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Akademický článek

Characterization of cardiac metabolism in iPSC-derived cardiomyocytes: lessons from maturation and disease modeling

Autor: Sofija Vučković, Rafeeh Dinani, Edgar E. Nollet, Diederik W. D. Kuster, Jan Willem Buikema, Riekelt H. Houtkooper, Miranda Nabben, Jolanda van der Velden, Birgit Goversen

Publikováno v: Stem Cell Research & Therapy, Vol 13, Iss 1, Pp 1-19 (2022)

Abstract Background Induced pluripotent stem cell-derived cardiomyocytes (iPSC-CMs) have emerged as a powerful tool for disease modeling, though their immature nature currently limits translation into clinical practice. Maturation strategies increasi

Externí odkaz: https://doaj.org/article/bb40feeeeb3d4261ada3ef35f1e56c6f

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Akademický článek

Autor: Maike Schuldt, Larissa M. Dorsch, Jaco C. Knol, Thang V. Pham, Tim Schelfhorst, Sander R. Piersma, Cris dos Remedios, Michelle Michels, Connie R. Jimenez, Diederik W. D. Kuster, Jolanda van der Velden

Publikováno v: Frontiers in Cardiovascular Medicine, Vol 8 (2021)

Background: Sex-differences in clinical presentation contribute to the phenotypic heterogeneity of hypertrophic cardiomyopathy (HCM) patients. While disease prevalence is higher in men, women present with more severe diastolic dysfunction and worse s

Externí odkaz: https://doaj.org/article/a7d1201b49794d8bb3ab768cc56619f0

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Akademický článek

Unraveling the Genotype‐Phenotype Relationship in Hypertrophic Cardiomyopathy: Obesity‐Related Cardiac Defects as a Major Disease Modifier

Autor: Edgar E. Nollet, B. Daan Westenbrink, Rudolf A. de Boer, Diederik W. D. Kuster, Jolanda van der Velden

Publikováno v: Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease, Vol 9, Iss 22 (2020)

Abstract Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiomyopathy and is characterized by asymmetric septal thickening and diastolic dysfunction. More than 1500 mutations in genes encoding sarcomere proteins are associated with H

Externí odkaz: https://doaj.org/article/604db015d6844cd691235e57ea3eeac1

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Akademický článek

Large-Scale Contractility Measurements Reveal Large Atrioventricular and Subtle Interventricular Differences in Cultured Unloaded Rat Cardiomyocytes

Autor: Edgar E. Nollet, Emmy M. Manders, Max Goebel, Valentijn Jansen, Cord Brockmann, Jorrit Osinga, Jolanda van der Velden, Michiel Helmes, Diederik W. D. Kuster

Publikováno v: Frontiers in Physiology, Vol 11 (2020)

The chambers of the heart fulfill different hemodynamic functions, which are reflected in their structural and contractile properties. While the atria are highly elastic to allow filling from the venous system, the ventricles need to be able to produ

Externí odkaz: https://doaj.org/article/828eff94062f4859ba1a8aa52304554c

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Akademický článek

Sex-specific cardiac remodeling in early and advanced stages of hypertrophic cardiomyopathy.

Autor: Louise L A M Nijenkamp, Ilse A E Bollen, Hans W M Niessen, Cris G Dos Remedios, Michelle Michels, Corrado Poggesi, Carolyn Y Ho, Diederik W D Kuster, Jolanda van der Velden

Publikováno v: PLoS ONE, Vol 15, Iss 5, p e0232427 (2020)

Hypertrophic cardiomyopathy (HCM) is the most frequent genetic cardiac disease with a prevalence of 1:500 to 1:200. While most patients show obstructive HCM and a relatively stable clinical phenotype (stage II), a small group of patients progresses t

Externí odkaz: https://doaj.org/article/591161600ceb43598f6013c5668a4b68

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The RNA-binding protein QKI governs a muscle-specific alternative splicing program that shapes the contractile function of cardiomyocytes

Autor: Pablo Montañés-Agudo, Simona Aufiero, Eva N Schepers, Ingeborg van der Made, Lucia Cócera-Ortega, Auriane C Ernault, Stéphane Richard, Diederik W D Kuster, Vincent M Christoffels, Yigal M Pinto, Esther E Creemers

Publikováno v: Cardiovascular research, 119(5), 1161-1174. Oxford University Press
Montañés-Agudo, P, Aufiero, S, Schepers, E N, van der Made, I, Cócera-Ortega, L, Ernault, A C, Richard, S, Kuster, D W D, Christoffels, V M, Pinto, Y M & Creemers, E E 2023, ' The RNA-binding protein QKI governs a muscle-specific alternative splicing program that shapes the contractile function of cardiomyocytes ', Cardiovascular Research, vol. 119, no. 5, pp. 1161-1174 . https://doi.org/10.1093/cvr/cvad007
Cardiovascular Research, 119(5), 1161-1174. Oxford University Press

Aims In the heart, splicing factors orchestrate the functional properties of cardiomyocytes by regulating the alternative splicing of multiple genes. Work in embryonic stem cells has shown that the splicing factor Quaking (QKI) regulates alternative

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b50ebc788e68dadde46220d5ca49892c
https://pure.amc.nl/en/publications/the-rnabinding-protein-qki-governs-a-musclespecific-alternative-splicing-program-that-shapes-the-contractile-function-of-cardiomyocytes(48df7777-6df4-415c-856b-d4c9b3858e67).html

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Akademický článek

Microsatellite Loci Reveal Genetic Diversity of Asian Callery Pear (Pyrus calleryana) in the Species Native Range and in the North American Cultivars

Autor: Shiwani Sapkota, Sarah L. Boggess, Robert N. Trigiano, William E. Klingeman, Denita Hadziabdic, David R. Coyle, Bode A. Olukolu, Ryan D. Kuster, Marcin Nowicki

Publikováno v: Life, Vol 11, Iss 6, p 531 (2021)

Pyrus calleryana Decne. (Callery pear) includes cultivars that in the United States are popular ornamentals in commercial and residential landscapes. Last few decades, this species has increasingly naturalized across portions of the eastern and south

Externí odkaz: https://doaj.org/article/4d5f33983ec74fd3a74ba8006394f23a

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Mitochondrial dysfunction in human hypertrophic cardiomyopathy is linked to cardiomyocyte architecture disruption and corrected by improving NADH-driven mitochondrial respiration

Autor: Edgar E Nollet, Inez Duursma, Anastasiya Rozenbaum, Moritz Eggelbusch, Rob C I Wüst, Stephan A C Schoonvelde, Michelle Michels, Mark Jansen, Nicole N van der Wel, Kenneth C Bedi, Kenneth B Margulies, Jeff Nirschl, Diederik W D Kuster, Jolanda van der Velden

Publikováno v: Nollet, E E, Duursma, I, Rozenbaum, A, Eggelbusch, M, Wüst, R C I, Schoonvelde, S A C, Michels, M, Jansen, M, van der Wel, N N, Bedi, K C, Margulies, K B, Nirschl, J, Kuster, D W D & van der Velden, J 2023, ' Mitochondrial dysfunction in human hypertrophic cardiomyopathy is linked to cardiomyocyte architecture disruption and corrected by improving NADH-driven mitochondrial respiration ', European heart journal . https://doi.org/10.1093/eurheartj/ehad028
European heart journal. Oxford University Press

Aims Genetic hypertrophic cardiomyopathy (HCM) is caused by mutations in sarcomere protein-encoding genes (i.e. genotype-positive HCM). In an increasing number of patients, HCM occurs in the absence of a mutation (i.e. genotype-negative HCM). Mitocho

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::fbdc2e438828d47bff141acdc72a2c34
https://hdl.handle.net/1871.1/0946409f-827c-4433-af9c-265b50db328c

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Amino terminus of cardiac myosin binding protein-C regulates cardiac contractility

Publikováno v: Lynch, T L, Kumar, M, McNamara, J W, Kuster, D W D, Sivaguru, M, Singh, R R, Previs, M J, Lee, K H, Kuffel, G, Zilliox, M J, Lin, B L, Ma, W, Gibson, A M, Blaxall, B C, Nieman, M L, Lorenz, J N, Leichter, D M, Leary, O P, Janssen, P M L, de Tombe, P P, Gilbert, R J, Craig, R, Irving, T, Warshaw, D M & Sadayappan, S 2021, ' Amino terminus of cardiac myosin binding protein-C regulates cardiac contractility ', Journal of Molecular and Cellular Cardiology, vol. 156, pp. 33-44 . https://doi.org/10.1016/j.yjmcc.2021.03.009
Journal of Molecular and Cellular Cardiology
Journal of Molecular and Cellular Cardiology, Elsevier, 2021, 156, pp.33-44. ⟨10.1016/j.yjmcc.2021.03.009⟩
J Mol Cell Cardiol
Journal of Molecular and Cellular Cardiology, 156, 33-44. Academic Press Inc.

International audience; Phosphorylation of cardiac myosin binding protein-C (cMyBP-C) regulates cardiac contraction through modulation of actomyosin interactions mediated by the protein's amino terminal (N′)-region (C0-C2 domains, 358 amino acids).

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b9aaf27df6df4e97fdee0c961f48de4d
https://doi.org/10.1016/j.yjmcc.2021.03.009

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Akademický článek

Cardiomyocyte Hypocontractility and Reduced Myofibril Density in End-Stage Pediatric Cardiomyopathy

Autor: Ilse A. E. Bollen, Marijke van der Meulen, Kyra de Goede, Diederik W. D. Kuster, Michiel Dalinghaus, Jolanda van der Velden

Publikováno v: Frontiers in Physiology, Vol 8 (2017)

Dilated cardiomyopathy amongst children (pediatric cardiomyopathy, pediatric CM) is associated with a high morbidity and mortality. Because little is known about the pathophysiology of pediatric CM, treatment is largely based on adult heart failure t

Externí odkaz: https://doaj.org/article/18579255b4e1400bad9412aba537eb9c

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