[HTML][HTML] Hypertrophic cardiomyopathy and the myosin mesa: viewing an old disease in a new light
The sarcomere is an exquisitely designed apparatus that is capable of generating force,
which in the case of the heart results in the pumping of blood throughout the body. At the …
which in the case of the heart results in the pumping of blood throughout the body. At the …
The myosin family of mechanoenzymes: from mechanisms to therapeutic approaches
…, S Nag, A Spudich, KM Ruppel… - Annual review of …, 2020 - annualreviews.org
Myosins are among the most fascinating enzymes in biology. As extremely allosteric
chemomechanical molecular machines, myosins are involved in myriad pivotal cellular functions …
chemomechanical molecular machines, myosins are involved in myriad pivotal cellular functions …
Effects of hypertrophic and dilated cardiomyopathy mutations on power output by human β-cardiac myosin
Hypertrophic cardiomyopathy is the most frequently occurring inherited cardiovascular disease,
with a prevalence of more than one in 500 individuals worldwide. Genetically acquired …
with a prevalence of more than one in 500 individuals worldwide. Genetically acquired …
Deciphering the super relaxed state of human β-cardiac myosin and the mode of action of mavacamten from myosin molecules to muscle fibers
…, MM Morck, JG Seidman, KM Ruppel… - Proceedings of the …, 2018 - National Acad Sciences
Mutations in β-cardiac myosin, the predominant motor protein for human heart contraction,
can alter power output and cause cardiomyopathy. However, measurements of the intrinsic …
can alter power output and cause cardiomyopathy. However, measurements of the intrinsic …
Altered cardiac energetics and mitochondrial dysfunction in hypertrophic cardiomyopathy
Background: Hypertrophic cardiomyopathy (HCM) is a complex disease partly explained by
the effects of individual gene variants on sarcomeric protein biomechanics. At the cellular …
the effects of individual gene variants on sarcomeric protein biomechanics. At the cellular …
The myosin mesa and the basis of hypercontractility caused by hypertrophic cardiomyopathy mutations
…, MS Sunitha, S Sutton, KM Ruppel… - Nature structural & …, 2017 - nature.com
Hypertrophic cardiomyopathy (HCM) is primarily caused by mutations in β-cardiac myosin
and myosin-binding protein-C (MyBP-C). Changes in the contractile parameters of myosin …
and myosin-binding protein-C (MyBP-C). Changes in the contractile parameters of myosin …
SETD3 is an actin histidine methyltransferase that prevents primary dystocia
…, DV Trivedi, KM Ruppel, JG Vilches-Moure, KM Casey… - Nature, 2019 - nature.com
For more than 50 years, the methylation of mammalian actin at histidine 73 has been known
to occur 1 . Despite the pervasiveness of His73 methylation, which we find is conserved in …
to occur 1 . Despite the pervasiveness of His73 methylation, which we find is conserved in …
[PDF][PDF] Ensemble force changes that result from human cardiac myosin mutations and a small-molecule effector
T Aksel, EC Yu, S Sutton, KM Ruppel, JA Spudich - Cell reports, 2015 - cell.com
… Solid lines show the best Michaelis-Menten fits for the ATPase rates (rate = kcat[actin]/(Km+[actin]).
Dashed lines mark the Km and half maximal ATPase rates (0.5 kcat). Confidence …
Dashed lines mark the Km and half maximal ATPase rates (0.5 kcat). Confidence …
Enzymatic activities correlate with chimaeric substitutions at the actin-binding face of myosin
TQP Uyeda, KM Ruppel, JA Spudich - Nature, 1994 - nature.com
MYOSINS are a functionally divergent group of mechanochemical enzymes involved in
various motile activities in cells 1 . Despite a high degree of conservation in the amino-acid …
various motile activities in cells 1 . Despite a high degree of conservation in the amino-acid …
Multidimensional structure-function relationships in human β-cardiac myosin from population-scale genetic variation
…, MS Sunitha, RE Taylor, KM Ruppel… - Proceedings of the …, 2016 - National Acad Sciences
Myosin motors are the fundamental force-generating elements of muscle contraction.
Variation in the human β-cardiac myosin heavy chain gene (MYH7) can lead to hypertrophic …
Variation in the human β-cardiac myosin heavy chain gene (MYH7) can lead to hypertrophic …