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

Targets for therapy in sarcomeric cardiomyopathies. Cardiovasc Res (2015) 0.96

The genetic landscape of cardiomyopathy and its role in heart failure. Cell Metab (2015) 0.92

Research priorities in sarcomeric cardiomyopathies. Cardiovasc Res (2015) 0.86

Modelling sarcomeric cardiomyopathies in the dish: from human heart samples to iPSC cardiomyocytes. Cardiovasc Res (2015) 0.85

Molecular effects of the myosin activator omecamtiv mecarbil on contractile properties of skinned myocardium lacking cardiac myosin binding protein-C. J Mol Cell Cardiol (2015) 0.84

Isolation and Mechanical Measurements of Myofibrils from Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes. Stem Cell Reports (2016) 0.80

Liver Kinase B1 complex acts as a novel modifier of myofilament function and localizes to the Z-disk in cardiac myocytes. Arch Biochem Biophys (2016) 0.79

Sex dimorphisms of crossbridge cycling kinetics in transgenic hypertrophic cardiomyopathy mice. Am J Physiol Heart Circ Physiol (2016) 0.77

Familial hypertrophic cardiomyopathy: functional variance among individual cardiomyocytes as a trigger of FHC-phenotype development. Front Physiol (2014) 0.76

Myocardial energy depletion and dynamic systolic dysfunction in hypertrophic cardiomyopathy. Nat Rev Cardiol (2016) 0.75

Modulating Beta-Cardiac Myosin Function at the Molecular and Tissue Levels. Front Physiol (2017) 0.75

Emerging pharmacologic and structural therapies for hypertrophic cardiomyopathy. Heart Fail Rev (2017) 0.75

Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation (2006) 9.44

A molecular basis for familial hypertrophic cardiomyopathy: a beta cardiac myosin heavy chain gene missense mutation. Cell (1990) 5.77

Effect of Ca2+ on cross-bridge turnover kinetics in skinned single rabbit psoas fibers: implications for regulation of muscle contraction. Proc Natl Acad Sci U S A (1988) 5.09

Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outflow tract obstruction. Circulation (2006) 3.82

Late sodium current inhibition reverses electromechanical dysfunction in human hypertrophic cardiomyopathy. Circulation (2012) 2.78

Determination of left ventricular mass by echocardiography in a normal population: effect of age and sex in addition to body size. Mayo Clin Proc (1994) 2.66

Pathogenesis of diverse clinical and pathological phenotypes in hypertrophic cardiomyopathy. Lancet (2000) 2.57

Single-molecule mechanics of R403Q cardiac myosin isolated from the mouse model of familial hypertrophic cardiomyopathy. Circ Res (2000) 2.35

Oxidation of myofibrillar proteins in human heart failure. J Am Coll Cardiol (2011) 2.13

Skeletal muscle expression and abnormal function of beta-myosin in hypertrophic cardiomyopathy. J Clin Invest (1993) 2.04

Protein kinase A does not alter economy of force maintenance in skinned rat cardiac trabeculae. Circ Res (1995) 1.92

Heterologous expression of a cardiomyopathic myosin that is defective in its actin interaction. J Biol Chem (1994) 1.77

Abnormal contractile properties of muscle fibers expressing beta-myosin heavy chain gene mutations in patients with hypertrophic cardiomyopathy. J Clin Invest (1995) 1.75

Progress with genetic cardiomyopathies: screening, counseling, and testing in dilated, hypertrophic, and arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circ Heart Fail (2009) 1.74

Structural interpretation of the mutations in the beta-cardiac myosin that have been implicated in familial hypertrophic cardiomyopathy. Proc Natl Acad Sci U S A (1995) 1.70

Myosin isoenzymes in normal and hypertrophied human ventricular myocardium. Circ Res (1983) 1.67

Myofibrillar ATPase activity and mechanical performance of skinned fibres from rabbit psoas muscle. J Physiol (1994) 1.64

Sarcomeric determinants of striated muscle relaxation kinetics. Pflugers Arch (2004) 1.63

Relaxation kinetics following sudden Ca(2+) reduction in single myofibrils from skeletal muscle. Biophys J (2002) 1.54

Species- and age-dependent changes in the relative amounts of cardiac myosin isoenzymes in mammals. Dev Biol (1981) 1.53

Allele-specific gene expression differences in humans. Hum Mol Genet (2004) 1.51

Modifier genes for hypertrophic cardiomyopathy. Curr Opin Cardiol (2002) 1.47

Perturbed length-dependent activation in human hypertrophic cardiomyopathy with missense sarcomeric gene mutations. Circ Res (2013) 1.44

R403Q and L908V mutant beta-cardiac myosin from patients with familial hypertrophic cardiomyopathy exhibit enhanced mechanical performance at the single molecule level. J Muscle Res Cell Motil (2000) 1.44

Force kinetics and individual sarcomere dynamics in cardiac myofibrils after rapid ca(2+) changes. Biophys J (2002) 1.41

The in vitro motility activity of beta-cardiac myosin depends on the nature of the beta-myosin heavy chain gene mutation in hypertrophic cardiomyopathy. J Muscle Res Cell Motil (1997) 1.40

Functional analysis of the mutations in the human cardiac beta-myosin that are responsible for familial hypertrophic cardiomyopathy. Implication for the clinical outcome. J Clin Invest (1996) 1.35

Functional effects of the hypertrophic cardiomyopathy R403Q mutation are different in an alpha- or beta-myosin heavy chain backbone. J Biol Chem (2008) 1.31

Altered crossbridge kinetics in the alphaMHC403/+ mouse model of familial hypertrophic cardiomyopathy. Circ Res (1999) 1.28

Functional consequences of mutations in the smooth muscle myosin heavy chain at sites implicated in familial hypertrophic cardiomyopathy. J Biol Chem (2000) 1.20

Impact of temperature on cross-bridge cycling kinetics in rat myocardium. J Physiol (2007) 1.19

The familial hypertrophic cardiomyopathy-associated myosin mutation R403Q accelerates tension generation and relaxation of human cardiac myofibrils. J Physiol (2008) 1.14

Tension generation and relaxation in single myofibrils from human atrial and ventricular myocardium. Pflugers Arch (2006) 1.12

Functional analysis of myosin mutations that cause familial hypertrophic cardiomyopathy. Biophys J (1998) 1.04

Point mutations in human beta cardiac myosin heavy chain have differential effects on sarcomeric structure and assembly: an ATP binding site change disrupts both thick and thin filaments, whereas hypertrophic cardiomyopathy mutations display normal assembly. J Cell Biol (1997) 1.01

Unequal expression of NF1 alleles. Nat Genet (1994) 0.99

Unequal allelic expression of wild-type and mutated β-myosin in familial hypertrophic cardiomyopathy. Basic Res Cardiol (2011) 0.98

Variability in the ratio of mutant to wildtype myosin heavy chain present in the soleus muscle of patients with familial hypertrophic cardiomyopathy. A new approach for the quantification of mutant to wildtype protein. FEBS Lett (1999) 0.97

Gene-specific increase in the energetic cost of contraction in hypertrophic cardiomyopathy caused by thick filament mutations. Cardiovasc Res (2014) 0.94

Mutations in MYH7 reduce the force generating capacity of sarcomeres in human familial hypertrophic cardiomyopathy. Cardiovasc Res (2013) 0.94

Effects of chronic atrial fibrillation on active and passive force generation in human atrial myofibrils. Circ Res (2010) 0.94

Myocardial contraction is 5-fold more economical in ventricular than in atrial human tissue. Cardiovasc Res (2005) 0.93

The R403Q myosin mutation implicated in familial hypertrophic cardiomyopathy causes disorder at the actomyosin interface. PLoS One (2007) 0.90

Familial hypertrophic cardiomyopathy: functional effects of myosin mutation R723G in cardiomyocytes. J Mol Cell Cardiol (2013) 0.89

Transgenic mouse α- and β-cardiac myosins containing the R403Q mutation show isoform-dependent transient kinetic differences. J Biol Chem (2013) 0.89

Isometric tension and mutant myosin heavy chain content in single skeletal myofibers from hypertrophic cardiomyopathy patients. J Mol Cell Cardiol (1997) 0.88

Biological, biochemical, and kinetic effects of mutations of the cardiomyopathy loop of Dictyostelium myosin II: importance of ALA400. J Biol Chem (2005) 0.85

Quantification of mutant versus wild-type myosin in human muscle biopsies using nano-LC/ESI-MS. Anal Chem (2007) 0.80

Nebulette knockout mice have normal cardiac function, but show Z-line widening and up-regulation of cardiac stress markers. Cardiovasc Res (2015) 0.76

The Relaxation Properties of Myofibrils Are Compromised by Amino Acids that Stabilize α-Tropomyosin. Biophys J (2017) 0.75

Impact of Genotype on the Occurrence of Atrial Fibrillation in Patients With Hypertrophic Cardiomyopathy. Am J Cardiol (2016) 0.75