16 research outputs found

    Early detachment of neuromuscular junction proteins in ALS mice with SODG93A mutation

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    The transgenic animals with mutant copper/zinc superoxide dismutase (SOD1) DNA develop paralytic motor neuron disease resembling human amyotrophic lateral sclerosis (ALS) patients and are commonly used as models for ALS. In the transgenic (Tg) mice with the G93A mutation of the human SOD1 gene SOD1G93A mice), the loss of ventral root axons and the synapses between the muscles and the motor neurons suggested that the motor neuron degeneration might proceed in a dying-back degeneration pattern. To reveal the relationship between axonal degeneration and the progression of the muscle atrophy in the SOD1G93A mice, we investigated the status of the neuromuscular junction along the disease progression. As a presynaptic or postsynaptic marker of neuromuscular junction (NMJ), anti-synaptic vesicle protein 2 (anti-SV2) antibody and α-bungarotoxin (α-BuTX) were chosen in this study and, as a marker of synaptic cleft, anti-agrin antibody was chosen in this study. In the immunohistochemistry of α-BuTX and anti-SV2 antibody, the percentages of double positive NMJs among α-BuTX single positive were decreased in Tg mice through time from ten weeks. The number of postsynaptic acethylcholine receptor (AChR) clusters did not decrease in Tg mice even at the end stage. Immunohistochemistry of α-BuTX and anti-agrin antibody revealed that the increase of immunopositive area of anti-agrin antibody around the muscle fiber in Tg mice from ten weeks of age. In this study, we revealed that the detachment of nerve terminals started at ten weeks in Tg mice. The levels of AChR did not change throughout 5–20 weeks of age in both groups of mice, and AChR remains clustering at NMJs, suggesting that the muscle abnormality is the result of detachment of nerve terminals

    Precision and Relative Accuracy of a Phased Array Doppler Sodar

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    We developed a phased array Doppler sodar and participated with the new sodar in the International Sodar Intercomparison Experiment (ISIE) at Boulder Atmospheric Observatory (BAO) in 1988. From the data obtained during this experiment, errors in wind measurement by the sodar were estimated by the method proposed for a microwave wind profiler by Strauch et al. The error in horizontal wind components was 0.35m/s at 75m and 0.60m/s at 300m when the mean wind speed was about 5m/s. The error is separated into random errors in radial velocity measurement of 0.29 and 0.47m/s at 75 and 300m, and errors caused by the assumption of uniform wind in the beam separation of 0.20 and 0.38m/s at 75 and 300m

    Microangiographic evaluation of the effects of heparin on progressive Masugi nephritis

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    Microangiographic evaluation of the effects of heparin on progressive Masugi nephritis. Unilateral progressive Masugi nephritis was produced in rabbits and studied by microangiography as well as light and immunofluorescence microscopy. The following five groups were studied: Group 1. Heparin was started simultaneously with nephrotoxic serum (NTS) and was given for one week. The animals were then sacrificed along with the untreated controls. Group 2. This was the same protocol as in group 1 but with three weeks' heparin. Group 3. Heparin was started one day after NTS and was given for three weeks. Group 4. Heparin was started one to two weeks after NTS and was given for three weeks. Group 5: late effect group. Heparin was started simultaneously with NTS, was given for four weeks, and the animals then were sacrificed 10 to 13 weeks later. Heparin dose was 5,000 U, s.c., per day in all treated groups. The number of glomeruli seen per unit of cortex by microangiography was significantly increased in the first through the third groups, as compared to the controls. Group 1 did not show this increase but there was some decrease of immunofluorescent fibrinogen. The late effect group (group 5) showed no modification by the treatment, suggesting that an initial improvement may have been negated by persistent immunologic insults after heparin withdrawal.Evaluation microangiographique des effets de l'héparine sur la néphrite progressive de Masugi. Une néphrite unilatérale de Masugi a été produite chez des lapins et étudiée par microangiographie et par microscopie photonique et immunofluorescence. Les cinq groupes suivants ont été étudiés: 1) groupe simultané une semaine, l'héparine est commencée en même temps que le sérum néphrotoxique (NTS) et administrée pendant une semaine, 2) groupe simultané trois semaines, semblable à groupe 1, mais l'administration d'héparine dure trois semaines, 3) groupe un jour, l'héparine est commencée un jour après NTS, et administrée pendant trois semaines, 4) groupe une semaine, l'héparine est commencée une à deux semaines après NTS et administrée pendant trois semaines, 5) groupe des effets tardifs, l'héparine est commencée en même temps que NTS, donnée pendant 4 semaines, les animaux sont sacrifiés 10 à 13 semaines plus tard. La dose d'héparine est de 5,000 U, s.c., par jour dans tous les groupes traités. Le nombre de glomérules vus par unité de cortex en microangiographie est significativement augmenté dans le premier et le troisième groupe par comparaison avec les contrôles. Le groupe une semaine n'a pas une telle augmentation, mais on observe une diminution de la fluorescence du fibrinogène. Le groupe des effets tardifs n'est pas modifié par le traitement, ce qui suggère que l'amélioration initiale peut avoir été effacée par la persistance du processus immunologique après l'arrêt de l'héparine

    Urinary Retention Suggesting Aseptic Meningitis: Meningitis-Retention Syndrome Without Physical Signs of Meningeal Irritation

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    Meningitis-retention syndrome (MRS) is the combination of aseptic meningitis and acute urinary retention that occurs in the absence of other neurological diseases. The cause(s) of MRS remain unclear. A 57-year-old Japanese woman was referred to our hospital for the evaluation of persistent fever and headache. The fever’s cause was initially unclear, but the presence of urinary retention raised concern about possible aseptic meningitis despite no physical indications of meningeal irritation. Only typical cases of MRS have been reported thus far to our knowledge, and it is important that clinicians are aware of MRS when it presents in this atypical form
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