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.
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Titel: Hypertrophic cardiomyopathy dysfunction mimicked in human engineered heart tissue and improved by sodium-glucose cotransporter 2 inhibitors.
Auteur: Wijnker PJM, Dinani R, van der Laan NC, Algül S, Knollmann BC, Verkerk AO, Remme CA, Zuurbier CJ, Kuster DWD, van der Velden J.
Magazine: Cardiovasc Res.
Auteur: Wijnker PJM, Dinani R, van der Laan NC, Algül S, Knollmann BC, Verkerk AO, Remme CA, Zuurbier CJ, Kuster DWD, van der Velden J.
Magazine: Cardiovasc Res.
Titel: Slower calcium handling balances faster crossbridge cycling in human MYBPC3 HCM.
Auteur: Pioner JM, Vitale G, Steczina S, Langione M, Margara F, Santini L, Giardini F, Lazzeri E, Piroddi N, Scellini B, Palandri C, Schuldt M, Spinelli V, Girolami F, Mazzarotto F, van der Velden J, Cerbai E, Tesi C, Olivotto I, Bueno-Orovio A, Sacconi L, Coppini R, Ferrantini C, Regnier M, Poggesi C.
Magazine: Circ Res
Auteur: Pioner JM, Vitale G, Steczina S, Langione M, Margara F, Santini L, Giardini F, Lazzeri E, Piroddi N, Scellini B, Palandri C, Schuldt M, Spinelli V, Girolami F, Mazzarotto F, van der Velden J, Cerbai E, Tesi C, Olivotto I, Bueno-Orovio A, Sacconi L, Coppini R, Ferrantini C, Regnier M, Poggesi C.
Magazine: Circ Res
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: Argiro A, Ho C, ?Day SM, van der Velden J, Cerbai E, Saberi S, Tardiff JC, Lakdawala NK, Olivotto I??????????????????????????????.
Auteur: Sex-related differences in genetic cardiomyopathies. Journal of the American Heart Association
Magazine: Journal of the American Heart Association
Auteur: Sex-related differences in genetic cardiomyopathies. Journal of the American Heart Association
Magazine: Journal of the American Heart Association
Titel: Real-Time Measurements of Calcium and Contractility Parameters in Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes.
Auteur: Dinani R, Manders E, Helmes M, Wang L, Knollmann B, Kuster DWD, van der Velden J.
Magazine: J Vis Exp.
Auteur: Dinani R, Manders E, Helmes M, Wang L, Knollmann B, Kuster DWD, van der Velden J.
Magazine: J Vis Exp.
Titel: EGFR/IGF1R Signaling Modulates Relaxation in Hypertrophic Cardiomyopathy.
Auteur: Algül S, Schuldt M, Manders E, Jansen V, Schlossarek S, de Goeij-de Haas R, Henneman AA, Piersma SR, Jimenez CR, Michels M, Carrier L, Helmes M, van der Velden J, Kuster DWD.
Magazine: Circ Res.
Auteur: Algül S, Schuldt M, Manders E, Jansen V, Schlossarek S, de Goeij-de Haas R, Henneman AA, Piersma SR, Jimenez CR, Michels M, Carrier L, Helmes M, van der Velden J, Kuster DWD.
Magazine: Circ Res.
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: Sex-related differences in protein expression in sarcomere mutation-positive hypertrophic cardiomyopathy.
Auteur: Schuldt M, Dorsch LM, Knol JC, Pham TV, Schelfhorst T, Piersma SR, dos Remedios C, Michels M, Jimenez CR, Kuster DWD, van der Velden J.
Magazine: Frontiers in Cardiovascular Medicine
Auteur: Schuldt M, Dorsch LM, Knol JC, Pham TV, Schelfhorst T, Piersma SR, dos Remedios C, Michels M, Jimenez CR, Kuster DWD, van der Velden J.
Magazine: Frontiers in Cardiovascular Medicine
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: Increased myocardial oxygen consumption precedes contractile dysfunction in hypertrophic cardiomyopathy caused by pathogenic TNNT2 gene variants.
Auteur: Parbhudayal RY, Harms HJ, Michels M, van Rossum AC, Germans T, van der Velden J.
Magazine: Journal of the American Heart Association
Auteur: Parbhudayal RY, Harms HJ, Michels M, van Rossum AC, Germans T, van der Velden J.
Magazine: Journal of the American Heart Association
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: Proteomic and functional studies reveal detyrosinated tubulin as treatment target in sarcomere mutation-induced hypertrophic cardiomyopathy.
Auteur: Schuldt M, Pei J, Harakalova M, Dorsch LM, Schlossarek S, Mokry M, PhD, Knol JC, Pham TV, Schelfhorst T, Piersma SR, dos Remedios C, Dalinghaus M, Michels M, Asselbergs FW, Moutin MJ, Carrier L, Jimenez CJ, van der Velden J,* Kuster DWD.*
Magazine: Circulation: Heart Failure
Auteur: Schuldt M, Pei J, Harakalova M, Dorsch LM, Schlossarek S, Mokry M, PhD, Knol JC, Pham TV, Schelfhorst T, Piersma SR, dos Remedios C, Dalinghaus M, Michels M, Asselbergs FW, Moutin MJ, Carrier L, Jimenez CJ, van der Velden J,* Kuster DWD.*
Magazine: Circulation: Heart Failure
Titel: Mechano-energetic uncoupling in heart failure.
Auteur: Aksentijevic D, Sedej S, Fauconnier J, Paillard M, Abdellatif M, Streckfuss-Bömeke K, Ventura-Clapier R, van der Velden J, de Boer RA, Bertero E, Dudek J, Sequeira V, Maack C.
Magazine: Nat Rev Cardiol.
Auteur: Aksentijevic D, Sedej S, Fauconnier J, Paillard M, Abdellatif M, Streckfuss-Bömeke K, Ventura-Clapier R, van der Velden J, de Boer RA, Bertero E, Dudek J, Sequeira V, Maack C.
Magazine: Nat Rev Cardiol.
Titel: Sex differences in Hypertrophic Cardiomyopathy: new insights.
Magazine: Current Opinion in Cardiology.
Magazine: Current Opinion in Cardiology.
Titel: Acute heart failure: mechanisms and pre-clinical models-a Scientific Statement of the ESC Working Group on Myocardial Function.
Auteur: Ciccarelli M…van der Velden J, Zacchigna S, Heymans S, Thum T, Tocchetti CG.
Magazine: Cardiovasc Res.
Auteur: Ciccarelli M…van der Velden J, Zacchigna S, Heymans S, Thum T, Tocchetti CG.
Magazine: Cardiovasc Res.
Titel: Multi-omics integration identifies key upstream regulators of pathomechanisms in hypertrophic cardiomyopathy due to truncating MYBPC3 mutations.
Auteur: Pei J, Schuldt M, Nagyova E, Gu Z, el Bouhaddani S, Yiangou L, Jansen M, Calis JJA, Dorsch LM, Snijders Blok Cvan den Dungen NAM, Lansu N, Boukens BJ, Efimov IR, Michels M, Verhaar MC, de Weger R, Vink A, van Steenbeek FG, Baas AF, Davis RP, Uh HW, Kuster DWD, Cheng C, Mokry M, van der Velden J, Asselbergs FW, Harakalova M.
Magazine: Clinical Genetics
Auteur: Pei J, Schuldt M, Nagyova E, Gu Z, el Bouhaddani S, Yiangou L, Jansen M, Calis JJA, Dorsch LM, Snijders Blok Cvan den Dungen NAM, Lansu N, Boukens BJ, Efimov IR, Michels M, Verhaar MC, de Weger R, Vink A, van Steenbeek FG, Baas AF, Davis RP, Uh HW, Kuster DWD, Cheng C, Mokry M, van der Velden J, Asselbergs FW, Harakalova M.
Magazine: Clinical Genetics
Titel: Wnt signaling and removal of cell-cell contact synergistically induce massive expansion of functional human iPSC-cardiomyocytes.
Auteur: Buikema JW, Lee S, Goodyer WR, Maas RG, Chirikian O, Li G, Miao Y, Paige SL, Lee D, Wu H, Paik DT, Rhee S, Tian L, Galdos FX, Puluca N, Beyersdorf B, Hu J, Beck A, Venkamatran S, Swami S, Wijnker P, Schuldt M, Dorsch LM, van Mil A, Red-Horse K, Wu JY, Geisen C, Hesse M, Serpooshan V, Jovinge S, Fleischmann BK, Doevendans PA, van der Velden J, Garcia KC, Wu JC, Sluijter JPG, Wu SM.
Magazine: Cell Stem Cell
Auteur: Buikema JW, Lee S, Goodyer WR, Maas RG, Chirikian O, Li G, Miao Y, Paige SL, Lee D, Wu H, Paik DT, Rhee S, Tian L, Galdos FX, Puluca N, Beyersdorf B, Hu J, Beck A, Venkamatran S, Swami S, Wijnker P, Schuldt M, Dorsch LM, van Mil A, Red-Horse K, Wu JY, Geisen C, Hesse M, Serpooshan V, Jovinge S, Fleischmann BK, Doevendans PA, van der Velden J, Garcia KC, Wu JC, Sluijter JPG, Wu SM.
Magazine: Cell Stem Cell
Titel: Cardiac disorders and pathophysiology of sarcomeric proteins.
Magazine: Physiological Reviews
Magazine: Physiological Reviews
Titel: Metabolomics in severe aortic stenosis reveals intermediates of nitric oxide synthesis as most distinctive markers.
Auteur: van Driel BO, Schuldt M, Algul S, Levin E, Güclü A, Germans T, van Rossum AC, Pei J, Harakalova M, Baas A, Jans JJM, van der Velden J.
Magazine: International Journal of Molecular Sciences
Auteur: van Driel BO, Schuldt M, Algul S, Levin E, Güclü A, Germans T, van Rossum AC, Pei J, Harakalova M, Baas A, Jans JJM, van der Velden J.
Magazine: International Journal of Molecular Sciences
Titel: Heterogeneous Dysregulation of Myosin Super-Relaxation and Energetics in Hypertrophic Cardiomyopathy.
Auteur: Ochala J, Feng M, Wang Q, Chaami C, Nollet EE, Lewis CTA, Hessel AL, Michels M, Bedi KC Jr, Margulies KB, Pinto JR, Campbell KS, Kuster DWD, van der Velden J.
Magazine: Circ Heart Fail.
Auteur: Ochala J, Feng M, Wang Q, Chaami C, Nollet EE, Lewis CTA, Hessel AL, Michels M, Bedi KC Jr, Margulies KB, Pinto JR, Campbell KS, Kuster DWD, van der Velden J.
Magazine: Circ Heart Fail.
Titel: Genotype-negative hypertrophic cardiomyopathy: Exploring the role of cardiovascular risk factors in disease expression..
Auteur: Schoonvelde SAC, Nollet EE, Zwetsloot PP, Knackstedt C, Germans T, Hirsch A, Schinkel AFL, van Slegtenhorst MA, Verhagen JMA, de Boer RA, van der Velden J, Michels M.
Magazine: Int J Cardiology
Auteur: Schoonvelde SAC, Nollet EE, Zwetsloot PP, Knackstedt C, Germans T, Hirsch A, Schinkel AFL, van Slegtenhorst MA, Verhagen JMA, de Boer RA, van der Velden J, Michels M.
Magazine: Int J Cardiology
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)
Titel: Massive expansion and cryopreservation of beating induced pluripotent stem cell-derived cardiomyocytes.
Auteur: Maas R, Lee S, Harakalova M, Snijders-Blok C, Goodyer WR, Hjortnaes J, Doevendans PAFM, van Laake LW, van der Velden J, Asselbergs FW, Wu J, Sluijter J, Wu SM, Buikema JW.
Magazine: Star protocols
Auteur: Maas R, Lee S, Harakalova M, Snijders-Blok C, Goodyer WR, Hjortnaes J, Doevendans PAFM, van Laake LW, van der Velden J, Asselbergs FW, Wu J, Sluijter J, Wu SM, Buikema JW.
Magazine: Star protocols
Titel: Experimental Models of Hypertrophic Cardiomyopathy: A Systematic Review.
Auteur: van den Dolder FW, Dinani R, Warnaar VAJ, Vuckovic S, Passadouro AS, Nassar AA, Ramsaroep AX, Burchell GB, Schoonmade LJ, van der Velden J, Goversen B.
Magazine: JACC Basic Transl Sci.
Auteur: van den Dolder FW, Dinani R, Warnaar VAJ, Vuckovic S, Passadouro AS, Nassar AA, Ramsaroep AX, Burchell GB, Schoonmade LJ, van der Velden J, Goversen B.
Magazine: JACC Basic Transl Sci.
Titel: Reciprocal organ interactions during heart failure – A position paper from the ESC-Working group of Myocardial function.
Auteur: 1. Ciccarelli M…van der Velden J, Zacchigna S, Thum T.
Magazine: Cardiovascular Research
Auteur: 1. Ciccarelli M…van der Velden J, Zacchigna S, Thum T.
Magazine: Cardiovascular Research
Titel: Distinct metabolomic signatures in preclinical and obstructive hypertrophic cardiomyopathy.
Auteur: Schuldt M, van Driel B, Algül S, Parbhdayal RY, Barge-Schaapveld DQCM, Güçlü A, Jansen M, Michels M, Baas AF, van de Wiel MA, Nieuwdorp M, Levin E, Germans T, Jans JJM, van der Velden J.
Magazine: Cells
Auteur: Schuldt M, van Driel B, Algül S, Parbhdayal RY, Barge-Schaapveld DQCM, Güçlü A, Jansen M, Michels M, Baas AF, van de Wiel MA, Nieuwdorp M, Levin E, Germans T, Jans JJM, van der Velden J.
Magazine: Cells
Titel: Animal models and animal-free innovations for cardiovascular research: current status and routes to be explored. Consensus document of the ESC working group on myocardial function and the ESC Working Group on Cellular Biology of the Heart.
Auteur: van der Velden J et al.
Magazine: Cardiovasc Res
Auteur: van der Velden J et al.
Magazine: Cardiovasc Res
Titel: Blood-based biomarkers for the prediction of hypertrophic cardiomyopathy prognosis: a systematic review and meta-analysis.
Auteur: Jansen M, Algül S, Bosman LP, Michels M, van der Velden J, de Boer RA, van Tintelen JP, Asselbergs FW, Baas AF.
Magazine: ESC Heart Failure
Auteur: Jansen M, Algül S, Bosman LP, Michels M, van der Velden J, de Boer RA, van Tintelen JP, Asselbergs FW, Baas AF.
Magazine: ESC Heart Failure
Titel: Effect of trimetazidine dihydrochloride therapy on myocardial external efficiency in pre-clinical individuals with a hypertrophic cardiomyopathy pathogenic variant: results of the ENERGY trial.
Auteur: van Driel BO, Schoonvelde SAC, Borodzicz-Jazdzyk S, Huurman R, Visch J, Robbers L, Harms H, Verhagen J, Vermeer A, van den Aardweg J, van Rossum AC, Germans T, Michels M, van der Velden J.
Magazine: Cardiovasc Research
Auteur: van Driel BO, Schoonvelde SAC, Borodzicz-Jazdzyk S, Huurman R, Visch J, Robbers L, Harms H, Verhagen J, Vermeer A, van den Aardweg J, van Rossum AC, Germans T, Michels M, van der Velden J.
Magazine: Cardiovasc Research
Titel: Targeted therapies in genetic cardiomyopathies: From molecular mechanisms to therapeutic targets. A position paper from the Heart Failure Association (HFA) and the Working Group on Myocardial Function of the European Society of Cardiology (ESC).
Auteur: de Boer RA…van der Velden J, Walsh R, Seferovic PM, Thum T.
Magazine: European Journal of Heart Failure
Auteur: de Boer RA…van der Velden J, Walsh R, Seferovic PM, Thum T.
Magazine: European Journal of Heart Failure
Titel: The microtubule signature in cardiac disease: etiology, disease stage, and age dependency.
Auteur: Algül S, Dorsch LM, Sorop O, Vink A, Michels M, Dos Remedios CG, Dalinghaus M, Merkus D, Duncker DJ, Kuster DWD, van der Velden J.
Magazine: J Comp Physiol B.
Auteur: Algül S, Dorsch LM, Sorop O, Vink A, Michels M, Dos Remedios CG, Dalinghaus M, Merkus D, Duncker DJ, Kuster DWD, van der Velden J.
Magazine: J Comp Physiol B.
Titel: Generation of induced pluripotent stem cell lines from five individuals from two families carrying a pathogenic Dutch MYBPC3 founder variant with variable degrees of hypertrophic cardiomyopathy.
Auteur: van den Dolder FW, Warnaar VAJ, Onderwater YL, Baas AF, Kuster DWD, van der Velden J.
Magazine: Stem Cell Res.
Auteur: van den Dolder FW, Warnaar VAJ, Onderwater YL, Baas AF, Kuster DWD, van der Velden J.
Magazine: Stem Cell Res.
Titel: Metabolic alterations in human hypertrophic cardiomyopathy.
Auteur: Nollet EE, Sequeira V, Maack C, Ochala J, van der Velden J.
Magazine: J Cardiovasc Aging
Auteur: Nollet EE, Sequeira V, Maack C, Ochala J, van der Velden J.
Magazine: J Cardiovasc Aging
Titel: Characterization of heterozygous and homozygous mouse models with the most common hypertrophic cardiomyopathy mutation MYBPC3c.2373InsG in the Netherlands.
Auteur: Hilderink S, Schuldt M, Goebel M, Jansen VJ, Manders E, Moorman S, Dorsch LM, van Steenbeek FG, van der Velden J, Kuster DWD.
Magazine: J Mol Cell Cardiol
Auteur: Hilderink S, Schuldt M, Goebel M, Jansen VJ, Manders E, Moorman S, Dorsch LM, van Steenbeek FG, van der Velden J, Kuster DWD.
Magazine: J Mol Cell Cardiol
Titel: Revealing the Functional Potential of human iPSC Models in Investigating Genetic Hypertrophic Cardiomyopathy
Auteur: Rafeeh Soleimanidinani
Auteur: Rafeeh Soleimanidinani
Titel: Metabolomics and metabolic therapy in hypertrophic cardiomyopathy
Auteur: Beau van Driel
Auteur: Beau van Driel
Titel: Unlocking the Molecular Cues for Cardiomyocyte Proliferation
Auteur: Qianliang Yuan
Auteur: Qianliang Yuan
Titel: Metabolic perturbations as trigger and driver of disease in hypertrophic cardiomyopathy
Auteur: Edgar Nollet
Auteur: Edgar Nollet