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Introduction
Haemophilia A (HA) and haemophilia B (HB) are X‐linked recessive diseases, caused by a large number of pathogenic variants in the F8 and F9 genes. With the exception of introns 22 and 1 inversions which are frequent in severe HA cases, about 2000 unique variants in F8 and 1000 in F9 have been described in databases and their recurrence remains limited.
Aim and methods
During routine...
A very Compact High Power Microwave system is developed at CEA Gramat. The 400 kV–85 ns square pulse generation is provided by a compact and innovative PFN-Marx allowing a 100 pps repetition rate. Its low inductance design provides a very fast rise time and its U-shape output agrees with severe geometry constraints. This Marx is charged by a compact 45 kV capacitor charger, based on double resonance...
Load Current Multiplier (LCM) continues to be successfully applied on Zebra at UNR. Here we consider further, expanded applications of LCM. Time-shaping of magnetic pressures on pulsed facilities is useful for such a number of High Energy Density Physics (HEDP) applications such as dynamic material properties studies1,2, high gain ICF research3 and instability growth studies4. The Dynamic LCM (DLCM)...
SPHINX is a microsecond linear transformer driver (LTD), used essentially for implosion of Z-pinch loads in direct drive mode. It can deliver a 6MA current pulse within 800ns into a Z-pinch load. The Dynamic Load Current Multiplier (DLCM) concept enables the current pulse to be modified by increasing its amplitude while reducing its rise time before being delivered to the load. This compact system...
SPHINX is a microsecond LTD driver, used essentially for implosion of Z-pinch loads in direct drive mode. It can deliver a 6MA current pulse within 800ns into a Z-pinch load1. The Dynamic Load Current Multiplier (DLCM) concept enables the current pulse to be modified by increasing its amplitude while reducing its rise time before being delivered to the load2. This compact system is made up of concentric...
SPHINX is a 6MA, 1-μs Linear Transformer Driver operated by the CEA Gramat (France) and primarily used for imploding Z-pinch loads for radiation effects studies. A method for performing magnetic ramp compression experiments, without modifying the generator operation scheme, is developed using a compact pulse shaping system. This system, a Dynamic Load Current Multiplier (DLCM), is inserted in vacuum...
X-ray spectroscopy is a key diagnostic for characterizing plasma radiation Sources. A wide range of spectroscopic techniques is used on the Sphinx machine to study the spectral emission from aluminum and argon z-pinch loads. Depending on the desired time or spectral resolution, either dispersive crystal-based, time-integrated and time-resolved methods or non-dispersive techniques, using a combination...
An all fiber 1550 nm heterodyne velocimeter (HetV) has been developed at CEA Gramat to measure thermal stress waves on samples irradiated by Sphinx Z-pinch source. Previously, wave's velocities were measured by VISAR, for which the setup and adjustement procedures were heavy compared to those of the all fiber HetV. For both diagnostics, the low velocity and fast rise time of the mechanical strain...
In this paper, we present recent analysis that allowed us to explain the full history of the temperature pulse shape and highlights the importance of the interaction between the axial plasma jet and the hohlraum wall in expansion, which artificially increases the temperature seen by diagnostics. Experimental/numerical results confrontation will be shown. Conclusion on this kind of vaccum hohlraum...
The SPHINX machine is a 6 MA, 1 μs driver based on the LTD technology, used for Z-pinch experiments. The main loads are cylindrical wire arrays which appear to be, despite the long implosion time of Sphinx driver, efficient plasma radiation sources especially with the use of multi-microsecond prepulse technique. Recently, new configurations have been studied : radial wire arrays to investigate the...
Summary form only given. The Sphinx machine1 has been used to implode cylindrical Z- pinches to produce k-shell emission from Aluminum wire arrays and Argon gas puffs. Despite the long implosion time of Sphinx driver, efficient plasma radiation sources were obtained especially with the use of multi microsecond prepulse technique for wire array loads4. However, the interest of such k-shell emitter...
Summary form only given. The Sphinx machine (F. Lassalle et al., 2008) is a 6 MA, 1 ?s driver based on the LTD technology, used for Z-pinch experiments. Despite the long implosion time (600 to 900 ns), Sphinx wire arrays with large radius (>40 mm) have already shown to behave and to be controlled by the same physical processes as faster Z-pinches. The use of a multi-microsecond current prepulse...
The Sphinx machine based on microsecond LTD technology was used to implode single wire arrays. These z-pinch experiments were performed on Sphinx driver using a multi-microsecond current prepulse. This prepulse dramatically changes the ablation phase leading to an improvement of the axial homogeneity of both the implosion and the final radiating column. As a consequence, the number of parameters and...
Summary form only given. The Sphinx machine is a 6 MA, 1 mus driver based on the LTD technology, used for Z-pinch experiments. Despite the long implosion time (600 ns to 800 ns), Sphinx wire arrays with large diameter (~14 cm) have already shown to behave and to be controlled by the same physical processes as faster Z-pinches. This allowed to find wire arrays configurations able to mitigate instabilities...
The Sphinx generator uses LTD technology to produce >4 MA pulsed power, driving large diameter (>=14 cm), long implosion time (>600 ns) aluminum wire array z-pinch implosions. A large diameter argon gas puff load developed by AASC for Sphinx was given initial testing in 2006. Specifically, we fielded a triple plenum, triple valve system that produces (1) a central 1 cm diameter gas column,...
The Sphinx machine developed at the Centre d'Etudes de Gramat is based on the 1-mus linear-transformer-driver (LTD) technology. Before 2006, it operated with 16 branches of eight LTD stages each and was used for implosion of Z-pinch loads in direct drive mode. At a charging voltage of 50 kV, the stored energy was 1.3 MJ, and the good reliability of the machine allowed many experiments on 4-MA 700-ns...
Summary form only given. The SPHINX machine developed at Centre d'Etudes de Gramat is based on the 1 microsecond LTD technology. Before 2006 it operated with 16 branches of 8 LTD stages each and was used for implosion of Z-pinch loads in direct drive mode. At a charging voltage of 50 kV the stored energy was 1 MJ and the level of reliability of the machine allowed for many experiments on 4 MA, 800...
Summary form only given. The Sphinx machine is based on the 1 microsecond LTD technology, and is used to implode nested aluminum wire arrays. Diagnostics have been continuously fielded and improved to characterize Z-pinch implosion, radiation pulse, generator behaviour as well as hohlraum results. Lines of sight consist of 16 radial lines of sight through apertures in the return current can, an axial...
Summary form only given. The Sphinx generator uses LTD technology to produce >4 MA pulsed power to drive large diameter (>14 cm), long implosion time (>600 ns) aluminum wire array Z-pinch implosions. Recently, an argon gas puff load was developed for use on Sphinx. Specifically, we prepared a triple plenum, triple valve system that drives (1) a central 1 cm diameter gas column, (2) a wide...
Axial radiation flux from an aluminum Z-pinch on SPHINX machine is used to drive 5 mm diameter, 7 mm height hohlraums. 2006 results demonstrated that >200 GW power in 10 ns could be achieved creating >35 eV radiation temperatures. A 20-30 ns delay between the axial power starting time and the temperature rise had, however, to be explained. In this paper, analysis of this behaviour based on 2D...
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