Normal passive viscoelasticity but abnormal myofibrillar force generation in human hypertrophic cardiomyopathy

Anita C. Hoskins, Adam Jacques, Sonya C. Bardswell, William J. McKenna, Victor Tsang, Cristobal G. dos Remedios, Elisabeth Ehler, Kim Adams, Shapour Jalilzadeh, Metin Avkiran, Hugh Watkins, Charles Redwood, Steven B. Marston, Jonathan C. Kentish

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60 Citations (Scopus)


Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular hypertrophy, increased ventricular stiffness and impaired diastolic filling. We investigated to what extent myocardial functional defects can be explained by alterations in the passive and active properties of human cardiac myofibrils. Skinned ventricular myocytes were prepared from patients with obstructive HCM (two patients with MYBPC3 mutations, one with a MYH7 mutation, and three with no mutation in either gene) and from four donors. Passive stiffness, viscous properties, and titin isoform expression were similar in HCM myocytes and donor myocytes. Maximal Ca2+-activated force was much lower in HCM myocytes (14 +/- 1 kN/m(2)) than in donor myocytes (23 +/- 3 kN/m(2); P
Original languageEnglish
Pages (from-to)737 - 745
Number of pages9
JournalJournal of Molecular and Cellular Cardiology
Issue number5
Publication statusPublished - Nov 2010

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