COS cell expression studies of P86L, P86R, P480L and P480Q Hunter's disease-causing mutations

AbstractThree missense mutations identified in the IDS gene of our Hunter's disease patients (P86L, P480L and P480Q) and the previously described P86R mutation were expressed in COS cells to evaluate their functional consequence on iduronate-2-sulfatase (IDS) activity and processing. The 86-proline residue belongs to the highly conserved pentapeptide C-X-P-S-R in which cysteine modification to a formylglycine is required for sulfatase activity. The substitution of the 86-proline residue led to a severe mutation as no mature form was targeted to the lysosome in agreement with the severe phenotype observed in patients carrying P86L and P86R mutations. Expression studies with P480L and P480Q mutant cDNAs showed the presence of a small amount of 55 kDa mature form in the lysosomes of transfected COS cells. IDS activity of the P480L and P480Q mutants in cell extracts represents 16.6% and 5.4% of the wild-type, respectively

Balanced homodyne detection of Bragg microhologramms in photopolymer for data storage

Wavelength multiplexed holographic bit oriented memories are serious competitors for high capacity data storage systems. For data recording, two interfering beams are required whereas one of them should be blocked for readout in previously proposed systems. This makes the system complex. To circumvent this difficulty and make the device simpler, we validated an architecture for such memories in which the same two beams are used for recording and reading out. This balanced homodyne scheme is validated by recording holograms in a Lippmann architecture

Vers une automatisation du contrôle visuel des produits

National audienc

Age-related changes in circadian sleep-wake regulation: Impact on cognitive performance and cerebral correlates

peer reviewe

Design, pointing control, and on-sky performance of the mid-infrared vortex coronagraph for the VLT/NEAR experiment

Vortex coronagraphs have been shown to be a promising avenue for high- contrast imaging in the close-in environment of stars at thermal infrared (IR) wavelengths. They are included in the baseline design of the mid-infrared extremely large telescope imager and spectrograph. To ensure good performance of these coronagraphs, a precise control of the centering of the star image in real time is needed. We previously developed and validated the quadrant analysis of coronagraphic images for tip-tilt sensing estimator (QACITS) pointing estimator to address this issue. While this approach is not wavelength-dependent in theory, it was never implemented for mid-IR observations, which leads to specific challenges and limitations. Here, we present the design of the mid-IR vortex coronagraph for the "new Earths in the α Cen Region (NEAR) experiment with the Very Large Telescope (VLT)/Very Large Telescope imager and spectrometer for the mid-infrared (VISIR) instrument and assess the performance of the QACITS estimator for the centering control of the star image onto the vortex coronagraph. We use simulated data and on-sky data obtained with VLT/VISIR, which was recently upgraded for observations assisted by adaptive optics in the context of the NEAR experiment. We demonstrate that the QACITS-based correction loop is able to control the centering of the star image onto the NEAR vortex coronagraph with a stability down to 0.015 λ / D rms over 4 h in good conditions. These results show that QACITS is a robust approach for precisely controlling in real time the centering of vortex coronagraphs for mid-IR observations.Peer reviewe

COVID-19 symptoms at hospital admission vary with age and sex: results from the ISARIC prospective multinational observational study

Background: The ISARIC prospective multinational observational study is the largest cohort of hospitalized patients with COVID-19. We present relationships of age, sex, and nationality to presenting symptoms. Methods: International, prospective observational study of 60 109 hospitalized symptomatic patients with laboratory-confirmed COVID-19 recruited from 43 countries between 30 January and 3 August 2020. Logistic regression was performed to evaluate relationships of age and sex to published COVID-19 case definitions and the most commonly reported symptoms. Results: ‘Typical’ symptoms of fever (69%), cough (68%) and shortness of breath (66%) were the most commonly reported. 92% of patients experienced at least one of these. Prevalence of typical symptoms was greatest in 30- to 60-year-olds (respectively 80, 79, 69%; at least one 95%). They were reported less frequently in children (≤ 18 years: 69, 48, 23; 85%), older adults (≥ 70 years: 61, 62, 65; 90%), and women (66, 66, 64; 90%; vs. men 71, 70, 67; 93%, each P < 0.001). The most common atypical presentations under 60 years of age were nausea and vomiting and abdominal pain, and over 60 years was confusion. Regression models showed significant differences in symptoms with sex, age and country. Interpretation: This international collaboration has allowed us to report reliable symptom data from the largest cohort of patients admitted to hospital with COVID-19. Adults over 60 and children admitted to hospital with COVID-19 are less likely to present with typical symptoms. Nausea and vomiting are common atypical presentations under 30 years. Confusion is a frequent atypical presentation of COVID-19 in adults over 60 years. Women are less likely to experience typical symptoms than men

IMPACT DE TROIS TYPES D'ALIMENTATION SUR LA CROISSANCE DE LYMNAEA TRUNCATULA MULLER ET LA PRODUCTION CERCARIENNE DE TROIS DIGENES

LIMOGES-BU Médecine pharmacie (870852108) / SudocLYON1-BU Santé (693882101) / SudocSudocFranceF

Mémoires holographiques microstructurées à réseaux de Lippmann

Le stockage holographique de données est actuellement une voie très prometteuse pour accroître de manière conséquente les capacités et débits des mémoires optiques amovibles. L'architecture de mémoire holographique visée à long terme par ces travaux consiste en une couche de données structurée en une matrice de microfibres perpendiculaires au disque. Les hologrammes sont des réseaux de Bragg multiplexes en longueur d'onde dans chaque microfibre. Le matériau d'enregistrement envisagé est un photopolymère dopé par des centres moléculaires bistables, le photopolymère servant la structuration en microfibres et les centres à l'enregistrement des réseaux. L'impact de la répartition discrète de ces centres et de la non linéarité de leur réponse optique sur le rapport signal sur bruit de lecture a tout d'abord été évalué par de premières modélisations. Par ailleurs, une procédure d'écriture-lecture en configuration de type Lippmann a été définie afin de simplifier l'architecture de la mémoire et accroître le signal de lecture grâce à une détection homodyne des hologrammes. La mise en œuvre pratique de cette procédure a alors par la suite été étudiée au travers de deux dispositifs expérimentaux d'écriture-lecture. Le premier montage a ainsi abouti à une démonstration de la procédure dans une configuration simplifiée, limitant les difficultés relatives au matériau et à la géométrie d'enregistrement, en bon accord avec notre modèle théorique. L'acquis obtenu avec cette première démonstration a ensuite été utilisé pour mettre en place un second dispositif afin de valider notre procédure dans une configuration de stockage optique plus réaliste.Holographic data storage is presently very promising to strongly increase the capacities and data rates of removable optical memories. The architecture of holographic memory targeted in the long term by the present work consists in a data layer that is structured into a matrix of microfibers set perpendicular to the disk surface. Holograms are Bragg gratings that are wavelength multiplexed in each microfiber. The targeted storage medium is a photopolymer doped with bistable molecular centers : the photopolymer is used for the microfiber structuration and the bistable centers for the grating recording. The influence of the discrete distribution of the centers and of the non-linearity of their optical response on the readout signal to noise ratio has firstly been estimated thanks to preliminary modeling work. Besides, a recording-readout procedure based on a Lippmann type configuration has been defined so as to simplify the memory architecture and increase the readout signal thanks to a homodyne detection of the holograms. The practical implementation of this procedure has been studied afterwards thanks to two experimental recording-readout setups. The first one has led to a demonstration of the procedure in a simplified configuration, limiting the difficulties arising from the recording material and geometry, that shows good agreement with our theoretical model. The results obtained have then been used to build a second setup to validate our procedure in a more realistic data storage configuration.ORSAY-PARIS 11-BU Sciences (914712101) / SudocSudocFranceF