Les ataxies épisodiques (EA) d’origine génétique sont un groupe de maladies possédant un phénotype et génotype hétérogènes, mais ont en commun la caractéristique d’un dysfonctionnement cérébelleux intermittent. Les EA de type 1 et 2 sont les plus largement reconnues des ataxies épisodiques autosomiques dominantes et sont causées par un dysfonctionnement des canaux ioniques voltage-dépendants dans les neurones. La présente étude se concentrera sur les mutations causant l'EA-1, retrouvées dans le senseur de voltage (VSD) de Kv1.1, un canal très proche de la famille des canaux Shaker. Nous avons caractérisé les propriétés électrophysiologiques de six mutations différentes à la position F244 et partiellement celles des mutations T284 A/M, R297 K/Q/A/H, I320T, L375F, L399I et S412 C/I dans la séquence du Shaker grâce à la technique du ‘’cut open voltage clamp’’ (COVC). Les mutations de la position F244 situées sur le S1 du canal Shaker sont caractérisées par un décalement des courbes QV et GV vers des potentiels dépolarisants et modifient le couplage fonctionnel entre le domaine VSD et le pore. Un courant de fuite est observé durant la phase d'activation des courants transitoires et peut être éliminé par l'application du 4-AP (4-aminopyridine) ou la réinsertion de l'inactivation de type N mais pas par le TEA (tétraéthylamonium). Dans le but de mieux comprendre les mécanismes moléculaires responsables de la stabilisation d’un état intermédiaire, nous avons étudié séparément la neutralisation des trois premières charges positives du S4 (R1Q, R2Q et R3Q). Il en est ressorti l’existence d’une interaction entre R2 et F244. Une seconde interface entre S1 et le pore proche de la surface extracellulaire agissant comme un second point d'ancrage et responsable des courants de fuite a été mis en lumière. Les résultats suggèrent une anomalie du fonctionnement du VSD empêchant la repolarisation normale de la membrane des cellules nerveuses affectées à la suite d'un potentiel d'action. / The genetic episodic ataxias form a group of disorders with heterogeneous phenotype and genotype, but share the common feature of intermittent cerebellar dysfunction. Episodic ataxia (EA) types 1 and 2 are most widely recognised amongst the autosomal dominant episodic ataxias and are caused by dysfunction of neuronal voltage-gated ion channels. The present study focuses on mutations causing EA-1 located in the voltage sensor domains (VSDs) of Kv1.1. A member of the Shaker channel family. Here, we have characterised the electrophysiological properties of six different mutations at the position of F244 and we also reported the partiality effects of these following mutations T284A/M, R297K/Q/A/H, I320T, L375F, L399I S412C/I on Shaker sequence using the cut open voltage clamp technique (COVC). We have shown that mutations of F244 in the S1 of the Shaker Kv channel positively shift the voltage dependence of the VSD movement and alter functional coupling between VSD and pore domain. The mutations causing immobilization of the VSD movement during activation and deactivation and responsible for creating a leak current during activation, are removed by the application of 4-AP (4-aminopyridine) or by reinsertion of N-type inactivation but not by TEA (tetraethylamonium). Insights into the molecular mechanisms responsible for the stabilization of the intermediate state have been investigated by separately neutralizing the first three charges (R1Q, R2Q and R3Q) in the S4 segment. The result suggests an interaction between R2 and F244 mutants. It was established that a second co-evolved interface exists between S1 and the pore helix near the extracellular surface and it acts as a second anchor point. It is also responsible for generation of leak currents. The results suggest a dysfunction of the VSD in which the affected nerve cells cannot efficiently repolarize following an action potential because of altered delayed rectifier function

Les ataxies épisodiques (EA) d’origine génétique sont un groupe de maladies possédant un phénotype et génotype hétérogènes, mais ont en commun la caractéristique d’un dysfonctionnement cérébelleux intermittent. Les EA de type 1 et 2 sont les plus largement reconnues des ataxies épisodiques autosomiques dominantes et sont causées par un dysfonctionnement des canaux ioniques voltage-dépendants dans les neurones. La présente étude se concentrera sur les mutations causant l'EA-1, retrouvées dans le senseur de voltage (VSD) de Kv1.1, un canal très proche de la famille des canaux Shaker. Nous avons caractérisé les propriétés électrophysiologiques de six mutations différentes à la position F244 et partiellement celles des mutations T284 A/M, R297 K/Q/A/H, I320T, L375F, L399I et S412 C/I dans la séquence du Shaker grâce à la technique du ‘’cut open voltage clamp’’ (COVC). Les mutations de la position F244 situées sur le S1 du canal Shaker sont caractérisées par un décalement des courbes QV et GV vers des potentiels dépolarisants et modifient le couplage fonctionnel entre le domaine VSD et le pore. Un courant de fuite est observé durant la phase d'activation des courants transitoires et peut être éliminé par l'application du 4-AP (4-aminopyridine) ou la réinsertion de l'inactivation de type N mais pas par le TEA (tétraéthylamonium). Dans le but de mieux comprendre les mécanismes moléculaires responsables de la stabilisation d’un état intermédiaire, nous avons étudié séparément la neutralisation des trois premières charges positives du S4 (R1Q, R2Q et R3Q). Il en est ressorti l’existence d’une interaction entre R2 et F244. Une seconde interface entre S1 et le pore proche de la surface extracellulaire agissant comme un second point d'ancrage et responsable des courants de fuite a été mis en lumière. Les résultats suggèrent une anomalie du fonctionnement du VSD empêchant la repolarisation normale de la membrane des cellules nerveuses affectées à la suite d'un potentiel d'action. / The genetic episodic ataxias form a group of disorders with heterogeneous phenotype and genotype, but share the common feature of intermittent cerebellar dysfunction. Episodic ataxia (EA) types 1 and 2 are most widely recognised amongst the autosomal dominant episodic ataxias and are caused by dysfunction of neuronal voltage-gated ion channels. The present study focuses on mutations causing EA-1 located in the voltage sensor domains (VSDs) of Kv1.1. A member of the Shaker channel family. Here, we have characterised the electrophysiological properties of six different mutations at the position of F244 and we also reported the partiality effects of these following mutations T284A/M, R297K/Q/A/H, I320T, L375F, L399I S412C/I on Shaker sequence using the cut open voltage clamp technique (COVC). We have shown that mutations of F244 in the S1 of the Shaker Kv channel positively shift the voltage dependence of the VSD movement and alter functional coupling between VSD and pore domain. The mutations causing immobilization of the VSD movement during activation and deactivation and responsible for creating a leak current during activation, are removed by the application of 4-AP (4-aminopyridine) or by reinsertion of N-type inactivation but not by TEA (tetraethylamonium). Insights into the molecular mechanisms responsible for the stabilization of the intermediate state have been investigated by separately neutralizing the first three charges (R1Q, R2Q and R3Q) in the S4 segment. The result suggests an interaction between R2 and F244 mutants. It was established that a second co-evolved interface exists between S1 and the pore helix near the extracellular surface and it acts as a second anchor point. It is also responsible for generation of leak currents. The results suggest a dysfunction of the VSD in which the affected nerve cells cannot efficiently repolarize following an action potential because of altered delayed rectifier function

Switching of shunt reactors and capacitor banks is known to cause a very high rate of rise of transient recovery voltage across the circuit breaker contacts. With improvements in circuit breaker technology, modern SF6 puffer circuits have been designed with less interrupter per pole than previous generations of SF6 circuit breakers. This has caused modern circuit breakers to operate with higher voltage stress in the dielectric recovery region after current interruption. Catastrophic failures of modern SF6 circuit breakers have been reported during shunt reactor and capacitor bank de-energisation. In those cases, evidence of cumulative re-strikes has been found to be the main cause of interrupter failure. Monitoring of voltage waveforms during switching would provide information about the magnitude and frequency of small re-ignitions and re-strikes. However, measuring waveforms at a moderately high frequency require plant outages to connect equipment. In recent years, there have been increasing interests in using RF measurements in condition monitoring of switchgear. The RF measurement technique used for measuring circuit breaker inter-pole switching time during capacitor bank closing is of particular interest. In this thesis, research has been carried out to investigate switching transients produced during circuit breaker switching capacitor banks and shunt reactors using a non-intrusive measurement technique. The proposed technique measures the high frequency and low frequency voltage waveforms during switching operations without the need of an outage. The principles of this measurement technique are discussed and field measurements were carried out at shunt rector and capacitor bank installation in two 275 kV air insulated substations. Results of the measurements are presented and discussed in this thesis. The proposed technique shows that it is relatively easy to monitor circuit breaker switching transients and useful information on switching instances can be extracted from the measured waveforms. Further research works are discussed to realise the full potential of the measuring technique.

The diploma thesis deals with conventional transformers and current and voltage sensors. The first half of thesis describes mainly the basic concepts and accuracy classes of these converters used in electrical substations. The emphasis is given primarily on the differences. In the second half of thesis is presented an exact type of converters, which are installed in the substation Medlánky. There is an analysis of measured data from long – term monitoring from this substation and comparing the results from conventional transformers compared to the results from more modern sensors.

Avec les progrès technologiques réalisés au cours de ces dernières décennies, la complexité et les vitesses de fonctionnement des circuits intégrés ont beaucoup été augmentées. Bien que ces évolutions aient permis de diminuer les dimensions et les tensions d’alimentations des circuits, la compatibilité électromagnétique (CEM) des composants a fortement été dégradée. Identifiée comme étant un verrou technologique, la CEM est aujourd’hui l’une des principales causes de « re-design » des circuits car les problématiques liées aux mécanismes de génération et de couplage du bruit ne sont pas suffisamment étudiées lors de leur conception.Ce manuscrit présente donc une méthodologie visant à étudier la propagation du bruit à travers les circuits intégrés par mesures et par simulations. Afin d’améliorer nos connaissances sur la propagation d’interférences électromagnétiques (IEM) et les mécanismes de couplage à travers les circuits, nous avons conçu un véhicule de test développé dans la technologie SMOS8MV® 0,25 µm de Freescale Semiconductor. Dans ce circuit, plusieurs fonctions élémentaires telles qu’un bus d’E/S et des blocs numériques ont été implémentées. Des capteurs de tensions asynchrones ont également été intégrés sur différentes alimentations de la puce pour analyser la propagation des perturbations injectées sur les broches du composant (injection DPI) et sur les conducteurs permettant d’alimenter ce dernier (injection BCI). En outre, nous proposons différents outils pour faciliter la modélisation et les simulations d’immunité des circuits intégrés (extraction des modèles de PCB, approches de modélisation des systèmes d’injection, méthode innovante permettant de prédire et de corréler les niveaux de tension/ de puissance injectés lors de mesures d’immunité conduite, flot de modélisation). Chaque outil et méthode de modélisation proposés sont évalués sur différents cas test. Enfin, pour évaluer notre démarche de modélisation, nous l’appliquons sur un bloc numérique de notre véhicule de test et comparons les résultats de simulations aux différentes mesures internes et externes réalisées sur le circuit / With technological advances in recent decades, the complexity and operating speeds of integrated circuits have greatly increased. While these developments have reduced dimensions and supply voltages of circuits, electromagnetic compatibility (EMC) of components has been highly degraded. Identified as a technological lock, EMC is now one of the main causes of circuits re-designs because issues related to generating and coupling noise mechanisms are not sufficiently studied during their design. This manuscript introduces a methodology to study propagation of electromagnetic disturbances through integrated circuits by measurements and simulations. To improve our knowledge about propagation of electromagnetic interferences (EMI) and coupling mechanisms through integrated circuits, we designed a test vehicle developed in the SMOS8MV® 0.25µm technology from Freescale Semiconductor. In this circuit, several basic functions such as I/O bus and digital blocks have been implemented. Asynchronous on-chip voltage sensors have also been integrated on different supplies of the chip to analyze propagation of disturbances injected on supply pins and wires of the component (DPI and BCI injection). In addition, we propose various tools to facilitate modeling and simulations of Integrated Circuit’s immunity (PCB model extraction, injection systems modeling approaches, innovative method to predict and correlate levels of voltage / power injected during conducted immunity measurements, modeling flow). Each tool and modeling method proposed is evaluated on different test cases. To assess our modeling approach, we finally apply it on a digital block of our test vehicle and compare simulation results to various internal and external measurements performed on the circuit

The aim of this Master´s thesis is use of instrument transformers and sensors on field of industry protection. We will compare current and voltage transformers, current sensor – based on Rogowski coil, voltage sensor – based on voltage divider. By this measure devices, we can monitoring values of analog quantities in medium voltage switchgear. It is impossible to compare, measure and analyze without this measure devices. There is protection terminal REF542plus, which can compile this values. The REF542plus ability are measuring, monitoring, remote control and protection. First, we will discuss about theory of sensors and convential instrument transformers and analysis of analog signal. We will compare analog input channel on sensor´s analog module and transformer´s analog module. There are few differences between type of analog modules. For analog signal analysis are important frequency filters and Analog/ Digital Convertor (sigma-delta). We will describe functions and options of REF542plus. In practical part of this project, we will test protection functions of protection terminal. First, protection terminal will be connected to sensors. Second protection terminal will be connected to transformers. For testing we chose Earth-fault directional protection and differential protection. We will make only secondary tests. That´s mean, input analog quantities to REF542plus will be simulated by tester. In all we will verify quality of protection. We will focus on lower settings of protection and we will inject protection by low current. Objectives are testing of trip characteristics and measuring of trip time.