Search Results - "Ruppel, M."
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The myosin mesa and the basis of hypercontractility caused by hypertrophic cardiomyopathy mutations
Published in Nature structural & molecular biology (01-06-2017)“…A working model for β-cardiac myosin in the sequestered state and binding assays reveal interactions between the myosin head and tail that are disrupted by…”
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Altered Cardiac Energetics and Mitochondrial Dysfunction in Hypertrophic Cardiomyopathy
Published in Circulation (New York, N.Y.) (23-11-2021)“…Hypertrophic cardiomyopathy (HCM) is a complex disease partly explained by the effects of individual gene variants on sarcomeric protein biomechanics. At the…”
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Hypertrophic cardiomyopathy: Mutations to mechanisms to therapies
Published in Frontiers in physiology (26-09-2022)“…Hypertrophic cardiomyopathy (HCM) affects more than 1 in 500 people in the general population with an extensive burden of morbidity in the form of arrhythmia,…”
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β-Cardiac myosin hypertrophic cardiomyopathy mutations release sequestered heads and increase enzymatic activity
Published in Nature communications (18-06-2019)“…Hypertrophic cardiomyopathy (HCM) affects 1 in 500 people and leads to hyper-contractility of the heart. Nearly 40 percent of HCM-causing mutations are found…”
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5
Cryo-EM structure of the folded-back state of human β-cardiac myosin
Published in Nature communications (31-05-2023)“…To save energy and precisely regulate cardiac contractility, cardiac muscle myosin heads are sequestered in an ‘off’ state that can be converted to an ‘on’…”
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6
Ensemble Force Changes that Result from Human Cardiac Myosin Mutations and a Small-Molecule Effector
Published in Cell reports (Cambridge) (12-05-2015)“…Cardiomyopathies due to mutations in human β-cardiac myosin are a significant cause of heart failure, sudden death, and arrhythmia. To understand the…”
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7
Direct aerosol chemical composition measurements to evaluate the physicochemical differences between controlled sea spray aerosol generation schemes
Published in Atmospheric measurement techniques (06-11-2014)“…Controlled laboratory studies of the physical and chemical properties of sea spray aerosol (SSA) must be under-pinned by a physically and chemically accurate…”
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8
Hypertrophic cardiomyopathy and the myosin mesa: viewing an old disease in a new light
Published in Biophysical reviews (01-02-2018)“…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…”
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A Marine Aerosol Reference Tank system as a breaking wave analogue for the production of foam and sea-spray aerosols
Published in Atmospheric measurement techniques (30-04-2013)“…In order to better understand the processes governing the production of marine aerosols a repeatable, controlled method for their generation is required. The…”
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Increase in elemental carbon values between 1970 and 2004 observed in a 300-year ice core from Holtedahlfonna (Svalbard)
Published in Atmospheric chemistry and physics (30-10-2014)“…Black carbon (BC) is a light-absorbing particle that warms the atmosphere–Earth system. The climate effects of BC are amplified in the Arctic, where its…”
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11
The effect of the microscopic and nanoscale structure on bone fragility
Published in Osteoporosis international (01-09-2008)“…Summary Bone mineral density is the gold-standard for assessing bone quantity and diagnosing osteoporosis. Although bone mineral density measurements assess…”
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Hypertrophic cardiomyopathy mutations in the pliant and light chain-binding regions of the lever arm of human β-cardiac myosin have divergent effects on myosin function
Published in eLife (29-06-2022)“…Mutations in the lever arm of β-cardiac myosin are a frequent cause of hypertrophic cardiomyopathy, a disease characterized by hypercontractility and eventual…”
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13
Do contemporary (1980–2015) emissions determine the elemental carbon deposition trend at Holtedahlfonna glacier, Svalbard?
Published in Atmospheric chemistry and physics (26-10-2017)“…The climate impact of black carbon (BC) is notably amplified in the Arctic by its deposition, which causes albedo decrease and subsequent earlier snow and ice…”
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14
Effects of troponin T cardiomyopathy mutations on the calcium sensitivity of the regulated thin filament and the actomyosin cross-bridge kinetics of human β-cardiac myosin
Published in PloS one (18-12-2013)“…Hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM) lead to significant cardiovascular morbidity and mortality worldwide. Mutations in the genes…”
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15
The complex composition of organic aerosols emitted during burning varies between Arctic and boreal peat
Published in Communications earth & environment (01-12-2024)“…Abstract Peatlands in the northern hemisphere are a major carbon storage but face an increased risk of wildfires due to climate change leading to large-scale…”
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Climatic impacts on an Arctic lake since 1300 AD: a multi-proxy lake sediment reconstruction from Prins Karls Forland, Svalbard
Published in Journal of paleolimnology (01-03-2023)“…On the remote Arctic archipelago of Svalbard, there is increasing evidence of environmental impacts from climate change. The analysis of lake sedimentary…”
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17
Polystyrene nanoparticles with tunable interfaces and softness
Published in Polymer (Guilford) (14-01-2014)“…Polystyrene nanoparticles, cross-linked with divinylbenzene (PS-(DVB) sub(x)) and having radii, R sub(p) [< or =] 10 nm, have been synthesized using batch and…”
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Human immunoglobulin gene allelic variation impacts germline-targeting vaccine priming
Published in npj vaccines (11-03-2024)“…Vaccine priming immunogens that activate germline precursors for broadly neutralizing antibodies (bnAbs) have promise for development of precision vaccines…”
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Controlling load-dependent kinetics of β-cardiac myosin at the single-molecule level
Published in Nature structural & molecular biology (01-06-2018)“…Concepts in molecular tension sensing in biology are growing and have their origins in studies of muscle contraction. In the heart muscle, a key parameter of…”
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Deciphering the super relaxed state of human β-cardiac myosin and the mode of action of mavacamten from myosin molecules to muscle fibers
Published in Proceedings of the National Academy of Sciences - PNAS (28-08-2018)“…Mutations in β-cardiac myosin, the predominant motor protein for human heart contraction, can alter power output and cause cardiomyopathy. However,…”
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