Altered Cortical Microarchitecture and Bone Metabolism in Patients with Monoclonal Gammopathy of Undetermined Significance

Patients with monoclonal gammopathy of undetermined significance (MGUS) are at increased fracture risk, and we have previously shown that MGUS patients have altered trabecular bone microarchitecture compared with controls. However, there are no data on whether the porosity of cortical bone, which may play a greater role in bone strength and the occurrence of fractures, is increased in MGUS. Thus, we studied cortical porosity and bone strength (apparent modulus) using high-resolution peripheral quantitative computed tomography imaging of the distal radius in 50 MGUS patients and 100 age-, gender-, and body mass index–matched controls. Compared with controls, MGUS patients had both significantly higher cortical porosity (+16.8%; P < .05) and lower apparent modulus (–8.9%; P < .05). Despite their larger radial bone size, MGUS patients have significantly increased cortical bone porosity and reduced bone strength relative to controls. This increased cortical porosity may explain the increased fracture risk seen in MGUS patients

Investigating the flow dynamics in the obstructed and stented ureter by means of a biomimetic artificial model

Double-J stenting is the most common clinical method employed to restore the upper urinary tract drainage, in the presence of a ureteric obstruction. After implant, stents provide an immediate pain relief by decreasing the pressure in the renal pelvis (P). However, their long-term usage can cause infections and encrustations, due to bacterial colonization and crystal deposition on the stent surface, respectively. The performance of double-J stents - and in general of all ureteric stents - is thought to depend significantly on urine flow field within the stented ureter. However very little fundamental research about the role played by fluid dynamic parameters on stent functionality has been conducted so far. These parameters are often difficult to assess in-vivo, requiring the implementation of laborious and expensive experimental protocols. The aim of the present work was therefore to develop an artificial model of the ureter (i.e. ureter model, UM) to mimic the fluid dynamic environment in a stented ureter. The UM was designed to reflect the geometry of pig ureters, and to investigate the values of fluid dynamic viscosity (μ), volumetric flow rate (Q ) and severity of ureteric obstruction (OB%) which may cause critical pressures in the renal pelvis. The distributed obstruction derived by the sole stent insertion was also quantified. In addition, flow visualisation experiments and computational simulations were performed in order to further characterise the flow field in the UM. Unique characteristics of the flow dynamics in the obstructed and stented ureter have been revealed with using the developed UM

A serendipitous all sky survey for bright objects in the outer solar system

We use seven yearʼs worth of observations from the Catalina Sky Survey and the Siding Spring Survey covering most of the northern and southern hemisphere at galactic latitudes higher than 20° to search for serendipitously imaged moving objects in the outer solar system. These slowly moving objects would appear as stationary transients in these fast cadence asteroids surveys, so we develop methods to discover objects in the outer solar system using individual observations spaced by months, rather than spaced by hours, as is typically done. While we independently discover eight known bright objects in the outer solar system, the faintest having $V=19.8\pm 0.1,$ no new objects are discovered. We find that the survey is nearly 100% efficient at detecting objects beyond 25 AU for $V\lesssim 19.1$ ($V\lesssim 18.6$ in the southern hemisphere) and that the probability that there is one or more remaining outer solar system object of this brightness left to be discovered in the unsurveyed regions of the galactic plane is approximately 32%

Dust Devil Tracks

Dust devils that leave dark- or light-toned tracks are common on Mars and they can also be found on the Earth’s surface. Dust devil tracks (hereinafter DDTs) are ephemeral surface features with mostly sub-annual lifetimes. Regarding their size, DDT widths can range between ∼1 m and ∼1 km, depending on the diameter of dust devil that created the track, and DDT lengths range from a few tens of meters to several kilometers, limited by the duration and horizontal ground speed of dust devils. DDTs can be classified into three main types based on their morphology and albedo in contrast to their surroundings; all are found on both planets: (a) dark continuous DDTs, (b) dark cycloidal DDTs, and (c) bright DDTs. Dark continuous DDTs are the most common type on Mars. They are characterized by their relatively homogenous and continuous low albedo surface tracks. Based on terrestrial and martian in situ studies, these DDTs most likely form when surficial dust layers are removed to expose larger-grained substrate material (coarse sands of ≥500 μm in diameter). The exposure of larger-grained materials changes the photometric properties of the surface; hence leading to lower albedo tracks because grain size is photometrically inversely proportional to the surface reflectance. However, although not observed so far, compositional differences (i.e., color differences) might also lead to albedo contrasts when dust is removed to expose substrate materials with mineralogical differences. For dark continuous DDTs, albedo drop measurements are around 2.5 % in the wavelength range of 550–850 nm on Mars and around 0.5 % in the wavelength range from 300–1100 nm on Earth. The removal of an equivalent layer thickness around 1 μm is sufficient for the formation of visible dark continuous DDTs on Mars and Earth. The next type of DDTs, dark cycloidal DDTs, are characterized by their low albedo pattern of overlapping scallops. Terrestrial in situ studies imply that they are formed when sand-sized material that is eroded from the outer vortex area of a dust devil is redeposited in annular patterns in the central vortex region. This type of DDT can also be found in on Mars in orbital image data, and although in situ studies are lacking, terrestrial analog studies, laboratory work, and numerical modeling suggest they have the same formation mechanism as those on Earth. Finally, bright DDTs are characterized by their continuous track pattern and high albedo compared to their undisturbed surroundings. They are found on both planets, but to date they have only been analyzed in situ on Earth. Here, the destruction of aggregates of dust, silt and sand by dust devils leads to smooth surfaces in contrast to the undisturbed rough surfaces surrounding the track. The resulting change in photometric properties occurs because the smoother surfaces have a higher reflectance compared to the surrounding rough surface, leading to bright DDTs. On Mars, the destruction of surficial dust-aggregates may also lead to bright DDTs. However, higher reflective surfaces may be produced by other formation mechanisms, such as dust compaction by passing dust devils, as this may also cause changes in photometric properties. On Mars, DDTs in general are found at all elevations and on a global scale, except on the permanent polar caps. DDT maximum areal densities occur during spring and summer in both hemispheres produced by an increase in dust devil activity caused by maximum insolation. Regionally, dust devil densities vary spatially likely controlled by changes in dust cover thicknesses and substrate materials. This variability makes it difficult to infer dust devil activity from DDT frequencies. Furthermore, only a fraction of dust devils leave tracks. However, DDTs can be used as proxies for dust devil lifetimes and wind directions and speeds, and they can also be used to predict lander or rover solar panel clearing events. Overall, the high DDT frequency in many areas on Mars leads to drastic albedo changes that affect large-scale weather patterns

The first ultracompact Roche lobe-filling hot subdwarf binary

We report the discovery of the first short period binary in which a hot subdwarf star (sdOB) fills its Roche lobe and started mass transfer to its companion. The object was discovered as part of a dedicated high-cadence survey of the Galactic Plane named the Zwicky Transient Facility and exhibits a period of Porb=39.3401(1) min, making it the most compact hot subdwarf binary currently known. Spectroscopic observations are consistent with an intermediate He-sdOB star with an effective temperature of Teff=42,400±300 K and a surface gravity of log(g)=5.77±0.05. A high-signal-to noise GTC+HiPERCAM light curve is dominated by the ellipsoidal deformation of the sdOB star and an eclipse of the sdOB by an accretion disk. We infer a low-mass hot subdwarf donor with a mass MsdOB=0.337±0.015 M⊙ and a white dwarf accretor with a mass MWD=0.545±0.020 M⊙. Theoretical binary modeling indicates the hot subdwarf formed during a common envelope phase when a 2.5−2.8 M⊙ star lost its envelope when crossing the Hertzsprung Gap. To match its current Porb, Teff, log(g), and masses, we estimate a post-common envelope period of Porb≈150 min, and find the sdOB star is currently undergoing hydrogen shell burning. We estimate that the hot subdwarf will become a white dwarf with a thick helium layer of ≈0.1 M⊙ and will merge with its carbon/oxygen white dwarf companion after ≈17 Myr and presumably explode as a thermonuclear supernova or form an R CrB star

Luminous Red Novae: Stellar Mergers or Giant Eruptions?

We present extensive datasets for a class of intermediate-luminosity optical transients known as luminous red novae. They show double-peaked light curves, with an initial rapid luminosity rise to a blue peak (at -13 to -15 mag), which is followed by a longer-duration red peak that sometimes is attenuated, resembling a plateau. The progenitors of three of them (NGC 4490-2011OT1, M 101-2015OT1, and SNhunt248), likely relatively massive blue to yellow stars, were also observed in a pre-eruptive stage when their luminosity was slowly increasing. Early spectra obtained during the first peak show a blue continuum with superposed prominent narrow Balmer lines, with P Cygni profiles. Lines of Fe II are also clearly observed, mostly in emission. During the second peak, the spectral continuum becomes much redder, H alpha is barely detected, and a forest of narrow metal lines is observed in absorption. Very late-time spectra (similar to 6 months after blue peak) show an extremely red spectral continuum, peaking in the infrared (IR) domain. H alpha is detected in pure emission at such late phases, along with broad absorption bands due to molecular overtones (such as TiO, VO). We discuss a few alternative scenarios for luminous red novae. Although major instabilities of single massive stars cannot be definitely ruled out, we favour a common envelope ejection in a close binary system, with possibly a final coalescence of the two stars. The similarity between luminous red novae and the outburst observed a few months before the explosion of the Type IIn SN 2011ht is also discussed

Characterization of HNRNPA1 mutations defines diversity in pathogenic mechanisms and clinical presentation

Mutations in HNRNPA1 encoding heterogeneous nuclear ribonucleoprotein (hnRNP) A1 are a rare cause of amyotrophic lateral sclerosis (ALS) and multisystem prate inopathy (MSP). hnRNPA1 is part of the group of RNA-binding proteins (RBPs) that assemble with RNA to form RNPs. hnRNPs are concentrated in the nucleus and function in pre-mRNA splicing, mRNA stability, and the regulation of transcription and translation. During stress, hnRNPs, mRNA, and other RBPs condense in the cytoplasm to form stress granules (SGs). SGs are implicated in the pathogenesis of (neuro-)degenerative diseases, including ALS and inclusion body myopathy (IBM). Mutations in RBPs that affect SG biology, including FUS, TOP-43, hnRNPA1, hnRNPA2B1, and TIA1, underlie ALS, IBM, and other neurodegenerative diseases. Here, we characterize 4 potentially novel HNRNPA1 mutations (yielding 3 protein variants: *321Eext*6, *321Qext*6, and G304Nfs*3) and 2 known HNRNPA1 mutations (P288A and D262V), previously connected to ALS and MSP, in a broad spectrum of patients with hereditary motor neuropathy, ALS, and myopathy. We establish that the mutations can have different effects on hnRNPA1 fibrillization, liquid-liquid phase separation, and SG dynamics. P288A accelerated fibrillization and decelerated SG disassembly, whereas *321Eext*6 had no effect on fibrillization but decelerated SG disassembly. By contrast, G3D4Nfs*3 decelerated fibrillization and impaired liquid phase separation. Our findings suggest different underlying pathomechanisms for HNRNPA1 mutations with a possible link to clinical phenotypes.Functional Genomics of Muscle, Nerve and Brain Disorder

A new class of Roche lobe–filling hot subdwarf binaries

We present the discovery of the second binary with a Roche lobe–filling hot subdwarf transferring mass to a white dwarf (WD) companion. This 56 minute binary was discovered using data from the Zwicky Transient Facility. Spectroscopic observations reveal an He-sdOB star with an effective temperature of T eff = 33,700 ± 1000 K and a surface gravity of log(g) = 5.54 ± 0.11. The GTC+HiPERCAM light curve is dominated by the ellipsoidal deformation of the He-sdOB star and shows an eclipse of the He-sdOB by an accretion disk as well as a weak eclipse of the WD. We infer a He-sdOB mass of M sdOB = 0.41 ± 0.04 M ⊙ and a WD mass of M WD = 0.68 ± 0.05 M ⊙. The weak eclipses imply a WD blackbody temperature of 63,000 ± 10,000 K and a radius R WD = 0.0148 ± 0.0020 R ⊙ as expected for a WD of such high temperature. The He-sdOB star is likely undergoing hydrogen shell burning and will continue transferring mass for ≈1 Myr at a rate of 10−9 M ⊙ yr−1, which is consistent with the high WD temperature. The hot subdwarf will then turn into a WD and the system will merge in ≈30 Myr. We suggest that Galactic reddening could bias discoveries toward preferentially finding Roche lobe–filling systems during the short-lived shell-burning phase. Studies using reddening-corrected samples should reveal a large population of helium core–burning hot subdwarfs with T eff ≈ 25,000 K in binaries of 60–90 minutes with WDs. Though not yet in contact, these binaries would eventually come into contact through gravitational-wave emission and explode as a subluminous thermonuclear supernova or evolve into a massive single WD

Velocity-space sensitivity of the time-of-flight neutron spectrometer at JET

The velocity-space sensitivities of fast-ion diagnostics are often described by so-called weight functions. Recently, we formulated weight functions showing the velocity-space sensitivity of the often dominant beam-target part of neutron energy spectra. These weight functions for neutron emission spectrometry (NES) are independent of the particular NES diagnostic. Here we apply these NES weight functions to the time-of-flight spectrometer TOFOR at JET. By taking the instrumental response function of TOFOR into account, we calculate time-of-flight NES weight functions that enable us to directly determine the velocity-space sensitivity of a given part of a measured time-of-flight spectrum from TOFOR