Multicenter evaluation of use of dried blood spot compared to conventional plasma in measurements of globotriaosylsphingosine (LysoGb3) concentration in 104 Fabry patients.

Abstract Objectives Fabry disease (FD) is an X-linked lysosomal storage disorder, resulting from a deficiency of the enzyme α-galactosidase A, responsible for breaking down glycolipids such as globotriaosylceramide and its deacylated derivative, globotriaosylsphingosine (LysoGb3). Here, we compare the levels of LysoGb3 in dried blood spots (DBS) and plasma in patients with classic and late-onset phenotypes. Methods LysoGb3 measurements were performed in 104 FD patients, 39 males and 65 females. Venous blood was collected. A portion was spotted onto filter paper and another portion separated to obtain plasma. The LysoGb3 concentrations in DBS and plasma were determined by highly sensitive electrospray ionization liquid chromatography tandem mass spectrometry. Agreement between different matrices was assessed using linear regression and Bland Altman analysis. Results The method on DBS was validated by evaluating its precision, accuracy, matrix effect, recovery, and stability. The analytical performances were verified by comparison of a total of 104 paired DBS and plasma samples from as many FD patients (representing 46 GLA variants). There was a strong correlation between plasma and the corresponding DBS LysoGb3 concentrations, with few exceptions. Discrepancies were observed in anemic patients with typically low hematocrit levels compared to the normal range. Conclusions The method proved to be efficient for the rapid analysis of LysoGb3. DBS provides a convenient, sensitive, and reproducible method for measuring LysoGb3 levels for diagnosis, initial phenotypic assignment, and therapeutic monitoring in patients with FD

ESPRESSO: The next European exoplanet hunter

The acronym ESPRESSO stems for Echelle SPectrograph for Rocky Exoplanets and Stable Spectroscopic Observations; this instrument will be the next VLT high resolution spectrograph. The spectrograph will be installed at the Combined-Coud\'e Laboratory of the VLT and linked to the four 8.2 m Unit Telescopes (UT) through four optical Coud\'e trains. ESPRESSO will combine efficiency and extreme spectroscopic precision. ESPRESSO is foreseen to achieve a gain of two magnitudes with respect to its predecessor HARPS, and to improve the instrumental radial-velocity precision to reach the 10 cm/s level. It can be operated either with a single UT or with up to four UTs, enabling an additional gain in the latter mode. The incoherent combination of four telescopes and the extreme precision requirements called for many innovative design solutions while ensuring the technical heritage of the successful HARPS experience. ESPRESSO will allow to explore new frontiers in most domains of astrophysics that require precision and sensitivity. The main scientific drivers are the search and characterization of rocky exoplanets in the habitable zone of quiet, nearby G to M-dwarfs and the analysis of the variability of fundamental physical constants. The project passed the final design review in May 2013 and entered the manufacturing phase. ESPRESSO will be installed at the Paranal Observatory in 2016 and its operation is planned to start by the end of the same year.Comment: 12 pages, figures included, accepted for publication in Astron. Nach

Light Isotope Abundances in Solar Energetic Particles measured by the Space Instrument NINA

This article reports nine Solar Energetic Particle events detected by the instrument NINA between October 1998 and April 1999. NINA is a silicon-based particle detector mounted on-board the Russian satellite Resurs-01-N4, which has flown at an altitude of about 800 km in polar inclination since July 1998. For every solar event the power-law He4 spectrum across the energy interval 10--50 MeV/n was reconstructed, and spectral indexes, gamma, from 1.8 to 6.8 extracted. Data of He3 and He4 were used to determine the He3/He4 ratio, that for some SEP events indicated an enrichment in He3. For the 1998 November 7 event the ratio reached a maximum value of 0.33+- 0.06, with spectral indexes of gamma = 2.5 +- 0.6 and gamma = 3.7 +- 0.3 for He3 and He4, respectively. The He3/He4 ratio averaged over the remaining events was 0.011 +- 0.004. For all events the deuterium-to-proton ratio was determined. The average value over all events was (3.9+-1.4) 10^{-5} across the energy interval 9--12 MeV/n. For one event (1998 November 24) this ratio yielded approximately 10 times higher than normal coronal values. Upper limits on the H3/H1 counting ratio for all events were determined. For the 1998 November 14 SEP event the high flux of heavy particles detected made it possible to reconstruct the carbon and oxygen flux.Comment: 42 pages, 14 figures, submitted to Journal of Geophysical Researc

Revisiting Proxima with ESPRESSO

We aim to confirm the presence of Proxima b using independent measurements obtained with the new ESPRESSO spectrograph, and refine the planetary parameters taking advantage of its improved precision. We analysed 63 spectroscopic ESPRESSO observations of Proxima taken during 2019. We obtained radial velocity measurements with a typical radial velocity photon noise of 26 cm/s. We ran a joint MCMC analysis on the time series of the radial velocity and full-width half maximum of the cross-correlation function to model the planetary and stellar signals present in the data, applying Gaussian process regression to deal with stellar activity. We confirm the presence of Proxima b independently in the ESPRESSO data. The ESPRESSO data on its own shows Proxima b at a period of 11.218 $\pm$ 0.029 days, with a minimum mass of 1.29 $\pm$ 0.13 Me. In the combined dataset we measure a period of 11.18427 $\pm$ 0.00070 days with a minimum mass of 1.173 $\pm$ 0.086 Me. We find no evidence of stellar activity as a potential cause for the 11.2 days signal. We find some evidence for the presence of a second short-period signal, at 5.15 days with a semi-amplitude of merely 40 cm/s. If caused by a planetary companion, it would correspond to a minimum mass of 0.29 $\pm$ 0.08 Me. We find that the FWHM of the CCF can be used as a proxy for the brightness changes and that its gradient with time can be used to successfully detrend the radial velocity data from part of the influence of stellar activity. The activity-induced radial velocity signal in the ESPRESSO data shows a trend in amplitude towards redder wavelengths. Velocities measured using the red end of the spectrograph are less affected by activity, suggesting that the stellar activity is spot-dominated. The data collected excludes the presence of extra companions with masses above 0.6 Me at periods shorter than 50 days.Comment: 25 pages, 26 figure

A precise architecture characterization of the π\pi Men planetary system

The bright star $\pi$ Men was chosen as the first target for a radial velocity follow-up to test the performance of ESPRESSO, the new high-resolution spectrograph at the ESO's Very-Large Telescope (VLT). The star hosts a multi-planet system (a transiting 4 M$_\oplus$ planet at $\sim$0.07 au, and a sub-stellar companion on a $\sim$2100-day eccentric orbit) which is particularly appealing for a precise multi-technique characterization. With the new ESPRESSO observations, that cover a time span of 200 days, we aim to improve the precision and accuracy of the planet parameters and search for additional low-mass companions. We also take advantage of new photometric transits of $\pi$ Men c observed by TESS over a time span that overlaps with that of the ESPRESSO follow-up campaign. We analyse the enlarged spectroscopic and photometric datasets and compare the results to those in the literature. We further characterize the system by means of absolute astrometry with Hipparcos and Gaia. We used the spectra of ESPRESSO for an independent determination of the stellar fundamental parameters. We present a precise characterization of the planetary system around $\pi$ Men. The ESPRESSO radial velocities alone (with typical uncertainty of 10 cm/s) allow for a precise retrieval of the Doppler signal induced by $\pi$ Men c. The residuals show an RMS of 1.2 m/s, and we can exclude companions with a minimum mass less than $\sim$2 M$_\oplus$ within the orbit of $\pi$ Men c). We improve the ephemeris of $\pi$ Men c using 18 additional TESS transits, and in combination with the astrometric measurements, we determine the inclination of the orbital plane of $\pi$ Men b with high precision ($i_{b}=45.8^{+1.4}_{-1.1}$ deg). This leads to the precise measurement of its absolute mass $m_{b}=14.1^{+0.5}_{-0.4}$ M$_{Jup}$, and shows that the planetary orbital planes are highly misaligned.Comment: Accepted for publication on A&

CUBES: a UV spectrograph for the future

In spite of the advent of extremely large telescopes in the UV/optical/NIR range, the current generation of 8-10m facilities is likely to remain competitive at ground-UV wavelengths for the foreseeable future. The Cassegrain U-Band Efficient Spectrograph (CUBES) has been designed to provide high-efficiency (>40%) observations in the near UV (305-400 nm requirement, 300-420 nm goal) at a spectral resolving power of R>20,000, although a lower-resolution, sky-limited mode of R ~ 7,000 is also planned. CUBES will offer new possibilities in many fields of astrophysics, providing access to key lines of stellar spectra: a tremendous diversity of iron-peak and heavy elements, lighter elements (in particular Beryllium) and light-element molecules (CO, CN, OH), as well as Balmer lines and the Balmer jump (particularly important for young stellar objects). The UV range is also critical in extragalactic studies: the circumgalactic medium of distant galaxies, the contribution of different types of sources to the cosmic UV background, the measurement of H2 and primordial Deuterium in a regime of relatively transparent intergalactic medium, and follow-up of explosive transients. The CUBES project completed a Phase A conceptual design in June 2021 and has now entered the Phase B dedicated to detailed design and construction. First science operations are planned for 2028. In this paper, we briefly describe the CUBES project development and goals, the main science cases, the instrument design and the project organization and management

T-REX OU4 HIRES: the high resolution spectrograph for the E-ELT

The goal of this unit was to consolidate the project for the construction of the high resolution spectrometer of the E-ELT (HIRES). The task included the development of scientific cases and tools to predict the instrumental performances. From the technical point of view it included several R&D activities in collaboration with highly specialized Italian companies; it culminated with the detailed design of a highly modular instrument based on well established technologies. From the management point of view it lead to the consolidation of a large international consortium that spans over 12 countries and includes most of the European and ESO-related institutes interested in high resolution spectroscopy. This consortium is led by INAF; its formal creation is awaiting the official call by ESO for the phase-A study for the HIRES instrument of the E-ELT

The European Solar Telescope

The European Solar Telescope (EST) is a project aimed at studying the magnetic connectivity of the solar atmosphere, from the deep photosphere to the upper chromosphere. Its design combines the knowledge and expertise gathered by the European solar physics community during the construction and operation of state-of-the-art solar telescopes operating in visible and near-infrared wavelengths: the Swedish 1m Solar Telescope, the German Vacuum Tower Telescope and GREGOR, the French Télescope Héliographique pour l'Étude du Magnétisme et des Instabilités Solaires, and the Dutch Open Telescope. With its 4.2 m primary mirror and an open configuration, EST will become the most powerful European ground-based facility to study the Sun in the coming decades in the visible and near-infrared bands. EST uses the most innovative technological advances: the first adaptive secondary mirror ever used in a solar telescope, a complex multi-conjugate adaptive optics with deformable mirrors that form part of the optical design in a natural way, a polarimetrically compensated telescope design that eliminates the complex temporal variation and wavelength dependence of the telescope Mueller matrix, and an instrument suite containing several (etalon-based) tunable imaging spectropolarimeters and several integral field unit spectropolarimeters. This publication summarises some fundamental science questions that can be addressed with the telescope, together with a complete description of its major subsystems