Amyotrophic lateral sclerosis (ALS) is a fatal degenerative motor neuron disorder. Ten percent of cases are inherited; most involve unidentified genes. We report here 13 mutations in the fused in ...sarcoma/translated in liposarcoma (FUS/TLS) gene on chromosome 16 that were specific for familial ALS. The FUS/TLS protein binds to RNA, functions in diverse processes, and is normally located predominantly in the nucleus. In contrast, the mutant forms of FUS/TLS accumulated in the cytoplasm of neurons, a pathology that is similar to that of the gene TAR DNA-binding protein 43 (TDP43), whose mutations also cause ALS. Neuronal cytoplasmic protein aggregation and defective RNA metabolism thus appear to be common pathogenic mechanisms involved in ALS and possibly in other neurodegenerative disorders.
Passive transfer of immunity is essential for the short- and long-term health of dairy calves. The objective of this study was to evaluate factors associated with colostrum quality and passive ...transfer status of US heifer calves. This study included 104 operations in 13 states that participated in the calf component of the National Animal Health Monitoring System's Dairy 2014 study. This 18-mo longitudinal study included 1,972 Holstein heifer calves from birth to weaning. Multivariable mixed linear regression models were selected using backward elimination model selection after univariate screening to determine which factors were associated with colostrum IgG and serum IgG concentrations. The mean colostrum IgG concentration was 74.4 g/L with 77.4% of colostrum samples having IgG concentrations >50 g/L. The final model for colostrum IgG included colostrum source and a categorized temperature-humidity index value (cTHI) for the month before calving. Mean colostrum IgG concentrations were highest for dams in third and higher lactations (84.7 g/L) and lowest for commercial colostrum replacers (40.3 g/L). Colostrum IgG concentrations were highest for cTHI ≥70 (72.6 g/L) and lowest for cTHI <40 (64.2 g/L). The mean serum IgG concentration was 21.6 g/L, with 73.3% of calves having serum IgG concentrations >15 g/L. The final model for serum IgG concentration included region, heat treatment of colostrum, colostrum source, timing to first feeding, volume of colostrum fed in the first 24 h, age of the calf at blood sampling, and colostrum IgG concentration. Mean serum IgG concentrations were highest for calves that received colostrum from first-lactation dams (25.7 g/L) and lowest for calves fed commercial colostrum replacer (16.6 g/L). Serum IgG concentrations were higher for calves fed heat-treated colostrum (24.4 g/L) than for calves fed untreated colostrum (20.5 g/L). Serum IgG concentration was positively associated with the volume of colostrum fed in the first 24 h and colostrum IgG concentration, and negatively associated with the number of hours from birth to colostrum feeding and age (days) at blood collection. Dairy producers should be encouraged to measure the quality of colostrum before administering it to calves and to measure serum IgG or a proxy such as serum total protein or Brix to evaluate passive immunity and colostrum management programs.
The injection and mixing of contaminant mass into the fuel in inertial confinement fusion (ICF) implosions is a primary factor preventing ignition. ICF experiments have recently achieved an ...alpha-heating regime, in which fusion self-heating is the dominant source of yield, by reducing the susceptibility of implosions to instabilities that inject this mass. We report the results of unique separated reactants implosion experiments studying pre-mixed contaminant as well as detailed high-resolution three-dimensional simulations that are in good agreement with experiments. At conditions relevant to mixing regions in high-yield implosions, we observe persistent chunks of contaminant that do not achieve thermal equilibrium with the fuel throughout the burn phase. The assumption of thermal equilibrium is made in nearly all computational ICF modeling and methods used to infer levels of contaminant from experiments. We estimate that these methods may underestimate the amount of contaminant by a factor of two or more.
We present narrow-band self-emission x-ray images from a titanium tracer layer placed at the fuel-shell interface in 60-laser-beam implosion experiments at the OMEGA facility. The images are acquired ...during deceleration with inferred convergences of ∼9-14. Novel here is that a systematically observed asymmetry of the emission is linked, using full sphere 3D implosion modeling, to performance-limiting low mode asymmetry of the drive.
Global Consensus Statement on Menopausal Hormone Therapy de Villiers, T. J.; Gass, M. L. S.; Haines, C. J. ...
Climacteric : the journal of the International Menopause Society,
04/2013, Letnik:
16, Številka:
2
Journal Article, Conference Proceeding
Recenzirano
Odprti dostop
The following Consensus Statement is endorsed by The American Society for Reproductive Medicine, The Asia Pacific Menopause Federation, The Endocrine Society, The European Menopause and Andropause ...Society, The International Menopause Society, The International Osteoporosis Foundation and The North American Menopause Society.
We study the luminosity gap, Δm12, between the first- and second-ranked galaxies in a sample of 59 massive (∼1015 M⊙) galaxy clusters, using data from the Hale Telescope, the Hubble Space Telescope, ...Chandra and Spitzer. We find that the Δm12 distribution, p(Δm12), is a declining function of Δm12 to which we fitted a straight line: p(Δm12) ∝−(0.13 ± 0.02)Δm12. The fraction of clusters with ‘large’ luminosity gaps is p(Δm12≥ 1) = 0.37 ± 0.08, which represents a 3σ excess over that obtained from Monte Carlo simulations of a Schechter function that matches the mean cluster galaxy luminosity function. We also identify four clusters with ‘extreme’ luminosity gaps, Δm12≥ 2, giving a fraction of . More generally, large luminosity gap clusters are relatively homogeneous, with elliptical/discy brightest cluster galaxies (BCGs), cuspy gas density profiles (i.e. strong cool cores), high concentrations and low substructure fractions. In contrast, small luminosity gap clusters are heterogeneous, spanning the full range of boxy/elliptical/discy BCG morphologies, the full range of cool core strengths and dark matter concentrations, and have large substructure fractions. Taken together, these results imply that the amplitude of the luminosity gap is a function of both the formation epoch and the recent infall history of the cluster. ‘BCG dominance’ is therefore a phase that a cluster may evolve through and is not an evolutionary ‘cul-de-sac’. We also compare our results with semi-analytic model predictions based on the Millennium Simulation. None of the models is able to reproduce all of the observational results on Δm12, underlining the inability of the current generation of models to match the empirical properties of BCGs. We identify the strength of active galactic nucleus feedback and the efficiency with which cluster galaxies are replenished after they merge with the BCG in each model as possible causes of these discrepancies.
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