Christoph Maack

This study evaluates mavacamten's effects on energy balance in RASopathy-associated HCM using hiPSC-CMs with a BRAF mutation.

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.

The paper identifies CAMK2-NR4A1 signaling as a driver of metabolic substrate switching in heart failure with reduced ejection fraction, linking it to cardiac dysfunction through glucose utilization and lipid depletion.

This study shows that IFN-gamma signaling in cardiomyocytes causes metabolic changes in the heart, including increased glucose uptake and reduced oxidative phosphorylation, which resemble changes in aging and heart failure.

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 shows that mitochondrial reactive oxygen species contribute to arrhythmias in hypertrophic cardiomyopathy by disrupting calcium handling and electrical conduction.

The paper shows that IDH2, a mitochondrial enzyme, acts as a major antioxidant defense in cardiomyocytes, with its activity compensating for reduced expression in heart failure, and identifies an epigenetic mechanism linking oxidative stress to cardiac function.

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.