In vivo cardiac myosin binding protein C gene transfer rescues myofilament contractile dysfunction in cardiac myosin binding protein C null mice.

ABSTRACT:

Background

Decreased expression of cardiac myosin binding protein C (cMyBPC) in the heart has been implicated as a consequence of mutations in cMyBPC that lead to abnormal contractile function at the myofilament level, thereby contributing to the development of hypertrophic cardiomyopathy in humans. It has not been established whether increasing the levels of cMyBPC in the intact heart can improve myofilament and in vivo contractile function and attenuate maladaptive remodeling processes because of reduced levels of cMyBPC.

Methods and results

We performed in vivo gene transfer of cMyBPC by direct injection into the myocardium of cMyBPC-deficient (cMyBPC(-/-)) mice, and mechanical experiments were conducted on skinned myocardium isolated from cMyBPC(-/-) hearts 21 days and 2

SUBMITTER: Merkulov S 

PROVIDER: S-EPMC4860813 | biostudies-literature | 2012 Sep

REPOSITORIES: biostudies-literature