Search

Your search keyword '"Kuchar, Martin"' showing total 275 results
Start Over Author "Kuchar, Martin"
275 results on '"Kuchar, Martin"'

10. Biallelic variants in HPDL cause pure and complicated hereditary spastic paraplegia.

Author

Wiessner, Manuela, Maroofian, Reza, Ni, Meng-Yuan, Pedroni, Andrea, Müller, Juliane S, Stucka, Rolf, Beetz, Christian, Efthymiou, Stephanie, Santorelli, Filippo M, Alfares, Ahmed A, Zhu, Changlian, Uhrova Meszarosova, Anna, Alehabib, Elham, Bakhtiari, Somayeh, Janecke, Andreas R, Otero, Maria Gabriela, Chen, Jin Yun Helen, Peterson, James T, Strom, Tim M, De Jonghe, Peter, Deconinck, Tine, De Ridder, Willem, De Winter, Jonathan, Pasquariello, Rossella, Ricca, Ivana, Alfadhel, Majid, van de Warrenburg, Bart P, Portier, Ruben, Bergmann, Carsten, Ghasemi Firouzabadi, Saghar, Jin, Sheng Chih, Bilguvar, Kaya, Hamed, Sherifa, Abdelhameed, Mohammed, Haridy, Nourelhoda A, Maqbool, Shazia, Rahman, Fatima, Anwar, Najwa, Carmichael, Jenny, Pagnamenta, Alistair, Wood, Nick W, Tran Mau-Them, Frederic, Haack, Tobias, Di Rocco, Maja, Ceccherini, Isabella, Iacomino, Michele, Zara, Federico, Salpietro, Vincenzo, Scala, Marcello, Rusmini, Marta, Xu, Yiran, Wang, Yinghong, Suzuki, Yasuhiro, Koh, Kishin, Nan, Haitian, Ishiura, Hiroyuki, Tsuji, Shoji, Lambert, Laëtitia, Schmitt, Emmanuelle, Lacaze, Elodie, Küpper, Hanna, Dredge, David, Skraban, Cara, Goldstein, Amy, Willis, Mary JH, Grand, Katheryn, Graham, John M, Lewis, Richard A, Millan, Francisca, Duman, Özgür, Dündar, Nihal, Uyanik, Gökhan, Schöls, Ludger, Nürnberg, Peter, Nürnberg, Gudrun, Catala Bordes, Andrea, Seeman, Pavel, Kuchar, Martin, Darvish, Hossein, Rebelo, Adriana, Bouçanova, Filipa, Medard, Jean-Jacques, Chrast, Roman, Auer-Grumbach, Michaela, Alkuraya, Fowzan S, Shamseldin, Hanan, Al Tala, Saeed, Rezazadeh Varaghchi, Jamileh, Najafi, Maryam, Deschner, Selina, Gläser, Dieter, Hüttel, Wolfgang, Kruer, Michael C, Kamsteeg, Erik-Jan, Takiyama, Yoshihisa, Züchner, Stephan, Baets, Jonathan, Synofzik, Matthis, Schüle, Rebecca, and Horvath, Rita

Subjects
Neurosciences, Neurodegenerative, Genetics, Clinical Research, Aetiology, 2.1 Biological and endogenous factors, Neurological, Animals, Female, Humans, Male, Mice, Mutation, Oxygenases, Pedigree, Rats, Spastic Paraplegia, Hereditary, Zebrafish, hereditary spastic paraplegia, HSP, autosomal recessive, mitochondrial disorder, HPDL, Genomics England Research Consortium, PREPARE network, Medical and Health Sciences, Psychology and Cognitive Sciences, Neurology & Neurosurgery
Abstract

Human 4-hydroxyphenylpyruvate dioxygenase-like (HPDL) is a putative iron-containing non-heme oxygenase of unknown specificity and biological significance. We report 25 families containing 34 individuals with neurological disease associated with biallelic HPDL variants. Phenotypes ranged from juvenile-onset pure hereditary spastic paraplegia to infantile-onset spasticity and global developmental delays, sometimes complicated by episodes of neurological and respiratory decompensation. Variants included bona fide pathogenic truncating changes, although most were missense substitutions. Functionality of variants could not be determined directly as the enzymatic specificity of HPDL is unknown; however, when HPDL missense substitutions were introduced into 4-hydroxyphenylpyruvate dioxygenase (HPPD, an HPDL orthologue), they impaired the ability of HPPD to convert 4-hydroxyphenylpyruvate into homogentisate. Moreover, three additional sets of experiments provided evidence for a role of HPDL in the nervous system and further supported its link to neurological disease: (i) HPDL was expressed in the nervous system and expression increased during neural differentiation; (ii) knockdown of zebrafish hpdl led to abnormal motor behaviour, replicating aspects of the human disease; and (iii) HPDL localized to mitochondria, consistent with mitochondrial disease that is often associated with neurological manifestations. Our findings suggest that biallelic HPDL variants cause a syndrome varying from juvenile-onset pure hereditary spastic paraplegia to infantile-onset spastic tetraplegia associated with global developmental delays.

Published
2021

19. Effect of Weighting Coefficients on Behavior of the DTC Method with Direct Calculation of Voltage Vector

Author

Baca, Jakub, Kuchar, Martin, Palacky, Petr, Angrisani, Leopoldo, Series Editor, Arteaga, Marco, Series Editor, Panigrahi, Bijaya Ketan, Series Editor, Chakraborty, Samarjit, Series Editor, Chen, Jiming, Series Editor, Chen, Shanben, Series Editor, Chen, Tan Kay, Series Editor, Dillmann, Rüdiger, Series Editor, Duan, Haibin, Series Editor, Ferrari, Gianluigi, Series Editor, Ferre, Manuel, Series Editor, Hirche, Sandra, Series Editor, Jabbari, Faryar, Series Editor, Jia, Limin, Series Editor, Kacprzyk, Janusz, Series Editor, Khamis, Alaa, Series Editor, Kroeger, Torsten, Series Editor, Liang, Qilian, Series Editor, Martin, Ferran, Series Editor, Ming, Tan Cher, Series Editor, Minker, Wolfgang, Series Editor, Misra, Pradeep, Series Editor, Möller, Sebastian, Series Editor, Mukhopadhyay, Subhas, Series Editor, Ning, Cun-Zheng, Series Editor, Nishida, Toyoaki, Series Editor, Pascucci, Federica, Series Editor, Qin, Yong, Series Editor, Seng, Gan Woon, Series Editor, Speidel, Joachim, Series Editor, Veiga, Germano, Series Editor, Wu, Haitao, Series Editor, Zhang, Junjie James, Series Editor, Zelinka, Ivan, editor, Brandstetter, Pavel, editor, Trong Dao, Tran, editor, Hoang Duy, Vo, editor, and Kim, Sang Bong, editor

Published
2020
Full Text
View/download PDF

23. Fault-Tolerant Control Based on Current Space Vectors against Total Sensor Failures.

Author

Tran, Cuong Dinh, Kuchar, Martin, Sotola, Vojtech, and Nguyen, Phuong Duy

Subjects
*FAULT-tolerant control systems, *VECTOR spaces, *INDUCTION motors, *DETECTORS, *FAULT diagnosis, *FAULT-tolerant computing, *INDUCTION machinery
Abstract

This paper proposes a fault-tolerant control (FTC) strategy using the current space vectors to diagnose sensor failures and enhance the sustained operation of a field-oriented (FO) controlled induction motor drive (IMD). Three space vectors are established for the sensor fault diagnosis technique, including one converted from the measured currents and the other two calculated from the current estimation technique, respectively, measured and with reference speeds. A mixed mathematical model using three space vectors and their components is proposed to accurately determine the fault condition of each sensor in the motor drive. After determining the operating status of each sensor, if the sensor signal is in good condition, the feedback signal to the controller will be the measured signal; otherwise, the estimated signal will be used instead of the failed signal. Failure states of the various sensors were simulated to check the effectiveness of the proposed technique in the Matlab/Simulink environment. The simulation results are positive: the IMD system applying the proposed FTC technique accurately detected the failed sensor and maintained stability during the operation. [ABSTRACT FROM AUTHOR]

Published
2024
Full Text
View/download PDF

26. Structural, Biochemical, and Computational Characterization of Sulfamides as Bimetallic Peptidase Inhibitors

Author

Novakova, Zora, primary, Tehrani, Zahra Aliakbar, additional, Jurok, Radek, additional, Motlova, Lucia, additional, Kutil, Zsofia, additional, Pavlicek, Jiri, additional, Shukla, Shivam, additional, Choy, Cindy J., additional, Havlinova, Barbora, additional, Baranova, Petra, additional, Berkman, Clifford E., additional, Kuchar, Martin, additional, Cerny, Jiri, additional, and Barinka, Cyril, additional

Published
2024
Full Text
View/download PDF

28. PI-Based Fuzzy Speed Controller with PWM Direct Torque Control for Induction Motor Drive

Author

Vo, Hau H., Brandstetter, Pavel, Kuchar, Martin, Dong, Chau S. T., Tran, Thinh C., Vo, Duy H., Angrisani, Leopoldo, Series editor, Arteaga, Marco, Series editor, Chakraborty, Samarjit, Series editor, Chen, Jiming, Series editor, Chen, Tan Kay, Series editor, Dillmann, Ruediger, Series editor, Duan, Haibin, Series editor, Ferrari, Gianluigi, Series editor, Ferre, Manuel, Series editor, Hirche, Sandra, Series editor, Jabbari, Faryar, Series editor, Kacprzyk, Janusz, Series editor, Khamis, Alaa, Series editor, Kroeger, Torsten, Series editor, Ming, Tan Cher, Series editor, Minker, Wolfgang, Series editor, Misra, Pradeep, Series editor, Möller, Sebastian, Series editor, Mukhopadhyay, Subhas Chandra, Series editor, Ning, Cun-Zheng, Series editor, Nishida, Toyoaki, Series editor, Panigrahi, Bijaya Ketan, Series editor, Pascucci, Federica, Series editor, Samad, Tariq, Series editor, Seng, Gan Woon, Series editor, Veiga, Germano, Series editor, Wu, Haitao, Series editor, Zhang, Junjie James, Series editor, Duy, Vo Hoang, editor, Dao, Tran Trong, editor, Zelinka, Ivan, editor, Kim, Sang Bong, editor, and Phuong, Tran Thanh, editor

Published
2018
Full Text
View/download PDF

31. Hexahydrocannabinol (HHC) and Δ9-tetrahydrocannabinol (Δ9-THC) driven activation of cannabinoid receptor 1 results in biased intracellular signaling.

Author

Durydivka, Oleh, Palivec, Petr, Gazdarica, Matej, Mackie, Ken, Blahos, Jaroslav, and Kuchar, Martin

Abstract

The Cannabis sativa plant has been used for centuries as a recreational drug and more recently in the treatment of patients with neurological or psychiatric disorders. In many instances, treatment goals include relief from posttraumatic disorders, anxiety, or to support treatment of chronic pain. Ligands acting on cannabinoid receptor 1 (CB1R) are also potential targets for the treatment of other health conditions. Using an evidence-based approach, pharmacological investigation of CB1R agonists is timely, with the aim to provide chronically ill patients relief using well-defined and characterized compounds from cannabis. Hexahydrocannabinol (HHC), currently available over the counter in many countries to adults and even children, is of great interests to policy makers, legal administrators, and healthcare regulators, as well as pharmacologists. Herein, we studied the pharmacodynamics of HHC epimers, which activate CB1R. We compared their key CB1R-mediated signaling pathway activities and compared them to the pathways activated by Δ9-tetrahydrocannabinol (Δ9-THC). We provide evidence that activation of CB1R by HHC ligands is only broadly comparable to those mediated by Δ9-THC, and that both HHC epimers have unique properties. Together with the greater chemical stability of HHC compared to Δ9-THC, these molecules have a potential to become a part of modern medicine. [ABSTRACT FROM AUTHOR]

Published
2024
Full Text
View/download PDF

40. The Effects of Peripubertal THC Exposure in Neurodevelopmental Rat Models of Psychopathology

Author

Di Bartolomeo, Martina, primary, Stark, Tibor, additional, Di Martino, Serena, additional, Iannotti, Fabio Arturo, additional, Ruda-Kucerova, Jana, additional, Romano, Giovanni Luca, additional, Kuchar, Martin, additional, Laudani, Samuele, additional, Palivec, Petr, additional, Piscitelli, Fabiana, additional, Wotjak, Carsten T., additional, Bucolo, Claudio, additional, Drago, Filippo, additional, Di Marzo, Vincenzo, additional, D’Addario, Claudio, additional, and Micale, Vincenzo, additional

Published
2023
Full Text
View/download PDF

41. Cannabidiol tempers alcohol intake and neuroendocrine and behavioural correlates in alcohol binge drinking adolescent rats. Focus on calcitonin gene-related peptide's brain levels

Author

Tringali, Giuseppe, Lavanco, Gianluca, Castelli, Valentina, Pizzolanti, Giuseppe, Kuchar, Martin, Currò, Diego, Cannizzaro, Carla, Brancato, Anna, Tringali, Giuseppe (ORCID:0000-0002-1030-9429), Tringali, Giuseppe, Lavanco, Gianluca, Castelli, Valentina, Pizzolanti, Giuseppe, Kuchar, Martin, Currò, Diego, Cannizzaro, Carla, Brancato, Anna, and Tringali, Giuseppe (ORCID:0000-0002-1030-9429)

Abstract

Alcohol binge drinking is common among adolescents and may challenge the signalling systems that process affective stimuli, including calcitonin gene-related peptide (CGRP) signalling. Here, we employed a rat model of adolescent binge drinking to evaluate reward-, social- and aversion-related behaviour, glucocorticoid output and CGRP levels in affect-related brain regions. As a potential rescue, the effect of the phytocannabinoid cannabidiol was explored. Adolescent male rats underwent the intermittent 20% alcohol two-bottle choice paradigm; at the binge day (BD) and the 24 h withdrawal day (WD), we assessed CGRP expression in medial prefrontal cortex (mPFC), nucleus accumbens (NAc), amygdala, hypothalamus and brainstem; in addition, we evaluated sucrose preference, social motivation and drive, nociceptive response, and serum corticosterone levels. Cannabidiol (40 mg/kg, i.p.) was administered before each drinking session, and its effect was measured on the above-mentioned readouts. At BD and WD, rats displayed decreased CGRP expression in mPFC, NAc and amygdala; increased CGRP levels in the brainstem; increased response to rewarding- and nociceptive stimuli and decreased social drive; reduced serum corticosterone levels. Cannabidiol reduced alcohol consumption and preference; normalised the abnormal corticolimbic CGRP expression, and the reward and aversion-related hyper-responsivity, as well as glucocorticoid levels in alcohol binge-like drinking rats. Overall, CGRP can represent both a mediator and a target of alcohol binge-like drinking and provides a further piece in the intricate puzzle of alcohol-induced behavioural and neuroendocrine sequelae. CBD shows promising effects in limiting adolescent alcohol binge drinking and rebalancing the bio-behavioural abnormalities.

Published
2023

44. Application of RBF Neural Network in Sensorless Control of A.C. Drive with Induction Motor

Author

Brandstetter, Pavel, Kuchar, Martin, Friedrich, Jiri, Kacprzyk, Janusz, Series editor, de la Puerta, José Gaviria, editor, Ferreira, Iván García, editor, Bringas, Pablo Garcia, editor, Klett, Fanny, editor, Abraham, Ajith, editor, de Carvalho, André C.P.L.F., editor, Herrero, Álvaro, editor, Baruque, Bruno, editor, Quintián, Héctor, editor, and Corchado, Emilio, editor

Published
2014
Full Text
View/download PDF

46. Social stress under binge-like alcohol withdrawal in adolescence: evidence of cannabidiol effect on maladaptive plasticity in rats.

Author

Brancato, Anna, Castelli, Valentina, Lavanco, Gianluca, D'Amico, Cesare, Feo, Salvatore, Pizzolanti, Giuseppe, Kuchar, Martin, and Cannizzaro, Carla

Subjects
CANNABIDIOL, ANIMAL behavior, NEURAL transmission, NEUROENDOCRINE system, ADRENOCORTICAL hormones, ALCOHOL withdrawal delirium, ANIMAL experimentation, BASAL ganglia, BINGE drinking, NEUROPLASTICITY, RATS, DOPAMINE, MOLECULAR biology, TEENAGERS' conduct of life, EXCITATORY amino acid agents, RESEARCH funding, PSYCHOLOGICAL stress, ADOLESCENCE
Abstract

Background: Alcohol binge drinking may compromise the functioning of the nucleus accumbens (NAc), i.e. the neural hub for processing reward and aversive responses. Methods: As socially stressful events pose particular challenges at developmental stages, this research applied the resident–intruder paradigm as a model of social stress, to highlight behavioural neuroendocrine and molecular maladaptive plasticity in rats at withdrawal from binge-like alcohol exposure in adolescence. In search of a rescue agent, cannabidiol (CBD) was selected due to its favourable effects on alcohol- and stress-related harms. Results: Binge-like alcohol exposed intruder rats displayed a compromised defensive behaviour against the resident and a blunted response of the stress system, in addition to indexes of abnormal dopamine (DA)/glutamate plasticity and dysfunctional spine dynamics in the NAc. CBD administration (60 mg/kg) was able to: (1) increase social exploration in the binge-like alcohol exposed intruder rats, at the expenses of freezing time, and in control rats, which received less aggressive attacks from the resident; (2) reduce corticosterone levels independently on alcohol previous exposure; (3) restore DA transmission and (4) facilitate excitatory postsynaptic strength and remodelling. Conclusions: Overall, the maladaptive behavioural and synaptic plasticity promoted by the intersection between binge-like alcohol withdrawal and exposure to adverse social stress can be rescued by a CBD détente effect that results in a successful defensive strategy, supported by a functional endocrine and synaptic plasticity. The current data highlight CBD's relevant therapeutic potential in alcohol- and stress-related harms, and prompt further investigation on its molecular targets. [ABSTRACT FROM AUTHOR]

Published
2023
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results