290 research outputs found

    Guillain-Barré Syndrome Animal Model: The First Proof of Molecular Mimicry in Human Autoimmune Disorder

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    Molecular mimicry between self and microbial components has been proposed as the pathogenic mechanism of autoimmune diseases, and this hypothesis is proven in Guillain-Barré syndrome. Guillain-Barré syndrome, the most frequent cause of acute neuromuscular paralysis, sometimes occurs after Campylobacter jejuni enteritis. Gangliosides are predominantly cell-surface glycolipids highly expressed in nervous tissue, whilst lipo-oligosaccharides are major components of the Gram-negative bacterium C. jejuni outer membrane. IgG autoantibodies to GM1 ganglioside were found in the sera from patients with Guillain-Barré syndrome. Molecular mimicry was demonstrated between GM1 and lipo-oligosaccharide of C. jejuni isolated from the patients. Disease models by sensitization of rabbits with GM1 and C. jejuni lipo-oligosaccharide were established. Guillain-Barré syndrome provided the first verification that an autoimmune disease is triggered by molecular mimicry. Its disease models are helpful to further understand the molecular pathogenesis as well as to develop new treatments in Guillain-Barré syndrome

    Conduction block in acute motor axonal neuropathy

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    Guillain-Barré syndrome is divided into two major subtypes, acute inflammatory demyelinating polyneuropathy and acute motor axonal neuropathy. The characteristic electrophysiological features of acute motor axonal neuropathy are reduced amplitude or absence of distal compound muscle action potentials indicating axonal degeneration. In contrast, autopsy study results show early nodal changes in acute motor axonal neuropathy that may produce motor nerve conduction block. Because the presence of conduction block in acute motor axonal neuropathy has yet to be fully recognized, we reviewed how often conduction block occurred and how frequently it either reversed or was followed by axonal degeneration. Based on Ho's criteria, acute motor axonal neuropathy was electrodiagnosed in 18 patients, and repeated motor nerve conduction studies were carried out on their median and ulnar nerves. Forearm segments of these nerves and the across-elbow segments of the ulnar nerve were examined to evaluate conduction block based on the consensus criteria of the American Association of Electrodiagnostic Medicine. Twelve (67%) of the 18 patients with acute motor axonal neuropathy had definite (n = 7) or probable (n = 5) conduction blocks. Definite conduction block was detected for one patient (6%) in the forearm segments of both nerves and probable conduction block was detected for five patients (28%). Definite conduction block was present across the elbow segment of the ulnar nerve in seven patients (39%) and probable conduction block in two patients (11%). Conduction block was reversible in seven of 12 patients and was followed by axonal degeneration in six. All conduction blocks had disappeared or begun to resolve within three weeks with no electrophysiological evidence of remyelination. One patient showed both reversible conduction block and conduction block followed by axonal degeneration. Clinical features and anti-ganglioside antibody profiles were similar in the patients with (n = 12) and without (n = 6) conduction block as well as in those with (n = 7) and without (n = 5) reversible conduction block, indicating that both conditions form a continuum; a pathophysiological spectrum ranging from reversible conduction failure to axonal degeneration, possibly mediated by antibody attack on gangliosides at the axolemma of the nodes of Ranvier, indicating that reversible conduction block and conduction block followed by axonal degeneration and axonal degeneration without conduction block constitute continuous electrophysiological conditions in acute motor axonal neuropath

    L’uniformisation des productions agricoles à l’époque d’Edo : des savoirs géographiques biaisés à l’origine de crises

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    A partir de l’analyse d’ouvrages édités à l’attention des masses populaires, de listes de productions et de traités agricoles, l’article propose une lecture critique des politiques agricoles mises en œuvre au Japon entre le début du XVIIe siècle et la fin du XIXe siècle. Il apparaît qu’au cours de cette période le pays a encouragé une uniformisation des productions agricoles sur l’ensemble de son territoire suite à l’application de savoirs importés de la Chine des Ming (1368-1644). Or, la capitale de l’empire Ming (Nankin) était située dans une région où les conditions climatiques subtropicales humides correspondaient à celles de l’Ouest du Japon, mais certainement pas à celles du Nord-est où les effets des famines ont été paradoxalement amplifiés. Cette aberration géographique peut s’expliquer à la fois par la sociologie de l’élite politique et par l’importance des représentations alimentaires du Kansai dans l’ensemble de la société d’Edo.Using works published for a popular readership, lists of products and agricultural treaties, this article proposes a critic of the agricultural policies implemented in Japan from the early seventeenth century to the late nineteenth century. It appears that the country practiced at this time a standardized agricultural production throughout its territory following the application of a knowledge imported from Ming China (1368-1644). But the capital of the Ming Empire (Nanking) was located in a region of China where the subtropical climate conditions matched western Japan, but certainly not to the Northeast areas where the effects of famine were amplified. This geographical misunderstanding can be explained by the sociology of the Edo’s political elite and by the importance of Kansai’s food habits representations in the whole Edo society

    Laser-induced-fluorescence measurement of thermal conductivity in warm dense matter generated by pulsed-power discharge

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    Thermal conductivity in warm dense matter is one of the interests for thermonuclear fusion scenarios. Alternative inertial confinement fusion, which is a fast ignition with applied magnetic field [1], has been considered to improve the coupling efficiency. The target behavior of the fast ignition with applied magnetic field depends on the anisotropic thermal conductivity. The magnetic confinement fusion (MCF) [2] Up to now, the heat load on the divertor in previous MCF systems has been unreached parameter. Thus, to predict properties of the divertor under these heat loads, several experiments have been performed using several methods[3-6]. To predict the performance of the tungsten divertor in MCF, we should analyze not only metallurgical properties but also thermophysical properties of ablated tungsten..

    Schwann cells are activated by ATP released from neurons in an in vitro cellular model of Miller Fisher syndrome

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    The neuromuscular junction is exposed to different types of insult, including mechanical trauma, toxins and autoimmune antibodies and, accordingly, has retained through evolution a remarkable ability to regenerate. Regeneration is driven by multiple signals that are exchanged among the cellular components of the junction. These signals are largely unknown. Miller Fisher syndrome is a variant of Guillain-Barr\ue9 syndrome caused by autoimmune antibodies specific for epitopes of peripheral axon terminals. Using an animal model of Miller Fisher syndrome, we recently reported that a monoclonal anti-polysialoganglioside GQ1b antibody plus complement damages nerve terminals with production of mitochondrial hydrogen peroxide, which activates Schwann cells. Several additional signaling molecules are likely to be involved in the activation of the regeneration program in these cells. Using an in vitro cellular model consisting of co-cultured primary neurons and Schwann cells, we found that ATP is released by neurons injured by the anti-GQ1b antibody plus complement. Neuron-derived ATP acts as an alarm messenger for Schwann cells, where it induces the activation of intracellular pathways, including calcium signaling, cAMP and CREB, which, in turn, produce signals that promote nerve regeneration. These results contribute to defining the cross-talk taking place at the neuromuscular junction when it is attacked by anti-gangliosides autoantibodies plus complement, which is crucial for nerve regeneration and is also likely to be important in other peripheral neuropathies

    Laser-induced-fluorescence measurement of thermal conductivity in warm dense matter generated by pulsed-power discharge

    Get PDF
    Thermal conductivity in warm dense matter is one of the interests for thermonuclear fusion scenarios. Alternative inertial confinement fusion, which is a fast ignition with applied magnetic field [1], has been considered to improve the coupling efficiency. The target behavior of the fast ignition with applied magnetic field depends on the anisotropic thermal conductivity. The magnetic confinement fusion (MCF) [2] Up to now, the heat load on the divertor in previous MCF systems has been unreached parameter. Thus, to predict properties of the divertor under these heat loads, several experiments have been performed using several methods[3-6]. To predict the performance of the tungsten divertor in MCF, we should analyze not only metallurgical properties but also thermophysical properties of ablated tungsten..
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