Tesis sobre el tema "Motoneurones"
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Fuente, Ruiz Sandra de la. "Development of new therapeutic strategies for Spinal Muscular Atrophy". Doctoral thesis, Universitat de Lleida, 2020. http://hdl.handle.net/10803/669753.
Texto completoLa atrofia muscular espinal (AME) es una enfermedad neurodegenerativa grave y la primera causa genética de muerte infantil. Se origina por la pérdida o mutación del gen Survival Motor Neuron 1 (SMN1) que causa una deficiencia de la proteína de Survival Motor Neuron (SMN). La reducción de esta proteína conduce predominantemente a la degeneración de las motoneuronas (MNs) de la médula espinal y, en consecuencia, produce atrofia y debilidad del músculo esquelético. Actualmente, solo se conoce parcialmente que mecanismos celulares y moleculares exactos son los responsables de la pérdida de función de las MNs. La reducción de SMN causa degeneración de neuritas y muerte celular sin características apoptóticas clásicas. La autofagia es un proceso importante y altamente regulado, esencial para la eliminación de orgánulos dañados y sustancias o proteínas tóxicas a través de la degradación con los lisosomas. La autofagia es especialmente importante en células post-mitóticas, como las MNs, donde la acumulación de autofagosomas provoca la interrupción del transporte axonal, la interferencia del tráfico intracelular y la degeneración de las neuritas. Lo que es bien sabido en la AME es que el nivel intracelular de proteína SMN define el inicio y la gravedad de la enfermedad y esto está parcialmente determinado por el número de copias del gen SMN2, la duplicación centromérica de SMN y el principal modificador de la AME. Por esa razón, comprender los procesos que regulan la degradación de SMN con la finalidad de identificar compuestos que aumentan los niveles de proteínas es el principal objetivo en el desarrollo terapéutico de AME. Las calpaínas son una familia de proteasas dependientes de calcio que se han relacionado con trastornos musculares y enfermedades neurodegenerativas. Específicamente, se ha descrito en el músculo que SMN puede ser proteolizada por calpaína. La actividad de la calpaína también está involucrada en la regulación de la autofagia mediante la modulación de múltiples de las proteínas involucradas en el proceso. El objetivo en el presente trabajo ha sido analizar la desregulación de la autofagia y determinar la participación de la calpaína en la regulación de la proteína SMN en las MNs para profundizar en el origen de la neurodegeneración y desarrollar un nuevo enfoque terapéutico para la AME. Con este fin, hemos analizado marcadores autofágicos en diferentes modelos in vitro de AME, tanto de ratón como de humano. Los resultados mostraron que los autofagosomas y los niveles de LC3 se encuentran aumentados en las muestras de AME en comparación con los controles, lo que sugiere una desregulación del proceso de autofagia a lo largo de la progresión de la enfermedad. Además, la reducción de los niveles endógenos de calpaína utilizando un shRNA muestraron un aumento de los niveles de Smn y LC3, a la vez que previene la degeneración neuritica que se produce en las MNs de ratón afectados por AME. Se obtuvieron resultados similares en experimentos in vitro utilizando un inhibidor farmacológico de calpaína, la calpeptina. Asimismo, la activación de calpaína producida por condiciones despolarizantes inducia la proteólisis de α-fodrina y de SMN, lo que confirma que calpain regula directamente los niveles de proteína SMN en las MNs. Además, el tratamiento con calpeptina in vivo mejoró significativamente la esperanza de vida y la función motora de dos modelos de ratones con AME, lo que demuestra la utilidad potencial de los inhibidores de la calpaína en la terapia para la enfermedad. Finalmente, el análisis de la vía de la calpaína en ratones y modelos celulares humanos de AME indicó un aumento de la actividad de la calpaína en las MNs con niveles reducidos de SMN. Por lo tanto, nuestros resultados demuestran que la actividad de la calpaína se encuentra sobreactivada en las MNs de AME y su inhibición puede tener un efecto beneficioso sobre el fenotipo de la enfermedad a través del aumento de SMN y la regulación del proceso de autofagia en las MNs de la médula espinal.
Spinal Muscular Atrophy (SMA) is a severe neurodegenerative disease and the first genetic cause of infant death. It is originated by the deletion or mutation of Survival Motor Neuron 1 (SMN1) gene causing a Survival Motor Neuron (SMN) protein deficiency. The reduction of this protein predominantly leads to the degeneration of spinal cord motoneurons (MNs) and consequently produces skeletal muscle atrophy and weakness. The exact cellular and molecular mechanisms responsible for MN loss of function are only partially known. SMN reduction causes neurite degeneration and cell death without classical apoptotic features. Autophagy is an important and highly regulated process, essential for the removal of damaged organelles and toxic substances or proteins through lysosome degradation. This mechanism is specifically important in post-mitotic cells like MNs where autophagosome accumulation causes axonal transport disruption, interference of intracellular space trafficking, and neurite degeneration. What is well known in SMA is that intracellular SMN protein levels are critical to define the disease onset and severity, and this is partially determined by the number of copies of SMN2, the centromeric duplication of the SMN gene and the main modifier of SMA. For that reason, understanding the processes of SMN stability and degradation to identify compounds that increase protein levels is a major goal in SMA therapeutics development. Calpains are a family of calcium-dependent proteases that have been related to muscle disorders and neurodegenerative diseases. Specifically, it has been described in muscle that SMN can be a proteolytic target of calpain. Calpain activity is also involved in autophagy regulation by modulation of multiple proteins involved in the process. The objectives in the present work have been to analyze the autophagy deregulation and determine the involvement of calpain in SMN protein regulation on MNs, in order to deepen in the origin of neurodegeneration and to develop a new therapeutic approach for SMA disease. To this end, we have analyzed autophagic markers in different mouse and human SMA in vitro models. The results showed that autophagosomes and LC3 levels were increased in SMA samples compared to controls, suggesting a deregulation of the autophagy process throughout the disease progression. Moreover, calpain knockdown using an shRNA approach showed an increase of both, Smn and LC3 levels and prevented neurite degeneration occurred in SMA affected mouse MNs. Similar results were obtained in in vitro experiments using a pharmacological calpain inhibitor, calpeptin. Likewise, calpain activation produced by depolarized conditions induced α-fodrin and SMN proteolysis, confirming that calpain directly regulates the SMN protein level in MNs. Additionally, calpeptin in vivo treatment significantly improved the lifespan and motor function of two severe SMA mouse models, demonstrating the potential utility of calpain inhibitors in SMA therapeutics. Finally, the analysis of calpain pathway members in mice and human cellular SMA models indicated an increase of calpain activity in SMN-reduced MNs. Thus, our results show that calpain activity is increased in SMA MNs and its inhibition may have a beneficial effect on the SMA phenotype through the increase of SMN and the regulation of the autophagy process in spinal cord MNs.
Sedel, Frédéric. "Mécanisme de programmation de la mort des motoneurones embryonnaires". Paris 6, 2005. http://www.theses.fr/2005PA066611.
Texto completoSahal, Anil. "The monoaminergic control of gamma motoneurones". Thesis, University of Glasgow, 2001. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.394767.
Texto completoLeroy, Félix. "Atteinte différentielle de deux populations de motoneurones spinaux chez le souriceau SOD1 G93A (modèle de la maladie de Charcot)". Thesis, Paris 5, 2013. http://www.theses.fr/2013PA05T063/document.
Texto completoIn the second postnatal week, the locomotor behavior of mice changes from crawling to walking. This is made possible by profound changes in motor units. Yet, how the discharge properties of spinal motoneurons evolve during post-‐natal maturation and whether they have an effect on the motor unit maturation remains an open question. In neonates, the spinal motoneurons display two modes of discharge. For threshold pulses, 33% of the motoneurons have a discharge that start at the current onset and adapts during the pulse (“immediate firing motoneurons”). The remaining 66% motoneurons fire with a large delay and the discharge then accelerates throughout the pulse (“delayed firing motoneurons”). Though the delayed firing pattern is quite common in spinal motoneurons of neonates, the ionic mechanisms that elicit this mode of discharge have received little attention. Using the patch-clamp technique to record P6‐P10 mouse motoneurons in a spinal cord slice preparation, I characterized the ionic currents that underlie the delayed firing pattern. This is caused by a combination of an A-like potassium current that acts on a short time scale and a slow‐inactivating potassium current that delays the discharge on a much longer time scale. I then investigated how these two potassium currents contribute to the recruitment threshold and how they shape the F-I function of delayed motoneurons in neonatal mice. The slow inactivating potassium current induces memory effects that have a strong impact on motoneuron excitability and on its discharge. Building on these results, I tried to correlate the discharge pattern to known physiological sub‐types. The delayed firing motoneurons have a larger input conductance, a higher rheobase, a narrower action potential, a shorter AHP and a more complex dendritic arbor than the immediate firing motoneurons. Additionally, only a sub-‐population of the delayed firing motoneurons expressed the chondrolectin protein, a fast motoneuron marker. Based on this body of corroborating evidence, the immediate firing motoneurons would be slow type motoneurons whereas the delayed firing motoneurons would be fast type motoneurons. Finally, numerous electrical and geometrical abnormalities have been observed in spinal motoneurons of SOD1 G934 mice (model of the amyotrophic lateral sclerosis) during the second post-natal week but the results were somehow contradictory. In relation to the known differential sensitivity to the disease exhibited by slow and fast motoneurons, I investigated whether the immediate and delayed firing motoneurons are equally affected by the SOD1 mutation. This is not the case. I found that the SOD1 mutation induced a decrease in the rheobase and a hyperpolarization of the voltage threshold only in the immediate firing motoneurons, thereby making them more excitable than in WT mice. Furthermore, the dendrites of the immediate firing motoneurons are substantially shorter (about 35%) in the mutant than in the WT. In sharp contrast, the excitability of the delayed firing motoneurons is unchanged and the dendritic tree is nearly unaffected (the dendrites only undergo a 10% elongation). These results allow for reconsidering the link between hyperexcitability and degenerescence of the motoneurons
Teixidó, Viyuela Laura. "Factors sèrics en l’Esclerosi Lateral Amiotròfica. Modulació del receptor de glutamat de tipus NMDA GluN1/GluN2A". Doctoral thesis, Universitat de Barcelona, 2011. http://hdl.handle.net/10803/79039.
Texto completoAmyotrophic lateral sclerosis (ALS) is a devastating neuromuscular disease, characterized by the selective degeneration of the superior motor neurons in the motor cortex and of the inferior motor neurons in the brain-stem and spinal cord. The familial form of the illness is associated with the mutation of the superoxide dismutase enzyme (SOD-1). This and other mutations accounts for fewer than 10% of cases; the rest, more than 90%, correspond to the sporadic form. In this study we tested the effect of sera from sporadic ALS patients and from mutated human SOD-1 (mSOD1 G93A) transgenic rats on N-methyl-D-aspartate receptors (NMDAR). We hypothesize that an endogenous excitotoxic factor is implicated in neuronal death in ALS, mediated by the activation of NMDAR noncanonical signalling pathways. Sera from ALS patients or healthy subjects were pretreated to inactivate complement pathways and dialysed to remove glutamate. Sera from mSOD1 G93A rats were obtained at different stages of the neurodegenerative progression. Sera from transgenic rats were also pretreated to eliminate complement system and glutamate. Immunoglobulins G (IgGs) from ALS patients and healthy subjects were obtained by affinity chromatography and dialyzed against phosphate-buffered saline. Human NMDAR were expressed in Xenopus laevis oocytes, and glutamate-induced currents were recorded using the two electrode voltage clamp technique. We observed that sera from sporadic ALS patients induced transient oscillatory currents in Xenopus oocytes expressing NMDAR with a total electric charge significantly higher than the electric charge carried by currents induced by sera from healthy subjects. The currents were inhibited by MK-801, a noncompetitive blocker of NMDAR. Results of sera from mSOD1 G93A transgenic rats were similar to those of sera from ALS patients; samples from patients with another type of neuromuscular disease did not exert this effect. IgG from ALS patients have a significant effect on NMDAR-injected oocytes and that response was doubled respect to the observed in the case of IgG from healthy subjects. Our data agree with the view that ALS patients sera contain some soluble factors that activates NMDAR, not opening directly the ionic conductance, but activating a non-canonical pathway.
Bouhadfane, Mouloud. "Propriétés électriques bistables des motoneurones de la moelle épinière : Identification des mécanismes ioniques sous-jacents". Thesis, Aix-Marseille, 2014. http://www.theses.fr/2014AIXM5030/document.
Texto completoPosture allowing an erect posture of the body relies on spiking activity of motoneurons innervating antigravitary muscle. Discharge could take the form of plateau potential on mature motoneurons of numerous vertebrates. To determine a possible concordance between the emergence of plateau potential and postural control development, we studied the maturation and ionic nature of plateau potential of motoneurons innervating triceps surae muscle of neonatal rat.The conclusion of our work allows us to propose a fundamental mechanism in the genesis of plateau potential on lumbar motoneurons. This mechanism based on a "ménage a trois" seems to play an important role in the neonatal motor development. This scientific advance could eventually lead to a better understanding of the origin of some sensori-motor impairments (spasticity, hyperalgesia...) and development of therapeutic strategies
SIMON, MAGDA. "Developpement postnatal des motoneurones spinaux lombaires du chat : identification des motoneurones alpha et gamma et remaniements de leur equipement synaptique". Paris 6, 1996. http://www.theses.fr/1996PA066391.
Texto completoAllard, Ludivine. "Dysfonctions mitochondriales et homéostasie bioénergétique des motoneurones dans un modèle de sclérose latérale amyotrophique". Thesis, Bordeaux 2, 2013. http://www.theses.fr/2013BOR22088/document.
Texto completoAmyotrophic lateral sclerosis (ALS) is a fatal adult-onset neurodegenerative disorder characterized by a loss of motor neurons, leading to muscle wasting and weakness. Mutations in superoxide dismutase-1 (SOD1) cause a form of ALS. As in ALS patients, the mutant SOD1 animal model of ALS reveals that not all motor neurons are equally susceptible to the disease process. An attractive mechanism underlying differential susceptibility is the variable bioenergetics need of distinct subsets of motor neurons. This implies that within the CNS, bioenergetics can modulate the pathological threshold. Even in the absence of loss in bioenergetics, one can envision a situation in which a pathological stress alters the level at which either the production or delivery of ATP becomes insufficient, precipitating the demise of the most vulnerable neuron types. In neurons, majority of ATP is produced by mitochondria and the homeostasis of ion gradients is the most energy-consuming process. Reduced mitochondrial function will modify the electrical properties of motor neurons if ATP availability becomes insufficient to allow ion pumps to maintain appropriate gradients. We demonstrated that the basal ATP intra-cellular concentration in motor neuron cultures lower in SOD1 mutated cells compared to wild type. Paradoxically to this result, the oxygen consumption rate of mitochondria is increase in mSOD1 cells and there is no evidence for an increase of consumption. Our results support the interesting hypothesis that there is an uncoupling between the respiratory chain and the ATP production. This uncoupling might be used as a strategy to minor the toxic properties of hyper stimulated mitochondrion
Conway, Elizabeth Ann. "The development of corticospinal projections in man". Thesis, University of Newcastle Upon Tyne, 1995. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.296148.
Texto completoEvans, Pamela. "Crossed reflexes of the human lower limbs : a study using the Hoffman reflex". Thesis, University College London (University of London), 2001. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.367596.
Texto completoCohen, Samia. "Les sémaphorines dans le développement des circuits neuronaux : distribution, fonction, régulation de la signalisation". Aix-Marseille 2, 2007. http://theses.univ-amu.fr.lama.univ-amu.fr/2007AIX22052.pdf.
Texto completoDuring embryonic development, the establishment of functional neuronal circuits requires the correct wiring of axonal projections. This process is controlled by axonal guidance cues like the semaphorins. In the first part of my thesis, I characterized the expression of secreted semaphorins and their receptors, neuropilins and Plexines, in motor neurons by in situ hybridization. The combinatorial expression of these molecules defines subpopulations of motor neurons. The genetic deletion of two secreted semaphorins disturbing the boundaries between these populations; we suggest that secreted semaphorins control the positional organization of motor neuron subpopulations. In a second part, I focused my work on the activity of a specific semaphorin, Sema3E. In Sema3E mutant embryos, projections of two neuronal types are affected. In culture, these two populations respond differentially to Sema3E which is either attractive or repulsive. The molecular study of this bi functionality shows that PlexinD1 receptor is necessary to both activities and that the presence of the co-receptor Neuropilin1 is necessary and sufficient to specify an attractive response. We discuss about receptors to the secreted semaphorins during their individual signaling, and suggest that the combinatorial expression of these cues might modulate the binary responses into graduated responses, or moreover, might generate new axonal guidance information that are required to establish the numerous existing neuronal connections. Key words: semaphorins, neuropilins, plexins, axonal guidance, motor pools, bi functionality
Martin, Peter Glen Medical Sciences Faculty of Medicine UNSW. "Control of human motoneurones during voluntary contraction and fatigue". Awarded by:University of New South Wales. Medical Sciences, 2007. http://handle.unsw.edu.au/1959.4/29404.
Texto completoBernard, Nathalie. "Implication de la Calréticuline et de CRMP4 dans la dégénérescence des motoneurones dans la Sclérose Latérale Amyotrophique". Thesis, Aix-Marseille 2, 2011. http://www.theses.fr/2011AIX22088.
Texto completoAmyotrophic Lateral Sclerosis (ALS) is characterized by the selective degeneration of upper and lower motoneurons (MNs). The most common familial form and best characterized mouse model of ALS is linked to mutations in the gene coding for the superoxide dismutase 1 (mSOD1). MNs expressing mSOD1 show an increased sensitivity to the death induced by Fas/NO activation. Our proteomic study identified two downstream effectors of NO, Calreticulin (CRT) and CRMP4. CRT is a chaperone-calcium-binding protein of the endoplasmic reticulum, which is decreased two-fold in vivo, in an early degenerating MNs sub-population, named vulnerable. The decrease in CRT expression is both necessary and sufficient to kill mSOD1 MNs through ER stress activation. CRMP4 is a neurite outgrowth regulator which expression is increased in vivo in mSOD1 MNs at a presymptomatic stage. CRMP4 overexpression is sufficient to induce peripheral denervation and, through a dying-back effect, to kill mSOD1 MNs. Our results point out CRT and CRMP-4 as two potential therapeutic targets for ALS
Livet, Jean. "Spécification, guidage axonal, migration, survie : le développement de sous-populations de motoneurones chez l'embryon de souris". Aix-Marseille 2, 2001. http://www.theses.fr/2001AIX22072.
Texto completoLi, Fei. "The effects of mild hypoxaemia on hypoglossal motoneurones activity in neonates". Thesis, University College London (University of London), 1996. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.339143.
Texto completoVassias-Jossic, Isabelle. "Modulation des propriétés intrinsèques et neurochimiques des motoneurones faciaux après axotomie". Paris 5, 2006. http://www.theses.fr/2006PA05S010.
Texto completoFacial nerve axotomy is a good model for studying neuronal plasticity in the peripheral nervous system. During the first 2 months after lesion, the expression pattern ol mRNA coding the 3 subunits of Ca2 activated K channel (SKi-3) and the f31 auxiliar’ subunit of the voltage-dependent sodium channels is modified. The lesion also severely modifies the neurochemical properties of the motoneurons. Major subunits of the GABI receptors (GABAA cLl, f32 and ‘2; GABAB lb and 2) and AMPA glutamate receptors (GIuR2-4) are strongly decreased as well at the level of mRNA and protein. These results are in agreement with the hypotheses according to which, in response to a lesion, the motoneurons decrease the expression of the molecules reserved for the synaptic transmission with the profit of those mplied in axonal regeneration. The blocking of a peripheral factor, the neuronal silence or an injury signal could be implied in these modulations
Camu, William. "Facteurs trophiques et toxiques des motoneurones de rat embryonnaire en culture". Montpellier 1, 1993. http://www.theses.fr/1993MON1T028.
Texto completoBarthélémy, Catherine. "Régulation de la mort cellulaire induite par Fas dans les motoneurones". Aix-Marseille 2, 2004. http://www.theses.fr/2004AIX22036.
Texto completoGarcès, Alain. "Sous-populations de motoneurones et dépendance neurotrophique au cours du développement". Aix-Marseille 2, 1999. http://www.theses.fr/1999AIX22066.
Texto completoManette, Olivier F. L. "Codage spatio-temporel des neurones cortico-motoneuronaux". Paris 6, 2007. http://www.theses.fr/2007PA066631.
Texto completoThe Cortico-Motoneuronal (CM) System is composed of populations of neurons located in the Motor Cortex. Theses neurons project directly to motoneurons in the spinal chord. The CM system is implicated in the control of voluntary movements of the hand. The aim of this thesis was both to understand: i) What type of information is carried by the CM system? ii) How is this information coded? In order to investigate theses questions, we have used recordings of CM neurons and muscular activities in the Macaque Monkey who performed a precision grip task between the index finger and the thumb. We have found that i) the transmitted information is directly correlated to the muscular activity. Ii) This information is coded by a combination of temporal and frequency coding. Theses results have been compiled in a formal model called TempUnit based on the principle of temporal summation
Duclos, Yann. "Etude des processus spinaux qui préparent à la réalisation d'un mouvement volontaire chez l'homme : implication précoce des motoneurones dans la préparation motrice". Thesis, Aix-Marseille 2, 2011. http://www.theses.fr/2011AIX20683.
Texto completoThe aim of this work was to analyze the effects of motor preparation on motoneuron (MN) activity. For this purpose, recordings of wrist extensor muscles motor unit activity were combined with time motor preparation paradigms in Human. Changes in the MN tonic discharge were found to occur during preparatory period, i.e. well before it is time to act. These changes were a lengthening of the mean inter-spike interval associated with a decrease of its variability. These data clearly demonstrate that spinal inhibitory mechanisms are activated during motor preparation and suggest the involvement of premotoneuronal interneurons. The modulations of motoneuronal activity induced by the motor preparation are neither specific to the agonist muscle involved in the motor response nor predictive of the performance. It is assumed that the inhibition acting on the MN during the motor preparation constitutes a general braking mechanism serving to prevent premature motor response, whereas the decrease of discharge variability would be a compensatory phenomenon, allowing to produce an efficient steady force in spite of lower motoneuronal activation. The involvement of the motoneuronal level in motor preparation demonstrates that advance information may influence the state of the motor system, including even the most peripheral motor neurons in the spinal cord, which supports the idea that motor preparation involves highly distributed functional processes. In addition, this work led us to argue in favor of the approximate entropy analysis as a suitable method for analyzing spike trains, allowing to detect changes in the regularity of the time-ordered inter-spikes intervals
Casas, Atala Mariana Victoria. "Caractérisation de modèles animaux pour l' étude des différents types de motoneurones". Paris 6, 2005. http://www.theses.fr/2006PA066014.
Texto completoDuflocq, Amandine. "Caractérisation du segment initial de l’axone des motoneurones lombaires chez la souris". Paris 6, 2011. http://www.theses.fr/2011PA066016.
Texto completoButtigieg, Dorothee. "Diversité des motoneurones au cours du développement normal et en situation pathologique". Thesis, Aix-Marseille, 2013. http://www.theses.fr/2013AIXM4046.
Texto completoMuscles are highly vulnerable whereas motor neurons innervating axial muscles are relatively resistant. Motor neurons also seem to differ in their response to several neurotrophic factors (NTF). I investigated the molecular mechanisms determining the distinct morphology and the differential NTF response of ALS-relevant motor neuron subsets. First, I demonstrated that mouse lumbar motor neurons innervating either axial muscles (MMC-MN) or hindlimb muscles (LMC-MN) display remarkable morphological differences. These differences involve a differential regulation of genes coding for Peripherin and Diacylglycerol kinase-b (DGK-β) which are regulated by the transcription factors FoxP1/HB9. Second, I showed that LMC-MN and MMC-MN respond differentially to the three neurotrophic factors HGF (Hepatocyte Growth Factor), Artemin and CNTF (Ciliary NeuroTrophic Factor). Their differential survival is explained by the corresponding receptor gene expression in specific pools of MMC-MN and LMC-MN. Third, I studied lumbar motor neurons in two pathological conditions: 1) alteration of Golgi apparatus and axonal trafficking induced by loss of TBCE (Tubulin Binding Cofactor E) 2) the effect of KCC2 increase at motor neuron plasma membrane on inhibitory synaptic transmission after trauma.Finally, I developed a new FACS-based method for isolating human iPS (induced Pluripotent Stem Cell)-derived motor neurons with both an HB9::GFP reporter lentivirus and an antibody directed against the low-affinity neurotrophin receptor p75
Ayala, Jové Ma Victoria (Maria Victoria). "Regulació de l'expresió de c-Jun durant el desenvolupament de les motoneurones espinals". Doctoral thesis, Universitat de Lleida, 2001. http://hdl.handle.net/10803/8104.
Texto completoCarlin, Kevin P. "Voltage-gated calcium currents in mouse spinal motoneurones, possible role in plateau potentials". Thesis, National Library of Canada = Bibliothèque nationale du Canada, 2000. http://www.collectionscanada.ca/obj/s4/f2/dsk1/tape2/PQDD_0013/NQ53052.pdf.
Texto completoReddad, Salima. "Motoneurones des muscles laryngés intrinsèques : études élecrophysiologiques et pharmacologique de leur activité respiratoire". Aix-Marseille 3, 1987. http://www.theses.fr/1987AIX30077.
Texto completoReddad, Salima. "Motoneurones des muscles larynges intrinsèques études électrophysiologique et pharmacologique de leur activité respiratoire /". Grenoble 2 : ANRT, 1987. http://catalogue.bnf.fr/ark:/12148/cb37609244q.
Texto completoSiarey, Richard John. "The actions of centrally acting muscle relaxants on synpatic reflexes of motoneurones in vitro". Thesis, University of Bristol, 1992. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.335366.
Texto completoMartinou, Jean-Claude. "Purification de motoneurones embryonnaires : caracterisation de facteurs de survie et de differenciation in vitro". Toulouse 3, 1988. http://www.theses.fr/1988TOU30140.
Texto completoKOUCHTIR, NEZHA. "Les motoneurones peroniers du chat : demonstration electrophysiologique d'une retroaction positive de la contraction musculaire". Paris 11, 1994. http://www.theses.fr/1994PA112281.
Texto completoTiret, Laurent. "Role du gene homeotique hoxc-8 dans l'identite des motoneurones brachiaux chez la souris". Paris 6, 1997. http://www.theses.fr/1997PA066568.
Texto completoCatone, Christelle. "Effets morphogènes et trophique de l'acetylcholine sur les motoneurones embryonnaires de rat en culture". Aix-Marseille 2, 2004. http://www.theses.fr/2004AIX22153.
Texto completoVouillot, Léna. "Développement des motoneurones chez un modèle amphibien d'amyotrophie spinale généré à l'aide de nucléases". Thesis, Evry-Val d'Essonne, 2014. http://www.theses.fr/2014EVRY0038.
Texto completoSpinal muscular atrophy (SMA) is a neurodegenerative disease characterized by motor neuron loss and skeletal muscle atrophy. In human the loss of function of the smn1 gene, the main supplier of survival motor neuron protein (SMN), leads to reduced levels of SMN and eventually to SMA. The anuran amphibian Xenopus tropicalis is a good animal model for the study of SMA and motor neurons development. Indeed the inhibition of the production of SMN using antisense morpholinos leads to caudal muscular atrophy in tadpoles. To develop an inheritable SMA model, we edited the smn gene in X. tropicalis using zinc-finger nucleases (ZFNs) and CRISPR/Cas system. As a first step, we designed the molecular tools needed to induce mutations of the smn gene using ZFN and CRISPR/cas9. Next we probed the efficiency of these tools and developed a method to identify mutations using T7EI and Surveyor endonucleases. We obtained a mutant frog and thus we will be able to produce homozygous mutant embryos for smn. In parallel we developed a transgenic line of Xenopus tropicalis frogs in which we can image motor neurons populations in vivo. The combination of both lines should enable to increase our knowledge and understanding of motor neuron physiopathology due to smn mutations
Martinou, Jean-Claude. "Purification de motoneurones embryonnaires caractérisation de facteurs de survie et de différenciation in vitro /". Grenoble 2 : ANRT, 1988. http://catalogue.bnf.fr/ark:/12148/cb37615925m.
Texto completoToader, Emil. "Activation parasympathique centrale mise en évidence par enregistrement des motoneurones cardiaques vagaux chez le rat". Phd thesis, Université Claude Bernard - Lyon I, 2008. http://tel.archives-ouvertes.fr/tel-00291046.
Texto completoHaase, Georg. "Thérapie génique de maladies dégénératives des motoneurones et transfert de gènes dans le nerf lésé". Paris 5, 1999. http://www.theses.fr/1999PA05CD05.
Texto completoToader, Emil-Codrut. "Activation parasympathique centrale mise en évidence par enregistrement des motoneurones cardiaques vagaux chez le rat". Lyon 1, 2008. http://tel.archives-ouvertes.fr/docs/00/29/10/46/PDF/ToaderE_08.pdf.
Texto completoThe parasympathetic supply to the heart is responsible for the short-term regulation of blood pressure (BP), by controlling the heart rate (HR) on a beat-by-beat basis. Decreased cardiac vagal activity is considered an index of poor outcome. The vagal projection of the nucleus ambiguus on the heart is the common pathway for different cardiac reflexes, like the cardiac baroreflex and the von Bezold-Jarisch (BJ) chemoreflex. Recent advances in extracellular recording methods of cardiac vagal motoneurons (CVM) in rat made easier the exploring of these two reflexes. Thus, a better understanding of the mechanisms and sites of action of two drugs could be achieved. 1). An α-2 adrenergic agonist, clonidine, a central hypotensive, influences the short-term regulation of BP. The present work shows an increase of the activity of CVM, together with the slope of the cardiac baroreflex at central level (BP-CVM relationship) when clonidine is administered systemically, in cumulative doses (10-100µg/kg i. V. ). A deeper analysis of the CVM activity revealed a possible new mechanism of action of clonidine by fast firing (“doublet”). 2). B-type natriuretic peptide (BNP), used in the treatment of the heart failure, increased significantly the bradycardia and the CVM activity during the activation of the BJ reflex. The bradycardia is proportional to the vagal activation. This proves, by subtractive logic, that the BNP site of action is on the afferent arm of the reflex. Cardiac vagal activation is a solution in the treatment of cardiovascular disorders
Funkelstein, Lydiane [Muriel]. "Contribution des sémaphorines au développement des motoneurones et dans la régénération de la moelle épinière". Aix-Marseille 2, 2005. http://theses.univ-amu.fr.lama.univ-amu.fr/2005AIX22016.pdf.
Texto completoPiazzon, Nathalie. "Rôle du complexe de Survie des MotoNeurones (SMN) dans la biogenèse des particules ARN/Protéines". Thesis, Nancy 1, 2008. http://www.theses.fr/2008NAN10063/document.
Texto completoSpinal muscular atrophy (SMA) is caused by reduced levels of the survival of motor neuron (SMN) protein. SMN protein is associated with the proteins Gemin 2 to 8 and unrip to form the SMN complex. Although the SMN protein is present in all cell types, SMA is restricted to a defect in motor neuron. SMN was recently proposed to have specific functions in mRNA transport and translation regulation in neuronal processes. The defective protein in Fragile X mental retardation syndrome (FMRP) also plays a role in transport of mRNPs and in their translation. In this study, we showed a link between the SMN complex and FMRP in neuronal cells suggesting a role for the SMN complex in these processes. Knowledges of the composition, interactions and functions of the SMN complex have advanced greatly in recent years. The emerging picture is that the SMN complex acts as a macromolecular chaperone of RNPs to increase the efficiency and fidelity of RNA–protein interactions, and to provide an opportunity for these interactions to be regulated. The second part of this study was to analyse the involvement of the SMN complex in the biogenesis of RNP different of UsnRNP. The specific defect of motor neuron led us to analyse the role of the SMN complex in the biogenesis of specific RNP to this cell types in particular the RNP BC200. Finally, we are also interested to the SMN complex involvement in the assembly and/or the function of the SRP particle, an ubiquitous particle
Martinot, Clemence. "Altérations des entrées synaptiques et origine de la vacuolisation dans les motoneurones de souris sod1g93a, modèle de la sclérose latérale amyotrophique". Thesis, Sorbonne Paris Cité, 2017. http://www.theses.fr/2017USPCB259/document.
Texto completoGlutamate excitotoxicity arising from excessive entry of calcium in the cell, has long been suggested to contribute to the degeneration of motoneurons in Amyotrophic Lateral Sclerosis (ALS). This hypothesis is enhanced by the observation of vacuoles on motoneurones dendritic tree. Such vacuoles were previously observed on neurons under excitotoxic stress. Excitotoxicity may stem from an intrinsic hyperexcitability of the motoneurons or from a shift of the balance of excitatory / inhibitory inputs received by the motoneurons toward more excitation. Thanks to an in vivo preparation that allows us to make intracellular recordings of motoneurons in adult mice, it was shown that spinal motoneurons do not display an intrinsic hyperexcitability just prior to their degeneration in SOD1 G93A mice, the standard model of ALS. Thus, to study excitotoxicity hypothesis, we decided to study dendritic vacuoles and undersand their genesis, and then to study synaptic inputs on motoneurons, to decipher if there is a hyperexcitability. We have shown, with intracellular labelling and immunohistochemistry, that vacuoles grow with age, that they appear in the intermembrane space of mitochondria, and that deficiency in autophagy prevent their elimination. With electrophysiological recordings, we have shown that monosynaptic EPSP amplitude is reduced in SOD1 mice. IPSP were less numerous and inhibitory interneurons were less excitable. These alterations were not due to synapses numbers, however synapses are preferentially localised on dendritic places that vacuolate, suggesting a link between synaptic activity and vacuolation. These results suggest that excitotoxicity might not be the mecanism of motoneuron death
Pambo-Pambo, Arnaud Brice. "Etude du développement postnatal des motoneurones lombaires de deux souches de souris transgéniques, modèles de la sclérose latérale amyotrophique". Thesis, Aix-Marseille 2, 2010. http://www.theses.fr/2010AIX20716.
Texto completoThe SOD1 murine models of Amyotrophic Lateral Sclerosis (ALS) allowed major progress in the understanding of mechanisms which could lead to a selective loss of motoneurons (Mns), but these models display differences in the severity and time course of the disease. Changes in intrinsic properties of motoneurons may induce changes in excitability and intracellular calcium homeostasis leading to motoneuron death.Therefore, we studied electrophysiological properties of lumbar Mns from SOD1G85R and SOD1G93A mice, low expressor lines, during the first two postnatal weeks in order to identify possible early presymptomatic abnormalities. Our studies were carried out on two in vitro preparations: the whole isolated spinal cord and acute spinal cord slices. Mutant Mns display, in the two preparations, a modified action potential characterized by an increased duration due to a decrease of the maximal speeds of depolarisation and repolarisation and a reduction of the spike amplitude. These alterations appeared between P2-P5 in SOD1G85R Mns and between P6-P10 in SOD1G93A Mns and suggest a decrease of the density of sodium and potassium channels related to action potential. We also showed on spinal cord slices between P6-P10 that the gain of frequency decreases for SOD1G85R Mns and increases for SOD1G93A Mns without any change in the density of persistent inward sodium or calcium currents in these different mutant Mns. We observed also that the resting membrane potential of SOD1G93A Mns on spinal cord slices is decreased. The membrane properties of SOD1G85R Mns between P6-P10 were less susceptible to changes in presence of an extracellular calcium overload. Differential effects of this extracellular calcium overload on membrane properties of WT and SOD1G85R Mns could be due to different alterations of the potential dependence of voltage-gated channels and/or to the modulation of some types of channels sensitive to extracellular calcium. An over-branching of dendritic arborization, similar to that previously described in SOD1G85R Mns, was observed in SOD1G93A at P8-P9 with the above-mentioned action potential alterations and a weak rheobasic current. These morphogical and electrical changes could indicate together alterations of kinetics and/or density of channels on different sites on these Mns. In conclusion, our work shows on one hand that SOD1G85R and SOD1G93A mutations induce similar alterations of lumbar Mns properties but time-shifted in these two murine models and on the other hand that some alterations seem to be specific to a given SOD1 mutation
Guigoni, Céline. "Infection in vitro des motoneurones spinaux par le virus de la rage : étude immunocytochimique et ultrastructurale". Aix-Marseille 3, 2002. http://www.theses.fr/2002AIX30046.
Texto completoRabies virus (RV), a neurotropic virus, infects only neurons in the nervous system. In this study, several stages of RV cycle were analyzed in purified spinal cord motoneuron (MNs) cultures through an immunocytochemical and ultrastructural method. Competitive adsorption experiments showed that RV may use NCAM and p75NTR molecules as receptors at the MNs surface. None the less, other unidentified molecules could play this role. Purified MNs survived RV infection and few of them became apoptotic. On the contrary, hippocampus neurons were susceptible to RV infection. These experiments suggest that MNs consist in a neuronal population which survive specifically RV infection. Electron microscopy observations suggest that MNs released RV particles at neurites by budding of cytolasmic membrane suggesting a polarized maturation of virals particles
Hivert, Bruno. "Approche électrophysiologique de l'étiologie de la sclérose latérale amyotrophique : effets de sérums sur motoneurones en culture". Angers, 1994. http://www.theses.fr/1994ANGE0013.
Texto completoAmendola, Julien. "Développement postnatal d'un modèle murin de sclérose latérale amyotrophique : Acquisitions sensori-motrices, fonctionnement des réseaux lombaires et caractérisation des propriétés électriques et morphologiques des motoneurones". Phd thesis, Université de la Méditerranée - Aix-Marseille II, 2008. http://tel.archives-ouvertes.fr/tel-00537888.
Texto completoDando, Simon Burke. "The role of 5-HT receptors in the modulation of the reflex activation of cardiac vagal motoneurones". Thesis, University College London (University of London), 1995. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.309288.
Texto completoGibbs, John Michael. "The organization of inputs to motoneurones studied in adults, during development and in children with cerebral palsy". Thesis, King's College London (University of London), 1996. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.338738.
Texto completoElson, R. C. "Identified flight interneurones and motoneurones of the locust that receive wing mechanoreceptor input : Physiology, structure and function". Thesis, University of Cambridge, 1985. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.354328.
Texto completoPearlstein, Edouard. "Organisation modulaire d'un réseau locomoteur : étude des motoneurones élévateurs et dépresseurs dans les appendices thoraciques de l'écrevisse". Aix-Marseille 3, 1996. http://www.theses.fr/1996AIX30011.
Texto completoLauney, Thomas. "Propriétés des récepteurs aux acides aminés excitateurs exprimés par les motoneurones crâniens de rat, en culture organotypique". Aix-Marseille 1, 1998. http://www.theses.fr/1998AIX11027.
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