Search

Your search keyword '"Gunn, Jamie"' showing total 12 results
Start Over Author "Gunn, Jamie"
12 results on '"Gunn, Jamie"'

1. Kinetic and hydrodynamic regimes in multi-particle-collision dynamics of a one-dimensional fluid with thermal walls

Author

Lepri, Stefano, Ciraolo, Guido, Di Cintio, Pierfrancesco, Gunn, Jamie, and Livi, Roberto

Subjects
Condensed Matter - Statistical Mechanics
Abstract

We study the non-equilibrium steady-states of a one-dimensional ($1D1V$) fluid in a finite space region of length $L$. Particles interact among themselves by multi-particle collisions and are in contact with two thermal-wall heat reservoirs, located at the boundaries of the region. After an initial ballistic regime, we find a crossover from a normal (kinetic) transport regime to an anomalous (hydrodynamic) one, above a characteristic size $L_*$. We argue that $L_*$ is proportional to the cube of the collision time among particles. Motivated by the physics of emissive divertors in fusion plasma, we consider the more general case of thermal walls injecting particles with given average (non-thermal) velocity. For fast and relatively cold particles, short systems fail to establish local equilibrium and display non-Maxwellian distributions of velocities., Comment: Accepted for publication in Physical Review Research

Published
2021
Full Text
View/download PDF

2. ABCC9-related Intellectual disability Myopathy Syndrome is a KATP channelopathy with loss-of-function mutations in ABCC9

Author

Smeland, Marie F., McClenaghan, Conor, Roessler, Helen I., Savelberg, Sanne, Hansen, Geir Åsmund Myge, Hjellnes, Helene, Arntzen, Kjell Arne, Müller, Kai Ivar, Dybesland, Andreas Rosenberger, Harter, Theresa, Sala-Rabanal, Monica, Emfinger, Chris H., Huang, Yan, Singareddy, Soma S., Gunn, Jamie, Wozniak, David F., Kovacs, Attila, Massink, Maarten, Tessadori, Federico, Kamel, Sarah M., Bakkers, Jeroen, Remedi, Maria S., Van Ghelue, Marijke, Nichols, Colin G., and van Haaften, Gijs

Published
2019
Full Text
View/download PDF

5. ABCC9-related Intellectual disability Myopathy Syndrome is a KATP channelopathy with loss-of-function mutations in ABCC9

Author

Smeland, Marie F, McClenaghan, Conor, Roessler, Helen I, Savelberg, Sanne, Hansen, Geir Åsmund Myge, Hjellnes, Helene, Arntzen, Kjell Arne, Müller, Kai Ivar, Dybesland, Andreas Rosenberger, Harter, Theresa, Sala-Rabanal, Monica, Emfinger, Chris H, Huang, Yan, Singareddy, Soma S, Gunn, Jamie, Wozniak, David F, Kovacs, Attila, Massink, Maarten, Tessadori, Federico, Kamel, Sarah M, Bakkers, Jeroen, Remedi, Maria S, Van Ghelue, Marijke, Nichols, Colin G, van Haaften, Gijs, Smeland, Marie F, McClenaghan, Conor, Roessler, Helen I, Savelberg, Sanne, Hansen, Geir Åsmund Myge, Hjellnes, Helene, Arntzen, Kjell Arne, Müller, Kai Ivar, Dybesland, Andreas Rosenberger, Harter, Theresa, Sala-Rabanal, Monica, Emfinger, Chris H, Huang, Yan, Singareddy, Soma S, Gunn, Jamie, Wozniak, David F, Kovacs, Attila, Massink, Maarten, Tessadori, Federico, Kamel, Sarah M, Bakkers, Jeroen, Remedi, Maria S, Van Ghelue, Marijke, Nichols, Colin G, and van Haaften, Gijs

Abstract

Mutations in genes encoding KATP channel subunits have been reported for pancreatic disorders and Cantú syndrome. Here, we report a syndrome in six patients from two families with a consistent phenotype of mild intellectual disability, similar facies, myopathy, and cerebral white matter hyperintensities, with cardiac systolic dysfunction present in the two oldest patients. Patients are homozygous for a splice-site mutation in ABCC9 (c.1320 + 1 G > A), which encodes the sulfonylurea receptor 2 (SUR2) subunit of KATP channels. This mutation results in an in-frame deletion of exon 8, which results in non-functional KATP channels in recombinant assays. SUR2 loss-of-function causes fatigability and cardiac dysfunction in mice, and reduced activity, cardiac dysfunction and ventricular enlargement in zebrafish. We term this channelopathy resulting from loss-of-function of SUR2-containing KATP channels ABCC9-related Intellectual disability Myopathy Syndrome (AIMS). The phenotype differs from Cantú syndrome, which is caused by gain-of-function ABCC9 mutations, reflecting the opposing consequences of KATP loss- versus gain-of-function.

Published
2019

6. ABCC9-related Intellectual disability Myopathy Syndrome is a KATP channelopathy with loss-of-function mutations in ABCC9

Author

Genetica Groep Van Haaften, Genetica Sectie Genoomdiagnostiek, Child Health, Medische Fysiologie, Circulatory Health, CMM Sectie Genomics and Bioinformatics, Cancer, Smeland, Marie F, McClenaghan, Conor, Roessler, Helen I, Savelberg, Sanne, Hansen, Geir Åsmund Myge, Hjellnes, Helene, Arntzen, Kjell Arne, Müller, Kai Ivar, Dybesland, Andreas Rosenberger, Harter, Theresa, Sala-Rabanal, Monica, Emfinger, Chris H, Huang, Yan, Singareddy, Soma S, Gunn, Jamie, Wozniak, David F, Kovacs, Attila, Massink, Maarten, Tessadori, Federico, Kamel, Sarah M, Bakkers, Jeroen, Remedi, Maria S, Van Ghelue, Marijke, Nichols, Colin G, van Haaften, Gijs, Genetica Groep Van Haaften, Genetica Sectie Genoomdiagnostiek, Child Health, Medische Fysiologie, Circulatory Health, CMM Sectie Genomics and Bioinformatics, Cancer, Smeland, Marie F, McClenaghan, Conor, Roessler, Helen I, Savelberg, Sanne, Hansen, Geir Åsmund Myge, Hjellnes, Helene, Arntzen, Kjell Arne, Müller, Kai Ivar, Dybesland, Andreas Rosenberger, Harter, Theresa, Sala-Rabanal, Monica, Emfinger, Chris H, Huang, Yan, Singareddy, Soma S, Gunn, Jamie, Wozniak, David F, Kovacs, Attila, Massink, Maarten, Tessadori, Federico, Kamel, Sarah M, Bakkers, Jeroen, Remedi, Maria S, Van Ghelue, Marijke, Nichols, Colin G, and van Haaften, Gijs

Published
2019

9. Radio-Frequency Sheaths Physics: Experimental Characterization on Tore Supra and Related Self-Consistent Modeling.

Author

Jacquot, Jonathan, Milanesio, Daniele, Colas, Laurent, Corre, Yann, Goniche, Marc, Gunn, Jamie, Heuraux, Stéphane, and Kubic, Martin

Subjects
RADIO frequency, PLASMA sheaths, FARADAY cage, ICR heating, HEAT flux, ANTENNAS in plasma, CANTILEVERS, TOKAMAKS
Abstract

During the 2011 experimental campaign, one of the three ion cyclotron resonance heating (ICRH) antennas in the Tore Supra (TS) tokamak was equipped with a new type of Faraday screen (FS). The new design aimed at minimizing RF sheaths as well as increasing the heat exhaust capability of the actively cooled screen. It proved to be inefficient for attenuating the RF-sheaths on the screen itself on the contrary to the heat exhaust concept that allowed operation despite higher heat fluxes on the antenna. In parallel, a new approach has been proposed to model self-consistently RF sheaths: the SSWICH (Self-consistent Sheaths and Waves for IC Heating) code. Simulations results from SSWICH coupled with the TOPICA antenna code were able to reproduce the difference between the two FS designs and part of the spatial pattern of heat loads and floating potential. The poloidal pattern is a reliable result that mainly depends on the electrical design of the antenna while the radial pattern is on the contrary highly sensitive to loosely constrained parameters such as perpendicular conductivity that generates a DC current circulation from the the private region inside the antenna limiters to the free SOL outside these limiters. Moreover the cantilevered bars seem to be the element in the design of the new screen that enhanced RF sheaths. [ABSTRACT FROM AUTHOR]

Published
2014
Full Text
View/download PDF

11. ABCC9-related Intellectual disability Myopathy Syndrome is a KATP channelopathy with loss-of-function mutations in ABCC9.

Author

Smeland, Marie F., McClenaghan, Conor, Roessler, Helen I., Savelberg, Sanne, Hansen, Geir Åsmund Myge, Hjellnes, Helene, Arntzen, Kjell Arne, Müller, Kai Ivar, Dybesland, Andreas Rosenberger, Harter, Theresa, Sala-Rabanal, Monica, Emfinger, Chris H., Huang, Yan, Singareddy, Soma S., Gunn, Jamie, Wozniak, David F., Kovacs, Attila, Massink, Maarten, Tessadori, Federico, and Kamel, Sarah M.

Subjects
INTELLECTUAL disabilities, DISABILITIES, MUSCLE diseases, RECESSIVE genes, SYNDROMES, GAIN-of-function mutations, ZEBRA danio
Abstract

Mutations in genes encoding KATP channel subunits have been reported for pancreatic disorders and Cantú syndrome. Here, we report a syndrome in six patients from two families with a consistent phenotype of mild intellectual disability, similar facies, myopathy, and cerebral white matter hyperintensities, with cardiac systolic dysfunction present in the two oldest patients. Patients are homozygous for a splice-site mutation in ABCC9 (c.1320 + 1 G > A), which encodes the sulfonylurea receptor 2 (SUR2) subunit of KATP channels. This mutation results in an in-frame deletion of exon 8, which results in non-functional KATP channels in recombinant assays. SUR2 loss-of-function causes fatigability and cardiac dysfunction in mice, and reduced activity, cardiac dysfunction and ventricular enlargement in zebrafish. We term this channelopathy resulting from loss-of-function of SUR2-containing KATP channels ABCC9-related Intellectual disability Myopathy Syndrome (AIMS). The phenotype differs from Cantú syndrome, which is caused by gain-of-function ABCC9 mutations, reflecting the opposing consequences of KATP loss- versus gain-of-function. ABCC9 encodes the SUR2 subunit of KATP channels and dominant genetic variants in ABCC9 have been associated with cardiac phenotypes. Here, the authors report recessive ABCC9 mutations in individuals with mild intellectual disability, myopathy and cardiac systolic dysfunction which is associated with loss of KATP channel function. [ABSTRACT FROM AUTHOR]

Published
2019
Full Text
View/download PDF

12. Langmuir probes design for the actively cooled divertor baffle in WEST

Author

J. P. Gunn, T. Batal, S. Salasca, Jean-Yves Pascal, Giuseppe Di Gironimo, Yann Corre, Maurizio Cacace, Cacace, Maurizio, Batal, Tristan, Corre, Yann, Di Gironimo, Giuseppe, Gunn, Jamie P., Pascal, Jean-Yve, and Salasca, Sophie

Subjects
Tokamak, Materials science, Mechanical Engineering, Nuclear engineering, Divertor, Baffle, Plasma, Tore Supra, law.invention, symbols.namesake, Nuclear magnetic resonance, Nuclear Energy and Engineering, law, symbols, Limiter, Langmuir probe, Electron temperature, General Materials Science, WEST, Tore Supra, Diagnostics, Divertor, Baffle, Langmuir probes, Civil and Structural Engineering
Abstract

The WEST project (W-Environment in Steady-State Tokamak) aims to transform the Tore Supra limiter configuration to an x-point divertor, providing a test bed for ITER-like plasma-facing components (actively cooled W monoblocs) under high heat flux, steady-state plasma irradiation. The lower divertor includes an actively cooled, W-coated CuCrZr baffle to provide neutral compression and improve particle exhaust. As part of the new diagnostic equipment of Tore Supra within WEST project, a set of Langmuir probes will find place on the baffle in order to provide plasma flux and electron temperature measurements for physics studies and real-time machine protection functions during steady-state discharges. On the baffle top surface, irradiation coming from the plasma, energetic ripple-ions losses, photons and energetic neutrals from charge exchange reactions produce power fluxes up to 3 MW/m2, representing a challenge for the Langmuir probes operating conditions. In this paper Copper–Chrome–Zirconium (CuCrZr) cylindrical probe concept design is proposed. Finite element thermo-mechanical analysis (FEA) confirmed the consistency of this solution under the steady-state plasma condition in the worst case (highest thermal load).

Published
2015

Catalog

Books, media, physical & digital resources
See catalog results