Jan Dudek

This study enhances iPSC-derived cardiomyocyte maturation by combining 3D culture with a metabolic medium, resulting in improved structural, electrophysiological, and functional characteristics.

The paper investigates how defective cardiolipin remodeling in Barth Syndrome affects pancreatic islet function and metabolism, finding improved glucose tolerance and compensatory metabolic adaptations in a mouse model.

This study shows that peroxisome dysfunction in mice and engineered human myocardium leads to heart failure through metabolic impairment and disrupted cellular respiration.

This study shows that Tafazzin-mediated cardiolipin remodeling is essential for metabolic stress response and effector function of inflammatory T cells, with implications for Barth syndrome.

This study compares patient-specific stem cell models of RBM20 mutations causing dilated cardiomyopathy and left ventricular non-compaction, identifying distinct molecular, cellular, and functional impairments.

This study creates a neuronal in vitro model of Fabry disease to investigate small fibre neuropathy mechanisms, finding Gb3 accumulation, altered ion channel function, and mitochondrial trafficking issues.