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Background: Cerebellar atrophy is characterized by loss of cerebellar tissue, with evidence on brain imaging of enlarged interfolial spaces compared to the foliae. Genetic ataxias associated with cerebellar atrophy are a heterogeneous group of disorders. We investigated the prevalence in Canada and the diagnostic yield of whole exome sequencing (WES) for this group of conditions. Methods: Between 2011 and 2017, WES was performed in 91 participants with cerebellar atrophy as part of one of two national research programs, Finding of Rare Genetic Disease Genes (FORGE) or Enhanced Care for Rare Genetic Diseases in Canada (Care4Rare). Results: A genetic diagnosis was established in 58% of cases (53/91). Pathogenic variants were found in 24 known genes, providing a diagnosis for 46/53 participants (87%), and in four novel genes, accounting for 7/53 cases (13%). 38/91 cases (42%) remained unsolved. The most common diagnoses were channelopathies in 12/53 patients (23%) and mitochondrial disorders in 9/53 (17%). Inheritance was autosomal recessive in the majority of cases. Additional clinical findings provided useful clues to some of the diagnoses. Conclusions: This is the first report on the prevalence of genetic ataxias associated with cerebellar atrophy in Canada, and the utility of WES for this group of conditions.
An important discriminant between leading models for the origin of the Magellanic Stream is the presence of a stellar counterpart to the HI gas stream: ram pressure stripping of gas by a putative hot Galactic halo would act only on Magellanic gas while gravitational tidal stripping would act on both gas and stars. Several previous attempts to find tidal stellar debris have failed to find carbon stars, A stars, or other main sequence stars in the Magellanic Stream (Mathewson et al. 1979; Recillas-Cruz 1982; Brück & Hawkins 1983; Guhathakurta & Lin 1999). However, there has long been a suggestion (Kunkel 1979; Lynden-Bell 1982) of a possible Magellanic association of satellite galaxies and globular clusters that have similar orbits and may derive from the break up of a greater Magellanic galaxy (Lynden-Bell & Lynden-Bell 1995; Majewski et al. 1997). Recent models (Moore & Davis 1994; Johnston 1998) of the tidal disruption of Large Magellanic Cloud (LMC)-like systems indicate a wide dispersal of debris, much wider than the rather confined HI stream, so that the contrast of tidal debris against the Galactic fore/background would be low. If true, this could explain some of the previous negative results for tidal debris searches.
NASA’s Space Interferometry Mission (SIM), scheduled for launch in 2009, will determine the positions of thousands of stars as faint as V = 20 to a precision better than 4 microarcseconds (µas). A key part of the mission is the Astrometric Grid, which is a reference frame of several thousand stars with V ≤ 13 against which all relative measurements will be calibrated. To serve as a reliable inertial reference frame, the Grid must be astrometrically stable against photocenter jitter (from planets, binary companions, flaring or spotting) at the ~ 4µas level. Sub–solar metallicity giant stars, by virtue of their intrinsic luminosity, can probe the Galaxy to greater distances than almost any other stellar type at the same apparent magnitude. Thus, distant (> 3 kpc) giants with V < 13 will have proportionately smaller astrometric jitter compared to other potential Astrometric Grid star candidates. The Grid Giant Star Survey is a patchwork all-sky survey to find sub–solar metallicity K giants for the Grid, and to provide a unique database for studies of Galactic stellar populations. We describe here the survey characteristics and give examples of results to date.
The ice-proximal diamict sediment deposited on the foreset of a grounding zone wedge in Glomar Challenger Basin on the eastern Ross Sea continental shelf yielded a low abundance assemblage of foraminifera at two piston core sites. We found 302 small well-preserved specimens representing 18 species of benthic foraminifera from 825 ml of sediment. Only three poorly preserved specimens of the planktonic foraminifera Neogloboquadrina pachyderma (sinistral) were found. Our combined analyses of preservation state, assemblage composition and stable isotopes suggest that the benthic foraminifera may be in situ. This possibility is of interest to palaeoclimatologists who use ice-proximal sediments on the Antarctic continental shelves to radiocarbon date the post-glacial retreat history.
We have obtained deep g, r, and i-band Subaru and ultra-deep 3.6 μm IRAC images of parts of the multiply-wrapped stellar stream around the nearby edge-on galaxy NGC 5907. We have fitted the surface brightness measurements of the stream with FSPS stellar population synthesis models to derive the metallicity and age of the brightest parts of the stream. The resulting relatively high metallicity ([Fe/H] = −0.3) is consistent with a major merger scenario but a satellite accretion event cannot be ruled out.
High Speed Sintering (HSS) is a novel additive manufacturing technology which currently uses Nylon 12 as the standard feedstock material. To expand the number of processable materials, the preferred characteristics of polymeric powder as a feedstock powder are presented, appropriate materials identified, parts made, and mechanical properties measured. Two commercially available laser sintering (LS) grade powders previously untested for HSS were selected, DuraForm® HST10 and ALM TPE 210-S. Tensile test specimens were manufactured using each material and mechanical properties analyzed and compared to the manufacturers' specification for LS. Tensile test specimens built using DuraForm® PA show higher tensile strength and elongation at break than LS whereas DuraForm® HST10 shows somewhat reduced tensile strength but slightly increased elongation at break. ALM TPE 210-S shows elongation at break of more than double that of LS demonstrating the capability of HSS to process viscous materials. The results indicate that HSS is capable of processing LS grade polymeric powders and may extend beyond.
We present first-principles studies of the effect of biaxial (0001)-strain on the electronic structure of wurtzite GaN, AlN, and InN. We provide accurate predictions for the valence band splittings as a function of strain which greatly facilitates the interpretation of data from samples with unintentional growth-induced strain. The present calculations are based on the total-energy pseudopotential method within the local-density formalism and include the spin-orbit interaction nonperturbatively. For a given biaxial strain, all structural parameters are determined by minimization of the total energy with respect to the electronic and ionic degrees of freedom. Our calculations predict that the valence band state Γ9(Γ6) lies energetically above the Γ7(Γ1) states in GaN and InN, in contrast to the situation in AlN. In all three nitrides, we find that the ordering of these two levels becomes reversed for some value of biaxial strain. In GaN, this crossing takes place already at 0.32% tensile strain. For larger tensile strains, the top of the valence band becomes well separated from the lower states. The computed crystal-field and spin-orbit splittings in unstrained materials as well as the computed deformation potentials agree well with the available experimental data.
We have performed systematic first-principles pseudopotential local density functional calculations of stacking faults in GaN and AlN. Their band offsets and the charge accumulation at stacking fault interfaces has been investigated, taking fully into account the effects of lattice relaxation and electric polarization. We find the stacking fault junctions to be of type I in both materials. However, the intrinsic valence band offsets are close to zero, so that the conduction band offsets result mostly from the differences in the energy gaps between the cubic and wurtzite phases. The charge accumulated at the interface between the cubic and wurtzite phase is found to be 0.009 and 0.003 C/m2 for the AlN and GaN stacking fault, respectively.
Increasing concentrations of selenium oxoanions in the environment are placing many animals at risk for reproduction failure and deformities. The understanding of binding mechanisms of selenium oxoanions to iron and manganese based oxide minerals could lead to enhanced understanding of selenium mobility in the environment. In this study, the binding mechanisms of selenium oxoanions, selenite and selenate, to non microwave-assisted and microwave-assisted synthetic Fe3O4, Mn3O4, and MnFe2O4 nanomaterials were investigated through the use of X-ray absorption spectroscopy. The X-ray absorption near-edge structure (XANES) spectroscopy studies revealed the oxidation state of selenite and selenate remains the same after binding occurs to all nanomaterials in pH 2, 4, or 6 environments. The binding modes of selenite and selenate were determined to be bidentate binuclear through use of Extended x-ray absorption fine structure (EXAFS) and were independent of nanomaterials, synthetic technique, and pH.
Disruption of cell membranes triggers rapid metabolic energy exhaustion, then acute cellular necrosis. Cell membrane dysfunction due to loss of structure integrity is the pathology of tissue death in trauma, muscular dystrophies, reperfusion injuries and common diseases. It is now established that certain PEG-based biocompatible polymers, such as Poloxamer 188, Poloxamine 1107 and PEG, are effective in sealing of injured cell membranes, and thus can prevent acute necrosis if delivered within a few hours after injury. Despite these broad applications of PEG-based polymers for human health, the fundamental mechanisms of how PEG-based polymers interact with cell membranes are still under debate. Here, the effects of PEG-based biocompatible polymers on phospholipid membrane integrity under external stimuli (osmotic stress and oxidative stress) were explored using giant unilamellar vesicles (GUVs) as model cell membranes. Through fluorescence leakage assays and time-lapse fluorescence microscopy, we directly observed that the surface-adsorbed P188 can efficiently inhibits the loss of structural integrity of giant unilamellar vesicles (GUVs) under hypo-osmotic stress. We propose that the adsorption of polymers on the membrane surface is responsible for the cell membrane resealing process, while the insertion of the hydrophobic portion of the polymers increases membrane permeability. To elucidate the mechanism by which hydrophilic polymers help restore membrane integrity while their hydrophobic counterparts disrupt it, 1H Overhauser Dynamic Nuclear Polarization (ODNP)-NMR spectroscopy, a newly developed NMR technique that provides unprecedented resolution for differentiating weak surface adsorption versus translocation of polymers to membranes, was employed to sensitively detect polymer-lipid membrane interactions through the modulation of local hydration dynamics in lipid membranes. Our study shows that P188—the most hydrophilic poloxamer known as a membrane sealant—weakly adsorbs onto the membrane surface, yet effectively retards membrane hydration dynamics. Contrarily, P181—the most hydrophobic poloxamer known as a membrane permeabilizer—initially penetrates past lipid headgroups and enhances intrabilayer water diffusivity. Consequently, our results illustrate that the relative hydrophilic/hydrophobic ratio of the polymer dictates its functions. These findings gleaned from local hydration dynamics are well supported by our thermodynamics and fluorescence data.
Opuntia ficus-indica (Ofi) cactus non-gelling (NE) mucilage nanofibers were electrospun with acetic acid solution and polyvinyl alcohol (PVA) as a polymer. The best fiber coverage was achieved with an aqueous 50% acetic acid solution and 9% low molecular weight PVA at a 70:30 PVA:Mucilage volume ratio. Other volume ratios (30:70 and 50:50) produced beads and other deformities. Fibers were formed with an average diameter of 180nm as measured by scanning electron microscopy (SEM) and atomic force microscopy (AFM). Favorable electrospinning conditions were used to fabricate a 1 cm x 1 cm Ofi nanofiber biomembrane. Heat flow (W/g) versus temperature peaks ranged from 214 – 222°C, which is comparable to endothermic peak ranges observed for crystalline PVA. This could possibly further indicate some form of crystallinity within the Ofi nanofiber membrane. The electrospun process used precursors that were biodegradable, non-toxic, and sustainable to optimize the mucilage nanofiber formation, which will help enhance the potential performance of the Ofi nanofiber biomembrane in filtration and sensory systems.
In this paper the spore-crystal complex of Bacillus thuringiensis var. israelensis (Bti) was immobilized by the sol-gel process in a hybrid polymer using as precursors the inorganic tetraethyl orthosilicate (TEOS) and the organic Polydimethylsiloxane (PDMS); in order to combine the advantages of both materials in a hybrid matrix to improve aspects such as the thermal stability, the hydrophobic properties and the porosity. Bti produces different crystals during sporulation phase; these are of protein nature and are used as bio-insecticides. It is important to mention that the insecticide attack is specific to the mosquito larva that causes dengue and black flies. The samples were characterized to ensure viability by performing growth kinetics with fermentations immersed in a flask, this microbial growth was monitored by dry weight, glucose consumption and characterized by Fourier Transform Infrared Spectroscopy (FTIR) to observe the interaction of materials with spore-crystal complex.
We report, for the first time, the application of the photoacoustic spectroscopy for monitoring the optical absorption spectra in aquatic lirium (Eichhornia Crassipes), before and after it was exposed to ultrasonic irradiations. We obtained a decrease in the amplitude of the bands of the chlorophylls a and b for the irradiated samples with ultrasound of 17 kHz and 1.5 mW/cm2 of power density, and therefore, damage in the centers producing the photosynthesis, due to the irradiation. These results show the utility of the ultrasonic irradiation, as well as, of the photosynthesis monitoring by means of the photoacoustic technique, for the elaboration and establishment of methodologies in the control of this aquatic plant, whose propagation causes many consequences extremely unfavorable for the environment, as well as for the diverse human activities that are developed in the bodies of water in the tropical and sub-tropical regions of the world.
We present the first systematic comparison of the detailed properties, including internal kinematics, chemical abundances, sizes, and dark matter masses, of Milky Way and M 31 dSphs as a part of the SPLASH Survey (Spectroscopic and Photometric Landscape of Andromeda’s Stellar Halo). Through Keck/DEIMOS spectroscopy of several hundred individual red giants in a half dozen M 31 galaxies, our results indicate both similarities and differences between the family of dSphs in the Milky Way and M 31. For example, we find that the luminosity-metallicity relation of dSphs in the two hosts is very similar between L = 105 and 107L⊙, the size distribution of M 31 dSphs extends to larger values at the same luminosity compared to Milky Way counterparts (especially at the bright end), and that the dark matter masses of M 31 dSphs are slightly smaller than similar luminosity Milky Way galaxies.
The generation of high density 2D hole gases is crucial for further progress in the field of electronic and optoelectronic nitride devices. In this paper, we present results of C-V profiling measurements for N-face GaN/AlGaN heterostructures and systematic theoretical studies of Mg- doped GaN/AlGaN gated heterostructures and superlattices. Our calculations are based on a self- consistent solution of the multiband k.p Schrödinger and Poisson equation and reveal that the hole 2D sheet density is mainly determined by the polarization induced interface charges. For an Aluminium concentration of 30%, the induced hole density in the heterostructure can reach values up to 1.5×1013 cm−2. In the GaN/AlGaN superlattices, the hole sheet density increases with the superlattice period and saturates for a period of 40 nm at a value of 1.5×1013 cm−2.
A low dose rate evaluation of several scintillators and wavelength shifters of interest to the CEBAF program was performed with a 60Co line source to a total dose of 160 Gray at a rate of 0.5 Gy/hr in a slowly flowing oxygen atmosphere. The samples included the following: (1) a 2 meter piece of Bicron BC412 scintillator being extensively used in the CEBAF Large Acceptance Spectrometer, (2) a 1 meter long piece of green scintillator doped with a uniquely fast green fluor (G2), (3) wavelength shifting fiber dopedalso with G2, and (4) grooved acrylic-based scintillator that is readout with the WLS fiber. The data indicates that the BC412 shows no loss in intrinsic light output, but does have an attenuation loss amounting to 10% at 1 meter and 16% at 2 meters. The fast green scintillator (and fibers) showed no damage. When read out by WLS fibers, the acrylic scintillator displayed a loss in intrinsic light output, but no change in attenuation.
We studied the equilibrium architecture of polymer layers strongly adsorbed from the melt. Immobilized layers of poly-(methyl methacrylate) (PMMA) were produced by the following method: 1) The polymer was spin-coated onto silanol bearing surfaces of single crystal and fused quartz, and annealed at melt conditions, 2) The annealed layer was quenched to room temperature (below the glass transition temperature) in order to “freeze in” the melt structure near the substrate, 3) Unbound material was leached away in good solvent (benzene) to leave a residual, strongly-adsorbed layer. The architecture of this layer was studied by neutron reflection. Data on dried adsorbed layers indicates a dense PMMA film whose thickness gradually increases with annealing time in the melt from a minimal value. Evidently, annealing gradually relaxes a rather flat non-equilibrium structure produced by spin-coating. The thicknesses, h, in a series of dry layers annealed long enough to achieve equilibrium conditions in the melt scale as h ∼ N1/2. Data on swollen layers suggest a dilute, extended layer, but the preliminary results cannot give a definitive confirmation of the brush structure predicted by Guiselin.11