tissue-specific pathologies in Barth Syndrome caused by TAFAZZIN deficiency, identifying mitophagy defects and potential therapeutic targets.

The paper shows that tafazzin deficiency in Barth Syndrome protects against ferroptosis by altering mitochondrial cardiolipin metabolism, affecting VDAC function and reducing ROS availability for ferroptosis.