Sarcomere inefficiency at the heart of hypertrophic cardiomyopathy.
De strijd tegen toxisch eiwit in erfelijke hartziekten
Erfelijke hartspierziekte wordt veroorzaakt door een toxisch eiwit. Wij hebben sterke aanwijzingen dat dit toxische eiwit een energietekort veroorzaakt dat leidt tot hartfalen en hartstilstand. Dit project onderzoekt de veranderingen in het hart die veroorzaakt worden door het energietekort en daardoor leiden tot nieuwe therapieën die het ziekteproces voorkomen.
Battling toxic protein in inherited cardiac disease
Inherited cardiac disease is caused by a toxic protein. We have strong indications that the toxic protein triggers an energy deficit in the heart which causes cardiac disease and sudden death. This project will establish key pathomechanisms triggered by the energy deficit leading to novel therapeutic approaches to treat disease.
Producten
Titel: Mitochondrial dysfunction in human hypertrophic cardiomyopathy is linked to cardiomyocyte architecture disruption and corrected by improving NADH-driven mitochondrial respiration.
Magazine: European Heart Journal
Magazine: European Heart Journal
Titel: Integrating Clinical Phenotype With Multiomics Analyses of Human Cardiac Tissue Unveils Divergent Metabolic Remodeling in Genotype-Positive and Genotype-Negative Patients With Hypertrophic Cardiomyopathy.
Magazine: Circ Genom Precis Med.
Magazine: Circ Genom Precis Med.
Titel: End-diastolic force pre-activates cardiomyocytes and determines contractile force; role of titin and calcium.
Magazine: Journal of Physiology
Magazine: Journal of Physiology
Titel: Characterization of cardiac metabolism in iPSC-derived cardiomyocytes: lessons from maturation and disease modelling.
Magazine: Stem Cell Res Ther.
Magazine: Stem Cell Res Ther.
Titel: Western diet triggers cardiac dysfunction in heterozygous Mybpc3-targeted knock-in mice: A two-hit model of hypertrophic cardiomyopathy.
Magazine: J Mol Cell Cardiol Plus.
Magazine: J Mol Cell Cardiol Plus.
Titel: Untying the knot: protein quality control in inherited cardiomyopathies.
Magazine: Pflügers Archiv-European Journal of Physiology
Magazine: Pflügers Archiv-European Journal of Physiology
Titel: Sex differences in Hypertrophic Cardiomyopathy: new insights.
Magazine: Current Opinion in Cardiology.
Magazine: Current Opinion in Cardiology.
Titel: Cardiac disorders and pathophysiology of sarcomeric proteins.
Magazine: Physiological Reviews
Magazine: Physiological Reviews
Titel: Large scale contractility measurements reveal large atrioventricular and subtle interventricular differences in cultured unloaded rat cardiomyocytes.
Magazine: Frontiers in Physiology, section Striated Muscle Physiology
Magazine: Frontiers in Physiology, section Striated Muscle Physiology
Titel: Unraveling the genotype-phenotype relationship in hypertrophic cardiomyopathy: obesity-related cardiac defects as a major disease modifier.
Magazine: Journal of the American Heart Association (JAHA)
Magazine: Journal of the American Heart Association (JAHA)