Search

Your search keyword '"Jansen, Mark"' showing total 693 results
Start Over Author "Jansen, Mark"
693 results on '"Jansen, Mark"'

2. Penetrance and Prognosis of MYH7 Variant-Associated Cardiomyopathies: Results From a Dutch Multicenter Cohort Study

Author

Jansen, Mark, de Brouwer, Remco, Hassanzada, Fahima, Schoemaker, Angela E., Schmidt, Amand F., Kooijman-Reumerman, Maria D., Bracun, Valentina, Slieker, Martijn G., Dooijes, Dennis, Vermeer, Alexa M.C., Wilde, Arthur A.M., Amin, Ahmad S., Lekanne Deprez, Ronald H., Herkert, Johanna C., Christiaans, Imke, de Boer, Rudolf A., Jongbloed, Jan D.H., van Tintelen, J. Peter, Asselbergs, Folkert W., and Baas, Annette F.

Published
2024
Full Text
View/download PDF

3. Seller Debt in Acquisitions of Private Firms: A Security Design Approach.

Author

Jansen, Mark, Phalippou, Ludovic, and Noe, Thomas

Subjects
PRIVATE companies, MERGERS & acquisitions, VALUE (Economics), DEBT, ASSETS (Accounting)
Abstract

We propose a security design model in which a potential acquirer approaches a firm with a value-add plan. The target has a single owner, who possesses private information: he alone knows whether his firm is compatible with the plan. The owner agrees that the acquirer will add value but believes that the value-add will not be as much as what the acquirer expects. Although the acquirer can choose any monotone limited liability security to offer along with cash, we show that, under general conditions, any security that is employed always takes the form of nonrecourse debt provided by the seller. Authors have furnished an Internet Appendix , which is available on the Oxford University Press Web site next to the link to the final published paper online [ABSTRACT FROM AUTHOR]

Published
2024
Full Text
View/download PDF

4. Natural History of MYH7-Related Dilated Cardiomyopathy

Author

Cabrera-Romero, Eva, Cobo-Marcos, Marta, Escobar-Lopez, Luis, Domínguez, Fernando, González-López, Esther, Gimeno-Blanes, Juan Ramón, Dooijes, Dennis, López Ledesma, Bernabé, Roche Fortea, Inés, Bermejo, Javier, Espinosa, Maria Angeles, Fernández, Ana Isabel, Vilches, Silvia, Gómez, Cristina, Gómez, Juan, Coto, Eliecer, Rodríguez Reguero, José Julián, Heymans, S.R.B., Brunner, H.G., López-Díaz, Javier, Truszkowska, Grażyna, Ploski, Rafal, Chmielewski, Przemysław, Johnson, Renee, Robles-Mezcua, Ainhoa, Díaz-Expósito, Arancha, Pérez-Cabeza, Alejandro I., Jiménez-Rubio, Clara, Payá, Vicente Climent, Favilli, Silvia, Syrris, Petros, Cannie, Douglas, Billon, Clarisse, Lopez-Sainz, Angela, Calvo, Margarita, Fernández de Bobadilla, Ángela Cacicedo, Onaindia-Gandarias, Jose Juan, Gaztañaga-Arantzamendi, Larraitz, Zamarreño-Golvano, Estibaliz, Limeres, Javier, Gutiérrez-García, Laura, Villacorta, Eduardo, Haas, Jan, Krebsova, Alice, Mogensen, Jens, Cesar, Sergi, Campuzano, Oscar, Gutiérrez, Raúl Franco, Alvarez-Rubio, Jorge, Cremer-Luengos, David, Antoniutti, Guido, Caimi-Martinez, Fiama, Macías, Rosa, Jiménez-Jáimez, Juan, Peña-Peña, María Luisa, Díez-Aja López, Salvador Lucas, Acereda, Tania Pino, Corada, Blanca Arnáez, Piqueras-Flores, Jesús, Negreira-Caamaño, Martin, Río, Jorge Martinez-del, Mogollón Jiménez, María Victoria, Villanueva, Elena, Gonzáles, José Luis, Fernández, Adrián, Toscanini, Ulises, Favaloro, Lilian E., Díez, Carlota Hernández, de Frutos, Fernando, Ochoa, Juan Pablo, Navarro-Peñalver, Marina, Baas, Annette, Bjerre, Jesper Vandborg, Zorio, Esther, Méndez, Irene, Lorca, Rebeca, Verdonschot, Job A.J., García-Granja, Pablo Elpidio, Bilinska, Zofia, Fatkin, Diane, Fuentes-Cañamero, M. Eugenia, García-Pinilla, José M., García-Álvarez, María I., Girolami, Francesca, Barriales-Villa, Roberto, Díez-López, Carles, Lopes, Luis R., Wahbi, Karim, García-Álvarez, Ana, Rodríguez-Sánchez, Ibon, Rekondo-Olaetxea, Javier, Rodríguez-Palomares, José F., Gallego-Delgado, María, Meder, Benjamin, Kubanek, Milos, Hansen, Frederikke G., Restrepo-Córdoba, María Alejandra, Palomino-Doza, Julián, Ruiz-Guerrero, Luis, Sarquella-Brugada, Georgia, Perez-Perez, Alberto José, Bermúdez-Jiménez, Francisco José, Ripoll-Vera, Tomas, Rasmussen, Torsten Bloch, Jansen, Mark, Sabater-Molina, Maria, Elliot, Perry M., and Garcia-Pavia, Pablo

Published
2022
Full Text
View/download PDF

5. Spillover Effects of the Opioid Epidemic on Consumer Finance.

Author

Jansen, Mark

Subjects
SUBPRIME loans, EXTERNALITIES, AUTOMOBILE industry, DEFAULT (Finance), OPIOID epidemic, CORPORATE finance, CREDIT ratings
Abstract

I examine the impact of the opioid epidemic on subprime auto lending. Using a difference-in-differences framework, I find that county-level increases in opioid abuse cause an increase in loan defaults. Moreover, I find that traditional credit scoring attributes (e.g., FICO score) fail to predict loan performance deterioration associated with opioid addiction. The weak predictive performance of traditional credit measures and the resulting higher default rates generate a negative externality for borrowers in opioid-afflicted areas, as evidenced by 5.7% higher loan costs for subprime borrowers. [ABSTRACT FROM AUTHOR]

Published
2023
Full Text
View/download PDF

6. Clinical Features and Outcomes of Pediatric MYH7-Related Dilated Cardiomyopathy.

Author

de Frutos, Fernando, Pablo Ochoa, Juan, Webster, Gregory, Jansen, Mark, Remior, Paloma, Rasmussen, Torsten B., Sabater-Molina, Maria, Barriales-Villa, Roberto, Girolami, Francesca, Cesar, Sergi, Eugenia Fuentes-Cañamero, M., Alvarez García-Rovés, Reyes, Wahbi, Karim, Limeres, Javier, Kubanek, Milos, Slieker, Martijn G., Sarquella-Brugada, Georgia, Abrams, Dominic J., Dooijes, Dennis, and Domínguez, Fernando

Published
2024
Full Text
View/download PDF

8. Integrating Clinical Phenotype With Multiomics Analyses of Human Cardiac Tissue Unveils Divergent Metabolic Remodeling in Genotype-Positive and Genotype-Negative Patients With Hypertrophic Cardiomyopathy

Author

Onderzoek Precision medicine, Nollet, Edgar E., Schuldt, Maike, Sequeira, Vasco, Binek, Aleksandra, Pham, Thang V., Schoonvelde, Stephan A.C., Jansen, Mark, Schomakers, Bauke V., van Weeghel, Michel, Vaz, Fred M., Houtkooper, Riekelt H., Van Eyk, Jennifer E., Jimenez, Connie R., Michels, Michelle, Bedi, Kenneth C., Margulies, Kenneth B., dos Remedios, Cristobal G., Kuster, Diederik W.D., van der Velden, Jolanda, Onderzoek Precision medicine, Nollet, Edgar E., Schuldt, Maike, Sequeira, Vasco, Binek, Aleksandra, Pham, Thang V., Schoonvelde, Stephan A.C., Jansen, Mark, Schomakers, Bauke V., van Weeghel, Michel, Vaz, Fred M., Houtkooper, Riekelt H., Van Eyk, Jennifer E., Jimenez, Connie R., Michels, Michelle, Bedi, Kenneth C., Margulies, Kenneth B., dos Remedios, Cristobal G., Kuster, Diederik W.D., and van der Velden, Jolanda

Published
2024

9. Integrating Clinical Phenotype With Multiomics Analyses of Human Cardiac Tissue Unveils Divergent Metabolic Remodeling in Genotype-Positive and Genotype-Negative Patients With Hypertrophic Cardiomyopathy

Author

Nollet, Edgar E., Schuldt, Maike, Sequeira, Vasco, Binek, Aleksandra, Pham, Thang V., Schoonvelde, Stephan A.C., Jansen, Mark, Schomakers, Bauke V., van Weeghel, Michel, Vaz, Fred M., Houtkooper, Riekelt H., Van Eyk, Jennifer E., Jimenez, Connie R., Michels, Michelle, Bedi, Kenneth C., Margulies, Kenneth B., dos Remedios, Cristobal G., Kuster, Diederik W.D., van der Velden, Jolanda, Nollet, Edgar E., Schuldt, Maike, Sequeira, Vasco, Binek, Aleksandra, Pham, Thang V., Schoonvelde, Stephan A.C., Jansen, Mark, Schomakers, Bauke V., van Weeghel, Michel, Vaz, Fred M., Houtkooper, Riekelt H., Van Eyk, Jennifer E., Jimenez, Connie R., Michels, Michelle, Bedi, Kenneth C., Margulies, Kenneth B., dos Remedios, Cristobal G., Kuster, Diederik W.D., and van der Velden, Jolanda

Abstract

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is caused by sarcomere gene mutations (genotype-positive HCM) in ≈50% of patients and occurs in the absence of mutations (genotype-negative HCM) in the other half of patients. We explored how alterations in the metabolomic and lipidomic landscape are involved in cardiac remodeling in both patient groups. METHODS: We performed proteomics, metabolomics, and lipidomics on myectomy samples (genotype-positive N=19; genotype-negative N=22; and genotype unknown N=6) from clinically well-phenotyped patients with HCM and on cardiac tissue samples from sex- and age-matched and body mass index-matched nonfailing donors (N=20). These data sets were integrated to comprehensively map changes in lipid-handling and energy metabolism pathways. By linking metabolomic and lipidomic data to variability in clinical data, we explored patient group-specific associations between cardiac and metabolic remodeling. RESULTS:HCM myectomy samples exhibited (1) increased glucose and glycogen metabolism, (2) downregulation of fatty acid oxidation, and (3) reduced ceramide formation and lipid storage. In genotype-negative patients, septal hypertrophy and diastolic dysfunction correlated with lowering of acylcarnitines, redox metabolites, amino acids, pentose phosphate pathway intermediates, purines, and pyrimidines. In contrast, redox metabolites, amino acids, pentose phosphate pathway intermediates, purines, and pyrimidines were positively associated with septal hypertrophy and diastolic impairment in genotype-positive patients. CONCLUSIONS: We provide novel insights into both general and genotype-specific metabolic changes in HCM. Distinct metabolic alterations underlie cardiac disease progression in genotype-negative and genotype-positive patients with HCM.

Published
2024

10. Penetrance and Prognosis of MYH7 Variant-Associated Cardiomyopathies: Results From a Dutch Multicenter Cohort Study

Author

Onderzoek Precision medicine, Genetica Klinische Genetica, Cardiologie patientenzorg, Child Health, Circulatory Health, Genetica Sectie Genoomdiagnostiek, Genetica Groep Van Tintelen, Cancer, Team Medisch, Jansen, Mark, de Brouwer, Remco, Hassanzada, Fahima, Schoemaker, Angela E, Schmidt, Amand F, Kooijman-Reumerman, Maria D, Bracun, Valentina, Slieker, Martijn G, Dooijes, Dennis, Vermeer, Alexa M C, Wilde, Arthur A M, Amin, Ahmad S, Lekanne Deprez, Ronald H, Herkert, Johanna C, Christiaans, Imke, de Boer, Rudolf A, Jongbloed, Jan D H, van Tintelen, J Peter, Asselbergs, Folkert W, Baas, Annette F, Onderzoek Precision medicine, Genetica Klinische Genetica, Cardiologie patientenzorg, Child Health, Circulatory Health, Genetica Sectie Genoomdiagnostiek, Genetica Groep Van Tintelen, Cancer, Team Medisch, Jansen, Mark, de Brouwer, Remco, Hassanzada, Fahima, Schoemaker, Angela E, Schmidt, Amand F, Kooijman-Reumerman, Maria D, Bracun, Valentina, Slieker, Martijn G, Dooijes, Dennis, Vermeer, Alexa M C, Wilde, Arthur A M, Amin, Ahmad S, Lekanne Deprez, Ronald H, Herkert, Johanna C, Christiaans, Imke, de Boer, Rudolf A, Jongbloed, Jan D H, van Tintelen, J Peter, Asselbergs, Folkert W, and Baas, Annette F

Published
2024

11. Product Sales Incentive Spillovers to the Lending Market: Evidence from Subprime Auto Loan Defaults.

Author

Jansen, Mark, Pierce, Lamar, Snyder, Jason, and Nguyen, Hieu

Subjects
CONSUMER behavior, BUSINESS planning, INDUSTRIAL organization (Economic theory), DEFAULT (Finance), AUTOMOBILE industry, SUBPRIME automobile loans
Abstract

This paper shows how convex incentives in vertical contracts between manufacturers and retailers can induce sales behavior with costs to consumers. We examine this problem in the automotive sector, where manufacturers commonly motivate new vehicle sales through dealer incentive programs with large discrete bonuses determined by monthly sales targets. Using subprime car loans from over 3,500 dealerships, we document high default rates on new car loans originated at the end of the month—the period when dealerships attempt to secure target-based bonuses by intensifying efforts to sell new cars. We provide evidence consistent with the observed higher default rates resulting from customers purchasing new vehicles at month end. New car purchases stretch borrower budgets and expose borrowers to rapid depreciation, which consigns the borrower with negative equity through much of the loan term. Our results imply that the quartile of customers with the highest payment-to-income ratio see default rates increase from 13.6% to 19.7% on the last day of the month. Although consumers bear high costs from increased defaults, we find no evidence that lenders that purchase the loans are hurt by the default increase. Our results demonstrate how the behaviors induced by convex incentive schemes for sales are borne by customers. This paper was accepted by David Simchi-Levi, business strategy. Supplemental Material: The data files and online appendix are available at https://doi.org/10.1287/mnsc.2023.4935. [ABSTRACT FROM AUTHOR]

Published
2024
Full Text
View/download PDF

16. Quantifying the performance gain of 100 cm2 bifacial four terminal perovskite-Si tandem modules

Author

Manshanden Petra, Coletti Gianluca, Rosca Victor, Jansen Mark J., de Groot Koen, de Graaff Gertjan J., Creatore M., Simurka Lukas, Najafi Mehrdad, Zardetto Valerio, Dogan Ilkar, Fledderus Henri, and Veenstra Sjoerd C.

Subjects
four terminal tandem, bifacial modules, perovskite, outdoor performance, Renewable energy sources, TJ807-830
Abstract

Improving the performance of solar modules requires the implementation of both spectral and directional irradiance optimization. The performance of bifacial four-terminal tandem minimodules with a 100 cm2 area is reported, both indoor and outdoor measurements. We demonstrate a 24.5 mW/cm2 (bifacial irradiance 200 W/m2) bifacial tandem power density, measured according to a tandem adapted IEC60904-1-2 protocol, which constitutes a 3 mW/cm2 gain with respect to monofacial use. In addition, we show that in outdoor measurements bifacial four terminal tandems outperform identical monofacial four terminal tandems by 26% on average, depending on incident angle, injection level and temperature, in a 10% albedo environment. The average gain is higher for outdoor performance than for indoor performance, due to variations in relative rear irradiance.

Published
2022
Full Text
View/download PDF

18. Penetrance and Prognosis of MYH7 Variant-Associated Cardiomyopathies

Author

Jansen, Mark, primary, de Brouwer, Remco, additional, Hassanzada, Fahima, additional, Schoemaker, Angela E., additional, Schmidt, Amand F., additional, Kooijman-Reumerman, Maria D., additional, Bracun, Valentina, additional, Slieker, Martijn G., additional, Dooijes, Dennis, additional, Vermeer, Alexa M.C., additional, Wilde, Arthur A.M., additional, Amin, Ahmad S., additional, Lekanne Deprez, Ronald H., additional, Herkert, Johanna C., additional, Christiaans, Imke, additional, de Boer, Rudolf A., additional, Jongbloed, Jan D.H., additional, van Tintelen, J. Peter, additional, Asselbergs, Folkert W., additional, and Baas, Annette F., additional

Published
2023
Full Text
View/download PDF

19. Untargeted Metabolomics Identifies Potential Hypertrophic Cardiomyopathy Biomarkers in Carriers of MYBPC3 Founder Variants

Author

Jansen, Mark, Schuldt, Maike, Driel, Beau O. van, Schmidt, Amand F., Christiaans, Imke, Crabben, Saskia N. van der, Hoedemaekers, Y.M., Asselbergs, Folkert W., Baas, Annette F., Jansen, Mark, Schuldt, Maike, Driel, Beau O. van, Schmidt, Amand F., Christiaans, Imke, Crabben, Saskia N. van der, Hoedemaekers, Y.M., Asselbergs, Folkert W., and Baas, Annette F.

Abstract

Contains fulltext : 290549.pdf (Publisher’s version ) (Open Access)

Published
2023

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

Author

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

Abstract

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). Mitochondrial dysfunction is thought to be a key driver of pathological remodelling in HCM. Reports of mitochondrial respiratory function and specific disease-modifying treatment options in patients with HCM are scarce. Methods and results: Respirometry was performed on septal myectomy tissue from patients with HCM (n = 59) to evaluate oxidative phosphorylation and fatty acid oxidation. Mitochondrial dysfunction was most notably reflected by impaired NADH-linked respiration. In genotype-negative patients, but not genotype-positive patients, NADH-linked respiration was markedly depressed in patients with an indexed septal thickness ≥10 compared with <10. Mitochondrial dysfunction was not explained by reduced abundance or fragmentation of mitochondria, as evaluated by transmission electron microscopy. Rather, improper organization of mitochondria relative to myofibrils (expressed as a percentage of disorganized mitochondria) was strongly associated with mitochondrial dysfunction. Pre-incubation with the cardiolipin-stabilizing drug elamipretide and raising mitochondrial NAD+ levels both boosted NADH-linked respiration. Conclusion: Mitochondrial dysfunction is explained by cardiomyocyte architecture disruption and is linked to septal hypertrophy in genotype-negative HCM. Despite severe myocardial remodelling mitochondria were responsive to treatments aimed at restoring respiratory function, eliciting the mitochondria as a drug target to prevent and ameliorate cardiac disease in HCM. Mitochondria-targeting therapy may particularly benefit genotype-negative patients with HCM, given the tight link between mitochondrial impairment and septal thickening in this subpopulation.

Published
2023

22. Circulating Acylcarnitines Associated with Hypertrophic Cardiomyopathy Severity:an Exploratory Cross-Sectional Study in MYBPC3 Founder Variant Carriers

Author

Jansen, Mark, Schmidt, A. F., Jans, J. J.M., Christiaans, I., van der Crabben, S. N., Hoedemaekers, Y. M., Dooijes, D., Jongbloed, J. D.H., Boven, L. G., Lekanne Deprez, R. H., Wilde, A. A.M., van der Velden, J., de Boer, R. A., van Tintelen, J. P., Asselbergs, F. W., Baas, A. F., Jansen, Mark, Schmidt, A. F., Jans, J. J.M., Christiaans, I., van der Crabben, S. N., Hoedemaekers, Y. M., Dooijes, D., Jongbloed, J. D.H., Boven, L. G., Lekanne Deprez, R. H., Wilde, A. A.M., van der Velden, J., de Boer, R. A., van Tintelen, J. P., Asselbergs, F. W., and Baas, A. F.

Abstract

Hypertrophic cardiomyopathy (HCM) is a relatively common genetic heart disease characterised by myocardial hypertrophy. HCM can cause outflow tract obstruction, sudden cardiac death and heart failure, but severity is highly variable. In this exploratory cross-sectional study, circulating acylcarnitines were assessed as potential biomarkers in 124 MYBPC3 founder variant carriers (59 with severe HCM, 26 with mild HCM and 39 phenotype-negative [G + P-]). Elastic net logistic regression identified eight acylcarnitines associated with HCM severity. C3, C4, C6-DC, C8:1, C16, C18 and C18:2 were significantly increased in severe HCM compared to G + P-, and C3, C6-DC, C8:1 and C18 in mild HCM compared to G + P-. In multivariable linear regression, C6-DC and C8:1 correlated to log-transformed maximum wall thickness (coefficient 5.01, p = 0.005 and coefficient 0.803, p = 0.007, respectively), and C6-DC to log-transformed ejection fraction (coefficient -2.50, p = 0.004). Acylcarnitines seem promising biomarkers for HCM severity, however prospective studies are required to determine their prognostic value. Graphical abstract: [Figure not available: see fulltext.].

Published
2023

23. Circulating Acylcarnitines Associated with Hypertrophic Cardiomyopathy Severity: an Exploratory Cross-Sectional Study in MYBPC3 Founder Variant Carriers

Author

Onderzoek Precision medicine, Brain, Genetica Sectie Metabole Diagnostiek, Child Health, Genetica, Genetica Sectie Genoomdiagnostiek, Circulatory Health, Arts-assistenten Radiotherapie, Genetica Groep Van Tintelen, Cancer, Team Medisch, Genetica Klinische Genetica, Jansen, Mark, Schmidt, A F, Jans, J J M, Christiaans, I, van der Crabben, S N, Hoedemaekers, Y M, Dooijes, D, Jongbloed, J D H, Boven, L G, Lekanne Deprez, R H, Wilde, A A M, van der Velden, J, de Boer, R A, van Tintelen, J P, Asselbergs, F W, Baas, A F, Onderzoek Precision medicine, Brain, Genetica Sectie Metabole Diagnostiek, Child Health, Genetica, Genetica Sectie Genoomdiagnostiek, Circulatory Health, Arts-assistenten Radiotherapie, Genetica Groep Van Tintelen, Cancer, Team Medisch, Genetica Klinische Genetica, Jansen, Mark, Schmidt, A F, Jans, J J M, Christiaans, I, van der Crabben, S N, Hoedemaekers, Y M, Dooijes, D, Jongbloed, J D H, Boven, L G, Lekanne Deprez, R H, Wilde, A A M, van der Velden, J, de Boer, R A, van Tintelen, J P, Asselbergs, F W, and Baas, A F

Published
2023

24. Untargeted Metabolomics Identifies Potential Hypertrophic Cardiomyopathy Biomarkers in Carriers of MYBPC3 Founder Variants

Author

Onderzoek Precision medicine, Genetica, Genetica Sectie Genoomdiagnostiek, Circulatory Health, Genetica Sectie Metabole Diagnostiek, Brain, Child Health, Cancer, Team Medisch, Genetica Klinische Genetica, Jansen, Mark, Schuldt, Maike, van Driel, Beau O, Schmidt, Amand F, Christiaans, Imke, van der Crabben, Saskia N, Hoedemaekers, Yvonne M, Dooijes, Dennis, Jongbloed, Jan D H, Boven, Ludolf G, Deprez, Ronald H Lekanne, Wilde, Arthur A M, Jans, Judith J M, van der Velden, Jolanda, de Boer, Rudolf A, van Tintelen, J Peter, Asselbergs, Folkert W, Baas, Annette F, Onderzoek Precision medicine, Genetica, Genetica Sectie Genoomdiagnostiek, Circulatory Health, Genetica Sectie Metabole Diagnostiek, Brain, Child Health, Cancer, Team Medisch, Genetica Klinische Genetica, Jansen, Mark, Schuldt, Maike, van Driel, Beau O, Schmidt, Amand F, Christiaans, Imke, van der Crabben, Saskia N, Hoedemaekers, Yvonne M, Dooijes, Dennis, Jongbloed, Jan D H, Boven, Ludolf G, Deprez, Ronald H Lekanne, Wilde, Arthur A M, Jans, Judith J M, van der Velden, Jolanda, de Boer, Rudolf A, van Tintelen, J Peter, Asselbergs, Folkert W, and Baas, Annette F

Published
2023

25. Penetrance and Prognosis of MYH7Variant-Associated Cardiomyopathies

Author

Jansen, Mark, de Brouwer, Remco, Hassanzada, Fahima, Schoemaker, Angela E., Schmidt, Amand F., Kooijman-Reumerman, Maria D., Bracun, Valentina, Slieker, Martijn G., Dooijes, Dennis, Vermeer, Alexa M.C., Wilde, Arthur A.M., Amin, Ahmad S., Lekanne Deprez, Ronald H., Herkert, Johanna C., Christiaans, Imke, de Boer, Rudolf A., Jongbloed, Jan D.H., van Tintelen, J. Peter, Asselbergs, Folkert W., and Baas, Annette F.

Abstract

MYH7variants cause hypertrophic cardiomyopathy (HCM), noncompaction cardiomyopathy (NCCM), and dilated cardiomyopathy (DCM). Screening of relatives of patients with genetic cardiomyopathy is recommended from 10 to 12 years of age onward, irrespective of the affected gene.

Published
2024
Full Text
View/download PDF

26. Untargeted Metabolomics Identifies Potential Hypertrophic Cardiomyopathy Biomarkers in Carriers of MYBPC3 Founder Variants

Author

Jansen, Mark, primary, Schuldt, Maike, additional, van Driel, Beau O., additional, Schmidt, Amand F., additional, Christiaans, Imke, additional, van der Crabben, Saskia N., additional, Hoedemaekers, Yvonne M., additional, Dooijes, Dennis, additional, Jongbloed, Jan D. H., additional, Boven, Ludolf G., additional, Deprez, Ronald H. Lekanne, additional, Wilde, Arthur A. M., additional, Jans, Judith J. M., additional, van der Velden, Jolanda, additional, de Boer, Rudolf A., additional, van Tintelen, J. Peter, additional, Asselbergs, Folkert W., additional, and Baas, Annette F., additional

Published
2023
Full Text
View/download PDF

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

Author

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

Published
2023
Full Text
View/download PDF

29. Natural History of MYH7-Related Dilated Cardiomyopathy

Author

de Frutos, Fernando, primary, Ochoa, Juan Pablo, additional, Navarro-Peñalver, Marina, additional, Baas, Annette, additional, Bjerre, Jesper Vandborg, additional, Zorio, Esther, additional, Méndez, Irene, additional, Lorca, Rebeca, additional, Verdonschot, Job A.J., additional, García-Granja, Pablo Elpidio, additional, Bilinska, Zofia, additional, Fatkin, Diane, additional, Fuentes-Cañamero, M. Eugenia, additional, García-Pinilla, José M., additional, García-Álvarez, María I., additional, Girolami, Francesca, additional, Barriales-Villa, Roberto, additional, Díez-López, Carles, additional, Lopes, Luis R., additional, Wahbi, Karim, additional, García-Álvarez, Ana, additional, Rodríguez-Sánchez, Ibon, additional, Rekondo-Olaetxea, Javier, additional, Rodríguez-Palomares, José F., additional, Gallego-Delgado, María, additional, Meder, Benjamin, additional, Kubanek, Milos, additional, Hansen, Frederikke G., additional, Restrepo-Córdoba, María Alejandra, additional, Palomino-Doza, Julián, additional, Ruiz-Guerrero, Luis, additional, Sarquella-Brugada, Georgia, additional, Perez-Perez, Alberto José, additional, Bermúdez-Jiménez, Francisco José, additional, Ripoll-Vera, Tomas, additional, Rasmussen, Torsten Bloch, additional, Jansen, Mark, additional, Sabater-Molina, Maria, additional, Elliot, Perry M., additional, Garcia-Pavia, Pablo, additional, Cabrera-Romero, Eva, additional, Cobo-Marcos, Marta, additional, Escobar-Lopez, Luis, additional, Domínguez, Fernando, additional, González-López, Esther, additional, Gimeno-Blanes, Juan Ramón, additional, Dooijes, Dennis, additional, López Ledesma, Bernabé, additional, Roche Fortea, Inés, additional, Bermejo, Javier, additional, Espinosa, Maria Angeles, additional, Fernández, Ana Isabel, additional, Vilches, Silvia, additional, Gómez, Cristina, additional, Gómez, Juan, additional, Coto, Eliecer, additional, Rodríguez Reguero, José Julián, additional, Heymans, S.R.B., additional, Brunner, H.G., additional, López-Díaz, Javier, additional, Truszkowska, Grażyna, additional, Ploski, Rafal, additional, Chmielewski, Przemysław, additional, Johnson, Renee, additional, Robles-Mezcua, Ainhoa, additional, Díaz-Expósito, Arancha, additional, Pérez-Cabeza, Alejandro I., additional, Jiménez-Rubio, Clara, additional, Payá, Vicente Climent, additional, Favilli, Silvia, additional, Syrris, Petros, additional, Cannie, Douglas, additional, Billon, Clarisse, additional, Lopez-Sainz, Angela, additional, Calvo, Margarita, additional, Fernández de Bobadilla, Ángela Cacicedo, additional, Onaindia-Gandarias, Jose Juan, additional, Gaztañaga-Arantzamendi, Larraitz, additional, Zamarreño-Golvano, Estibaliz, additional, Limeres, Javier, additional, Gutiérrez-García, Laura, additional, Villacorta, Eduardo, additional, Haas, Jan, additional, Krebsova, Alice, additional, Mogensen, Jens, additional, Cesar, Sergi, additional, Campuzano, Oscar, additional, Gutiérrez, Raúl Franco, additional, Alvarez-Rubio, Jorge, additional, Cremer-Luengos, David, additional, Antoniutti, Guido, additional, Caimi-Martinez, Fiama, additional, Macías, Rosa, additional, Jiménez-Jáimez, Juan, additional, Peña-Peña, María Luisa, additional, Díez-Aja López, Salvador Lucas, additional, Acereda, Tania Pino, additional, Corada, Blanca Arnáez, additional, Piqueras-Flores, Jesús, additional, Negreira-Caamaño, Martin, additional, Río, Jorge Martinez-del, additional, Mogollón Jiménez, María Victoria, additional, Villanueva, Elena, additional, Gonzáles, José Luis, additional, Fernández, Adrián, additional, Toscanini, Ulises, additional, Favaloro, Lilian E., additional, and Díez, Carlota Hernández, additional

Published
2022
Full Text
View/download PDF

32. Quantifying the performance gain of 100 cm2bifacial four terminal perovskite-Si tandem modules

Author

Manshanden, Petra, Coletti, Gianluca, Rosca, Victor, Jansen, Mark J., de Groot, Koen, de Graaff, Gertjan J., Creatore, M., Simurka, Lukas, Najafi, Mehrdad, Zardetto, Valerio, Dogan, Ilkar, Fledderus, Henri, Veenstra, Sjoerd C., Manshanden, Petra, Coletti, Gianluca, Rosca, Victor, Jansen, Mark J., de Groot, Koen, de Graaff, Gertjan J., Creatore, M., Simurka, Lukas, Najafi, Mehrdad, Zardetto, Valerio, Dogan, Ilkar, Fledderus, Henri, and Veenstra, Sjoerd C.

Abstract

Improving the performance of solar modules requires the implementation of both spectral and directional irradiance optimization. The performance of bifacial four-terminal tandem minimodules with a 100 cm2 area is reported, both indoor and outdoor measurements. We demonstrate a 24.5 mW/cm2 (bifacial irradiance 200 W/m2) bifacial tandem power density, measured according to a tandem adapted IEC60904-1-2 protocol, which constitutes a 3 mW/cm2 gain with respect to monofacial use. In addition, we show that in outdoor measurements bifacial four terminal tandems outperform identical monofacial four terminal tandems by 26% on average, depending on incident angle, injection level and temperature, in a 10% albedo environment. The average gain is higher for outdoor performance than for indoor performance, due to variations in relative rear irradiance.

Published
2022

33. Natural History of MYH7-Related Dilated Cardiomyopathy

Author

Frutos, F. de, Ochoa, J.P., Navarro-Peñalver, M., Baas, A, Bjerre, J.V., Zorio, E., Méndez, I., Lorca, R., Verdonschot, J.A.J., García-Granja, P.E., Bilinska, Z., Fatkin, D., Fuentes-Cañamero, M.E., García-Pinilla, J.M., García-Álvarez, M.I., Girolami, F., Barriales-Villa, R., Díez-López, C., Lopes, L.R., Wahbi, K., García-Álvarez, A., Rodríguez-Sánchez, I., Rekondo-Olaetxea, J., Rodríguez-Palomares, J.F., Gallego-Delgado, M., Meder, B., Kubanek, M., Hansen, F.G., Restrepo-Córdoba, M.A., Palomino-Doza, J., Ruiz-Guerrero, L., Sarquella-Brugada, G., Perez-Perez, A.J., Bermúdez-Jiménez, F.J., Ripoll-Vera, T., Rasmussen, T.B., Jansen, Mark, Sabater-Molina, M., Brunner, H.G., Elliot, P.M., Garcia-Pavia, P., Frutos, F. de, Ochoa, J.P., Navarro-Peñalver, M., Baas, A, Bjerre, J.V., Zorio, E., Méndez, I., Lorca, R., Verdonschot, J.A.J., García-Granja, P.E., Bilinska, Z., Fatkin, D., Fuentes-Cañamero, M.E., García-Pinilla, J.M., García-Álvarez, M.I., Girolami, F., Barriales-Villa, R., Díez-López, C., Lopes, L.R., Wahbi, K., García-Álvarez, A., Rodríguez-Sánchez, I., Rekondo-Olaetxea, J., Rodríguez-Palomares, J.F., Gallego-Delgado, M., Meder, B., Kubanek, M., Hansen, F.G., Restrepo-Córdoba, M.A., Palomino-Doza, J., Ruiz-Guerrero, L., Sarquella-Brugada, G., Perez-Perez, A.J., Bermúdez-Jiménez, F.J., Ripoll-Vera, T., Rasmussen, T.B., Jansen, Mark, Sabater-Molina, M., Brunner, H.G., Elliot, P.M., and Garcia-Pavia, P.

Abstract

Contains fulltext : 287886.pdf (Publisher’s version ) (Open Access), BACKGROUND: Variants in myosin heavy chain 7 (MYH7) are responsible for disease in 1% to 5% of patients with dilated cardiomyopathy (DCM); however, the clinical characteristics and natural history of MYH7-related DCM are poorly described. OBJECTIVES: We sought to determine the phenotype and prognosis of MYH7-related DCM. We also evaluated the influence of variant location on phenotypic expression. METHODS: We studied clinical data from 147 individuals with DCM-causing MYH7 variants (47.6% female; 35.6 ± 19.2 years) recruited from 29 international centers. RESULTS: At initial evaluation, 106 (72.1%) patients had DCM (left ventricular ejection fraction: 34.5% ± 11.7%). Median follow-up was 4.5 years (IQR: 1.7-8.0 years), and 23.7% of carriers who were initially phenotype-negative developed DCM. Phenotypic expression by 40 and 60 years was 46% and 88%, respectively, with 18 patients (16%) first diagnosed at <18 years of age. Thirty-six percent of patients with DCM met imaging criteria for LV noncompaction. During follow-up, 28% showed left ventricular reverse remodeling. Incidence of adverse cardiac events among patients with DCM at 5 years was 11.6%, with 5 (4.6%) deaths caused by end-stage heart failure (ESHF) and 5 patients (4.6%) requiring heart transplantation. The major ventricular arrhythmia rate was low (1.0% and 2.1% at 5 years in patients with DCM and in those with LVEF of ≤35%, respectively). ESHF and major ventricular arrhythmia were significantly lower compared with LMNA-related DCM and similar to DCM caused by TTN truncating variants. CONCLUSIONS: MYH7-related DCM is characterized by early age of onset, high phenotypic expression, low left ventricular reverse remodeling, and frequent progression to ESHF. Heart failure complications predominate over ventricular arrhythmias, which are rare.

Published
2022

34. Blood-based biomarkers for the prediction of hypertrophic cardiomyopathy prognosis: a systematic review and meta-analysis

Author

Onderzoek Precision medicine, Genetica, Child Health, Circulatory Health, Team Medisch, Genetica Klinische Genetica, Jansen, Mark, Algül, Sila, Bosman, Laurens P, Michels, Michelle, van der Velden, Jolanda, de Boer, Rudolf A, van Tintelen, J Peter, Asselbergs, Folkert W, Baas, Annette F, Onderzoek Precision medicine, Genetica, Child Health, Circulatory Health, Team Medisch, Genetica Klinische Genetica, Jansen, Mark, Algül, Sila, Bosman, Laurens P, Michels, Michelle, van der Velden, Jolanda, de Boer, Rudolf A, van Tintelen, J Peter, Asselbergs, Folkert W, and Baas, Annette F

Published
2022

35. Automatic Identification of Patients With Unexplained Left Ventricular Hypertrophy in Electronic Health Record Data to Improve Targeted Treatment and Family Screening

Author

Onderzoek Precision medicine, Genetica Klinische Genetica, Team Medisch, Circulatory Health, Arts Assistenten Cardiologie, Sammani, Arjan, Jansen, Mark, de Vries, Nynke M, de Jonge, Nicolaas, Baas, Annette F, Te Riele, Anneline S J M, Asselbergs, Folkert W, Oerlemans, Marish I F J, Onderzoek Precision medicine, Genetica Klinische Genetica, Team Medisch, Circulatory Health, Arts Assistenten Cardiologie, Sammani, Arjan, Jansen, Mark, de Vries, Nynke M, de Jonge, Nicolaas, Baas, Annette F, Te Riele, Anneline S J M, Asselbergs, Folkert W, and Oerlemans, Marish I F J

Published
2022

42. Distinct Metabolomic Signatures in Preclinical and Obstructive Hypertrophic Cardiomyopathy

Author

Schuldt, Maike, primary, van Driel, Beau, additional, Algül, Sila, additional, Parbhudayal, Rahana Y., additional, Barge-Schaapveld, Daniela Q. C. M., additional, Güçlü, Ahmet, additional, Jansen, Mark, additional, Michels, Michelle, additional, Baas, Annette F., additional, van de Wiel, Mark A., additional, Nieuwdorp, Max, additional, Levin, Evgeni, additional, Germans, Tjeerd, additional, Jans, Judith J. M., additional, and van der Velden, Jolanda, additional

Published
2021
Full Text
View/download PDF

45. Interlaboratory comparison of angular-dependent photovoltaic device measurements: Results and impact on energy rating

Author

Riedel, Nicholas, Santamaria Lancia, Adrian Alejo, Plag, Fabian, Kröger, Ingo, Vogt, Malte R., Schinke, Carsten, Davidsen, Rasmus Schmidt, Amdemeskel, Mekbib Wubishet, Jansen, Mark J., Manshanden, Petra, Slooff, Lenneke H., Carr, Anna J., Bliss, Martin, Betts, Tom, Mayo, Mikel Ezquer, Jauregui, Iñigo Petrina, Balenzategui, José L., Roldan, Ruben, Bellenda, Giovanni, Caccivio, Mauro, Kräling, Ulli, Neuberger, Frank, Zirzow, Daniel, Crimmins, Jim, Robinson, Charles, King, Bruce, Teasdale, Wesley, Kedir, Cherif, Watts, John, Desharnais, Ryan, Poulsen, Peter Behrensdorff, Jakobsen, Michael Linde, Benatto, Gisele Alves dos Reis, Riedel, Nicholas, Santamaria Lancia, Adrian Alejo, Plag, Fabian, Kröger, Ingo, Vogt, Malte R., Schinke, Carsten, Davidsen, Rasmus Schmidt, Amdemeskel, Mekbib Wubishet, Jansen, Mark J., Manshanden, Petra, Slooff, Lenneke H., Carr, Anna J., Bliss, Martin, Betts, Tom, Mayo, Mikel Ezquer, Jauregui, Iñigo Petrina, Balenzategui, José L., Roldan, Ruben, Bellenda, Giovanni, Caccivio, Mauro, Kräling, Ulli, Neuberger, Frank, Zirzow, Daniel, Crimmins, Jim, Robinson, Charles, King, Bruce, Teasdale, Wesley, Kedir, Cherif, Watts, John, Desharnais, Ryan, Poulsen, Peter Behrensdorff, Jakobsen, Michael Linde, and Benatto, Gisele Alves dos Reis

Abstract

This paper presents the results from an extensive interlaboratory comparison of angular-dependent measurements on encapsulated photovoltaic (PV) cells. Twelve international laboratories measure the incident angle modifier of two unique PV devices. The absolute measurement agreement is ±2.0% to the weighted mean for angles of incidence (AOI) ≤ 65°, but from 70°–85° the range of measurement deviations increases rapidly from 2.5%–23%. The proficiency of the measurements is analyzed using the expanded uncertainties published by seven of the laboratories, and it is found that most of the angular-dependent measurements are reproducible for AOI ≤ 80°. However, at 85° one laboratory’s measurement do not agree to the weighted mean within the stated uncertainty, and measurement uncertainty as high as 16% is needed for the laboratories without uncertainty to be comparable. The poor agreement obtained at 85° indicates that the PV community should place minimal reliance on angular-dependent measurements made at this extreme angle until improvements can be demonstrated. The cloud-based Daidalos ray tracing model is used to simulate the angular-dependent losses of the mono-Si device and it is found that the simulation agrees to the median measurement within 0.6% for AOI ≤ 70° and within 1.4% for AOI ≤ 80°. Finally, the impact that the angular-dependent measurement deviations have on climate specific energy rating (CSER) is evaluated for the six climates described in the IEC 61853-4 standard. When one outlier measurement is excluded, the angular-dependent measurements reported in this work cause a 1.0%–1.8% range in CSER and a 1.0%–1.5% range in annual energy yield, depending on the climate.

Published
2021

46. Distinct metabolomic signatures in preclinical and obstructive hypertrophic cardiomyopathy

Author

Schuldt, Maike, van Driel, Beau, Algül, Sila, Parbhudayal, Rahana Y., Barge-Schaapveld, Daniela Q.C.M., Güçlü, Ahmet, Jansen, Mark, Michels, Michelle, Baas, Annette F., van de Wiel, Mark A., Nieuwdorp, Max, Levin, Evgeni, Germans, Tjeerd, Jans, Judith J.M., van der Velden, Jolanda, Schuldt, Maike, van Driel, Beau, Algül, Sila, Parbhudayal, Rahana Y., Barge-Schaapveld, Daniela Q.C.M., Güçlü, Ahmet, Jansen, Mark, Michels, Michelle, Baas, Annette F., van de Wiel, Mark A., Nieuwdorp, Max, Levin, Evgeni, Germans, Tjeerd, Jans, Judith J.M., and van der Velden, Jolanda

Abstract

Hypertrophic Cardiomyopathy (HCM) is a common inherited heart disease with poor risk prediction due to incomplete penetrance and a lack of clear genotype–phenotype correlations. Advanced imaging techniques have shown altered myocardial energetics already in preclinical gene variant carriers. To determine whether disturbed myocardial energetics with the potential to serve as biomarkers are also reflected in the serum metabolome, we analyzed the serum metabolome of asymptomatic carriers in comparison to healthy controls and obstructive HCM patients (HOCM). We performed non-quantitative direct-infusion high-resolution mass spectrometry-based untargeted metabolomics on serum from fasted asymptomatic gene variant carriers, symptomatic HOCM patients and healthy controls (n = 31, 14 and 9, respectively). Biomarker panels that discriminated the groups were identified by performing multivariate modeling with gradient-boosting classifiers. For all three group-wise comparisons we identified a panel of 30 serum metabolites that best discriminated the groups. These metabolite panels performed equally well as advanced cardiac imaging modalities in distinguishing the groups. Seven metabolites were found to be predictive in two different comparisons and may play an important role in defining the disease stage. This study reveals unique metabolic signatures in serum of preclinical carriers and HOCM patients that may potentially be used for HCM risk stratification and precision therapeutics.

Published
2021

47. Distinct Metabolomic Signatures in Preclinical and Obstructive Hypertrophic Cardiomyopathy

Author

Genetica Klinische Genetica, Circulatory Health, Genetica Sectie Metabole Diagnostiek, Child Health, Schuldt, Maike, van Driel, Beau, Algül, Sila, Parbhudayal, Rahana Y, Barge-Schaapveld, Daniela Q C M, Güçlü, Ahmet, Jansen, Mark, Michels, Michelle, Baas, Annette F, van de Wiel, Mark A, Nieuwdorp, Max, Levin, Evgeni, Germans, Tjeerd, Jans, Judith J M, van der Velden, Jolanda, Genetica Klinische Genetica, Circulatory Health, Genetica Sectie Metabole Diagnostiek, Child Health, Schuldt, Maike, van Driel, Beau, Algül, Sila, Parbhudayal, Rahana Y, Barge-Schaapveld, Daniela Q C M, Güçlü, Ahmet, Jansen, Mark, Michels, Michelle, Baas, Annette F, van de Wiel, Mark A, Nieuwdorp, Max, Levin, Evgeni, Germans, Tjeerd, Jans, Judith J M, and van der Velden, Jolanda

Published
2021

48. Applying copyright theory to secondary markets: an analysis of the future of 17 U.S.C. (section)109(a) pursuant to Costco Wholesale Corp. v. Omega S.A.

Author

Jansen, Mark

Subjects
Copyright infringement -- Laws, regulations and rules, Secondary market -- Intellectual property -- Laws, regulations and rules, Costco Wholesale Corp. v. Omega S.A. (131 S. Ct. 565 (2010)), Government regulation, Copyright Act of 1976 (17 U.S.C. 109(a))
Abstract

CONTENTS I. Introduction II. The Omega Case III. Background Information A. "Gray Market" Goods B. Legislative History C. Judicial History IV. Philosophical Justifications A. Creation B. Distribution V. Effects on [...], The U.S. Copyright Act grants copyright owners the exclusive right to distribute their copyrighted works. The first sale doctrine, codified in [section] 109(a) of the Copyright Act, curtails these distribution rights by exhausting the owner&apos;s exclusive right after the copyrighted item is placed in the stream of commerce. However, it is not clear whether the language used in the Act, "copies made under this title," is inclusive of copies manufactured abroad or limited to copies manufactured in the United States. The Supreme Court recently interpreted [section] 109(a) in Costco Wholesale Corp. v. Omega S.A.; yet, the Court&apos;s holding did little to clarify the ambiguity surrounding the application of the first sale doctrine. The Court&apos;s failure to resolve the issue has the potential to cause significant harm to the U.S. economy and eliminate the rights of consumers and small business. This Article suggests that the solution to determining whether the first sale doctrine is, in fact, applicable to copies manufactured abroad, is to incentivize Congress to amend [section] 109(a) and bring it into conformity with the true aims of copyright law--to promote knowledge via creation and distribution. In doing so, Congress should redraft this portion of the Act broadly to accommodate the domestic sale of copyrighted goods lawfully manufactured and sold abroad. This proposed expansion of the first sale doctrine will yield new discoveries and stimulate learning in accordance with the aims of copyright law.

Published
2011

Catalog

Books, media, physical & digital resources
See catalog results