Faster cross-bridge detachment and increased tension cost in human hypertrophic cardiomyopathy with the R403Q MYH7 mutation

E.R. Witjas-Paalberends; C. Ferrara; B. Scellini; N. Piroddi; J. Montag; C. Tesi; G.J.M. Stienen; M. Michels; C.Y. Ho; T. Kraft; C. Poggesi; J. van der Velden

doi:10.1113/jphysiol.2014.274571

Faster cross-bridge detachment and increased tension cost in human hypertrophic cardiomyopathy with the R403Q MYH7 mutation

E.R. Witjas-Paalberends, C. Ferrara, B. Scellini, N. Piroddi, J. Montag, C. Tesi, G.J.M. Stienen, M. Michels, C.Y. Ho, T. Kraft, C. Poggesi, J. van der Velden

Physics of Living Systems

Research output: Contribution to Journal › Article › Academic › peer-review

Abstract

The first mutation associated with hypertrophic cardiomyopathy (HCM) is the R403Q mutation in the gene encoding β-myosin heavy chain (β-MyHC). R403Q locates in the globular head of myosin (S1), responsible for interaction with actin, and thus motor function of myosin. Increased cross-bridge relaxation kinetics caused by the R403Q mutation might underlie increased energetic cost of tension generation; however, direct evidence is absent. Here we studied to what extent cross-bridge kinetics and energetics are related in single cardiac myofibrils and multicellular cardiac muscle strips of three HCM patients with the R403Q mutation and nine sarcomere mutation-negative HCM patients (HCM

Original language	English
Pages (from-to)	3257-3272
Journal	Journal of Physiology - London
Volume	592
Issue number	15
DOIs	https://doi.org/10.1113/jphysiol.2014.274571
Publication status	Published - 2014

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Witjas-Paalberends, E. R., Ferrara, C., Scellini, B., Piroddi, N., Montag, J., Tesi, C., Stienen, G. J. M., Michels, M., Ho, C. Y., Kraft, T., Poggesi, C., & van der Velden, J. (2014). Faster cross-bridge detachment and increased tension cost in human hypertrophic cardiomyopathy with the R403Q MYH7 mutation. Journal of Physiology - London, 592(15), 3257-3272. https://doi.org/10.1113/jphysiol.2014.274571

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title = "Faster cross-bridge detachment and increased tension cost in human hypertrophic cardiomyopathy with the R403Q MYH7 mutation",

abstract = "The first mutation associated with hypertrophic cardiomyopathy (HCM) is the R403Q mutation in the gene encoding β-myosin heavy chain (β-MyHC). R403Q locates in the globular head of myosin (S1), responsible for interaction with actin, and thus motor function of myosin. Increased cross-bridge relaxation kinetics caused by the R403Q mutation might underlie increased energetic cost of tension generation; however, direct evidence is absent. Here we studied to what extent cross-bridge kinetics and energetics are related in single cardiac myofibrils and multicellular cardiac muscle strips of three HCM patients with the R403Q mutation and nine sarcomere mutation-negative HCM patients (HCM",

author = "E.R. Witjas-Paalberends and C. Ferrara and B. Scellini and N. Piroddi and J. Montag and C. Tesi and G.J.M. Stienen and M. Michels and C.Y. Ho and T. Kraft and C. Poggesi and \{van der Velden\}, J.",

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doi = "10.1113/jphysiol.2014.274571",

language = "English",

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Witjas-Paalberends, ER, Ferrara, C, Scellini, B, Piroddi, N, Montag, J, Tesi, C, Stienen, GJM, Michels, M, Ho, CY, Kraft, T, Poggesi, C & van der Velden, J 2014, 'Faster cross-bridge detachment and increased tension cost in human hypertrophic cardiomyopathy with the R403Q MYH7 mutation', Journal of Physiology - London, vol. 592, no. 15, pp. 3257-3272. https://doi.org/10.1113/jphysiol.2014.274571

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T1 - Faster cross-bridge detachment and increased tension cost in human hypertrophic cardiomyopathy with the R403Q MYH7 mutation

AU - Witjas-Paalberends, E.R.

AU - Ferrara, C.

AU - Scellini, B.

AU - Piroddi, N.

AU - Montag, J.

AU - Tesi, C.

AU - Stienen, G.J.M.

AU - Michels, M.

AU - Ho, C.Y.

AU - Kraft, T.

AU - Poggesi, C.

AU - van der Velden, J.

PY - 2014

Y1 - 2014

N2 - The first mutation associated with hypertrophic cardiomyopathy (HCM) is the R403Q mutation in the gene encoding β-myosin heavy chain (β-MyHC). R403Q locates in the globular head of myosin (S1), responsible for interaction with actin, and thus motor function of myosin. Increased cross-bridge relaxation kinetics caused by the R403Q mutation might underlie increased energetic cost of tension generation; however, direct evidence is absent. Here we studied to what extent cross-bridge kinetics and energetics are related in single cardiac myofibrils and multicellular cardiac muscle strips of three HCM patients with the R403Q mutation and nine sarcomere mutation-negative HCM patients (HCM

AB - The first mutation associated with hypertrophic cardiomyopathy (HCM) is the R403Q mutation in the gene encoding β-myosin heavy chain (β-MyHC). R403Q locates in the globular head of myosin (S1), responsible for interaction with actin, and thus motor function of myosin. Increased cross-bridge relaxation kinetics caused by the R403Q mutation might underlie increased energetic cost of tension generation; however, direct evidence is absent. Here we studied to what extent cross-bridge kinetics and energetics are related in single cardiac myofibrils and multicellular cardiac muscle strips of three HCM patients with the R403Q mutation and nine sarcomere mutation-negative HCM patients (HCM

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DO - 10.1113/jphysiol.2014.274571

M3 - Article

SN - 0022-3751

VL - 592

SP - 3257

EP - 3272

JO - Journal of Physiology - London

JF - Journal of Physiology - London

IS - 15

ER -

Faster cross-bridge detachment and increased tension cost in human hypertrophic cardiomyopathy with the R403Q MYH7 mutation

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