RESUMO
The present work concerns the development of a W sources assessment system in the framework of the tungsten-W environment in steady state tokamak project that aims at equipping the existing Tore Supra device with a tungsten divertor in order to test actively cooled tungsten Plasma Facing Components (PFCs) in view of preparing ITER operation. The goal is to assess W sources and D recycling with spectral, spatial, and temporal resolution adapted to the PFCs observed. The originality of the system is that all optical elements are installed in the vacuum vessel and compatible with steady state operation. Our system is optimized to measure radiance as low as 1016 Ph/(m2 s sr). A total of 240 optical fibers will be deployed to the detection systems such as the "Filterscope," developed by Oak Ridge National Laboratory (USA) and consisting of photomultiplier tubes and filters, or imaging spectrometers dedicated to Multiview analysis.
RESUMO
A poorly understood feature of the tauopathies is their very different clinical presentations. The frontotemporal lobar degeneration (FTLD) spectrum is dominated by motor and emotional/psychiatric abnormalities, whereas cognitive and memory deficits are prominent in the early stages of Alzheimer's disease (AD). We report two novel mouse models overexpressing different human tau protein constructs. One is a full-length tau carrying a double mutation [P301S/G335D; line 66 (L66)] and the second is a truncated 3-repeat tau fragment which constitutes the bulk of the PHF core in AD corresponding to residues 296-390 fused with a signal sequence targeting it to the endoplasmic reticulum membrane (line 1; L1). L66 has abundant tau pathology widely distributed throughout the brain, with particularly high counts of affected neurons in hippocampus and entorhinal cortex. The pathology is neuroanatomically static and declines with age. Behaviourally, the model is devoid of a higher cognitive phenotype but presents with sensorimotor impairments and motor learning phenotypes. L1 displays a much weaker histopathological phenotype, but shows evidence of neuroanatomical spread and amplification with age that resembles the Braak staging of AD. Behaviourally, the model has minimal motor deficits but shows severe cognitive impairments affecting particularly the rodent equivalent of episodic memory which progresses with advancing age. In both models, tau aggregation can be dissociated from abnormal phosphorylation. The two models make possible the demonstration of two distinct but nevertheless convergent pathways of tau molecular pathogenesis. L1 appears to be useful for modelling the cognitive impairment of AD, whereas L66 appears to be more useful for modelling the motor features of the FTLD spectrum. Differences in clinical presentation of AD-like and FTLD syndromes are therefore likely to be inherent to the respective underlying tauopathy, and are not dependent on presence or absence of concomitant APP pathology.
