The Solar Neighborhood. XIX. Discovery and Characterization of 33 New Nearby White Dwarf Systems

We present spectra for 33 previously unclassified white dwarf systems brighter than V = 17 primarily in the southern hemisphere. Of these new systems, 26 are DA, 4 are DC, 2 are DZ, and 1 is DQ. We suspect three of these systems are unresolved double degenerates. We obtained VRI photometry for these 33 objects as well as for 23 known white dwarf systems without trigonometric parallaxes, also primarily in the southern hemisphere. For the 56 objects, we converted the photometry values to fluxes and fit them to a spectral energy distribution using the spectroscopy to determine which model to use (i.e. pure hydrogen, pure helium, or metal-rich helium), resulting in estimates of effective temperature and distance. Eight of the new and 12 known systems are estimated to be within the NStars and Catalogue of Nearby Stars (CNS) horizons of 25 pc, constituting a potential 18% increase in the nearby white dwarf sample. Trigonometric parallax determinations are underway via CTIOPI for these 20 systems. One of the DCs is cool so that it displays absorption in the near infrared. Using the distance determined via trigonometric parallax, we are able to constrain the model-dependent physical parameters and find that this object is most likely a mixed H/He atmosphere white dwarf similar to other cool white dwarfs identified in recent years with significant absorption in the infrared due to collision-induced absorptions by molecular hydrogen.Comment: 33 pages, 10 figures, accepted for publication in the Astronomical Journa

PABPN1 gene therapy for oculopharyngeal muscular dystrophy

International audienceOculopharyngeal muscular dystrophy (OPMD) is an autosomal dominant, late-onset muscle disorder characterized by ptosis, swallowing difficulties, proximal limb weakness and nuclear aggregates in skeletal muscles. OPMD is caused by a trinucleotide repeat expansion in the PABPN1 gene that results in an N-terminal expanded polyalanine tract in polyA-binding protein nuclear 1 (PABPN1). Here we show that the treatment of a mouse model of OPMD with an adeno-associated virus-based gene therapy combining complete knockdown of endogenous PABPN1 and its replacement by a wild-type PABPN1 substantially reduces the amount of insoluble aggregates, decreases muscle fibrosis, reverts muscle strength to the level of healthy muscles and normalizes the muscle transcriptome. The efficacy of the combined treatment is further confirmed in cells derived from OPMD patients. These results pave the way towards a gene replacement approach for OPMD treatment

The James Webb Space Telescope Mission

Twenty-six years ago a small committee report, building on earlier studies, expounded a compelling and poetic vision for the future of astronomy, calling for an infrared-optimized space telescope with an aperture of at least $4m$. With the support of their governments in the US, Europe, and Canada, 20,000 people realized that vision as the $6.5m$ James Webb Space Telescope. A generation of astronomers will celebrate their accomplishments for the life of the mission, potentially as long as 20 years, and beyond. This report and the scientific discoveries that follow are extended thank-you notes to the 20,000 team members. The telescope is working perfectly, with much better image quality than expected. In this and accompanying papers, we give a brief history, describe the observatory, outline its objectives and current observing program, and discuss the inventions and people who made it possible. We cite detailed reports on the design and the measured performance on orbit.Comment: Accepted by PASP for the special issue on The James Webb Space Telescope Overview, 29 pages, 4 figure

Longitudinal Imaging of the Ageing Mouse

Several non-invasive imaging techniques are used to investigate the effect of pathologies and treatments over time in mouse models. Each preclinical in vivo technique provides longitudinal and quantitative measurements of changes in tissues and organs, which are fundamental for the evaluation of alterations in phenotype due to pathologies, interventions and treatments. However, it is still unclear how these imaging modalities can be used to study ageing with mice models. Almost all age related pathologies in mice such as osteoporosis, arthritis, diabetes, cancer, thrombi, dementia, to name a few, can be imaged in vivo by at least one longitudinal imaging modality. These measurements are the basis for quantification of treatment effects in the development phase of a novel treatment prior to its clinical testing. Furthermore, the non-invasive nature of such investigations allows the assessment of different tissue and organ phenotypes in the same animal and over time, providing the opportunity to study the dysfunction of multiple tissues associated with the ageing process. This review paper aims to provide an overview of the applications of the most commonly used in vivo imaging modalities used in mouse studies: micro-computed-tomography, preclinical magnetic-resonance-imaging, preclinical positron-emission-tomography, preclinical single photon emission computed tomography, ultrasound, intravital microscopy, and whole body optical imaging