English (United Kingdom)

https://curated-unify.zendy.io/wp-json/zendy-region/v1/featured_content/oa?rat=en

https://curated-unify.zendy.io/wp-json/zendy-region/v1/highlighted_journal/

Global: Zendy Plus annual

Presents the access of premium content as premium feature

Premium Content

Presents the keyphrase highlighting as premium feature

Keyphrase Highlighting

Presents the summarisation as premium feature

Summarisation

Insights

Presents the pdf analysis as premium feature

PDF Analysis

Presents the zaia usage as premium feature

ZAIA

Global: Zendy Plus monthly

Global: Zendy Tools annual

Global: Zendy Tools monthly

Global: Zendy Free Plan

Significance Familial hypertrophic cardiomyopathy is a major cause of sudden death in young athletes. The mouse model for heart disease showed that the severe R403Q mutation in α-cardiac myosin heavy chain leads to increased ATPase activity and in vitro motility. But the β-myosin heavy chain is the preferred isoform in all larger mammals, leading us to generate a transgenic R403Q rabbit model. In contrast to the mouse model, we find a significant reduction in actin motility by R403Q rabbit myosin. Even more importantly, single myofibrils with the mutant myosin in the environment of a native thick filament showed diminished kinetics, force, and power output, leading to the conclusion that loss-of-function is the initial cause of cardiac hypertrophy.

Hypertrophic cardiomyopathy R403Q mutation in rabbit β-myosin reduces contractile function at the molecular and myofibrillar levels