Molecular Model of Prion Transmission to Humans

To assess interspecies barriers to transmission of transmissible spongiform encephalopathies, we investigated the ability of disease-associated prion proteins (PrPd) to initiate conversion of the human normal cellular form of prion protein of the 3 major PRNP polymorphic variants in vitro. Protein misfolding cyclic amplification showed that conformation of PrPd partly determines host susceptibility

On the Spectral Evolution of Cool, Helium-Atmosphere White Dwarfs: Detailed Spectroscopic and Photometric Analysis of DZ Stars

We present a detailed analysis of a large spectroscopic and photometric sample of DZ white dwarfs based on our latest model atmosphere calculations. We revise the atmospheric parameters of the trigonometric parallax sample of Bergeron, Leggett, & Ruiz (12 stars) and analyze 147 new DZ white dwarfs discovered in the Sloan Digital Sky Survey. The inclusion of metals and hydrogen in our model atmosphere calculations leads to different atmospheric parameters than those derived from pure helium models. Calcium abundances are found in the range from log (Ca/He) = -12 to -8. We also find that fits of the coolest objects show peculiarities, suggesting that our physical models may not correctly describe the conditions of high atmospheric pressure encountered in the coolest DZ stars. We find that the mean mass of the 11 DZ stars with trigonometric parallaxes, = 0.63 Mo, is significantly lower than that obtained from pure helium models, = 0.78 Mo, and in much better agreement with the mean mass of other types of white dwarfs. We determine hydrogen abundances for 27% of the DZ stars in our sample, while only upper limits are obtained for objects with low signal-to-noise ratio spectroscopic data. We confirm with a high level of confidence that the accretion rate of hydrogen is at least two orders of magnitude smaller than that of metals (and up to five in some cases) to be compatible with the observations. We find a correlation between the hydrogen abundance and the effective temperature, suggesting for the first time empirical evidence of a lower temperature boundary for the hydrogen screening mechanism. Finally, we speculate on the possibility that the DZA white dwarfs could be the result of the convective mixing of thin hydrogen-rich atmospheres with the underlying helium convection zone.Comment: 67 pages, 32 figures, accepted for publication in Ap

A Large, Uniform Sample of X-Ray-emitting AGNs: Selection Approach and an Initial Catalog from the ROSAT All-Sky and Sloan Digital Sky Surveys

Many open questions in X-ray astronomy are limited by the relatively small number of objects in uniform optically identified and observed samples, especially when rare subclasses are considered or when subsets are isolated to search for evolution or correlations between wavebands. We describe the initial results of a new program aimed to ultimately yield ~10⁴ fully characterized X-ray source identifications—a sample about an order of magnitude larger than earlier efforts. The technique is detailed and employs X-ray data from the ROSAT All-Sky Survey (RASS) and optical imaging and spectroscopic follow-up from the Sloan Digital Sky Survey (SDSS); these two surveys prove to be serendipitously very well matched in sensitivity. As part of the SDSS software pipelines, optical objects in the SDSS photometric catalogs are automatically positionally cross-correlated with RASS X-ray sources. Then priorities for follow-on SDSS optical spectra of candidate counterparts are automatically assigned using an algorithm based on the known ratios of f_x/f_opt for various classes of X-ray emitters at typical RASS fluxes of ~10⁻¹³ ergs cm⁻² s⁻¹. SDSS photometric parameters for optical morphology, magnitude, and colors, plus FIRST radio information, serve as proxies for object class. Initial application of this approach to RASS/SDSS data from 1400 deg² of sky provides a catalog of more than 1200 spectroscopically confirmed quasars and other AGNs that are probable RASS identifications. Most of these are new identifications, and only a few percent of the AGN counterparts are likely to be random superpositions. The magnitude and redshift ranges of the counterparts are very broad, extending over 15 < m < 21 and 0.03 < z < 3.6, respectively. Although most identifications are quasars and Seyfert 1 galaxies, a variety of other AGN subclasses are also sampled. Substantial numbers of rare AGN types are found, including more than 130 narrow-line Seyfert 1 galaxies and 45 BL Lac candidates. These early results already provide a very sizable set of source identifications, demonstrate the utility of the sample in multiwaveband investigations, and show the capability of the joint RASS/SDSS approach to efficiently proceed toward the largest homogeneously selected/observed sample of X-ray–emitting quasars and other kinds of AGNs

A Large, Uniform Sample of X-ray Emitting AGN from the ROSAT All-Sky and Sloan Digital Sky Surveys: the Data Release 5 Sample

We describe further results of a program aimed to yield ~10^4 fully characterized optical identifications of ROSAT X-ray sources. Our program employs X-ray data from the ROSAT All-Sky Survey (RASS), and both optical imaging and spectroscopic data from the Sloan Digital Sky Survey (SDSS). RASS/SDSS data from 5740 deg^2 of sky spectroscopically covered in SDSS Data Release 5 (DR5) provide an expanded catalog of 7000 confirmed quasars and other AGN that are probable RASS identifications. Again in our expanded catalog, the identifications as X-ray sources are statistically secure, with only a few percent of the SDSS AGN likely to be randomly superposed on unrelated RASS X-ray sources. Most identifications continue to be quasars and Seyfert 1s with 15<m<21 and 0.01<z<4; but the total sample size has grown to include very substantial numbers of even quite rare AGN, e.g., now including several hundreds of candidate X-ray emitting BL Lacs and narrow-line Seyfert 1 galaxies. In addition to exploring rare subpopulations, such a large total sample may be useful when considering correlations between the X-ray and the optical, and may also serve as a resource list from which to select the "best" object (e.g., X-ray brightest AGN of a certain subclass, at a preferred redshift or luminosity) for follow-on X-ray spectral or alternate detailed studies.Comment: Accepted for publication in AJ; 32 pages, including 11 figures, and 6 example table

HECTD2 Is Associated with Susceptibility to Mouse and Human Prion Disease

Prion diseases are fatal transmissible neurodegenerative disorders, which include Scrapie, Bovine Spongiform Encephalopathy (BSE), Creutzfeldt-Jakob Disease (CJD), and kuru. They are characterised by a prolonged clinically silent incubation period, variation in which is determined by many factors, including genetic background. We have used a heterogeneous stock of mice to identify Hectd2, an E3 ubiquitin ligase, as a quantitative trait gene for prion disease incubation time in mice. Further, we report an association between HECTD2 haplotypes and susceptibility to the acquired human prion diseases, vCJD and kuru. We report a genotype-associated differential expression of Hectd2 mRNA in mouse brains and human lymphocytes and a significant up-regulation of transcript in mice at the terminal stage of prion disease. Although the substrate of HECTD2 is unknown, these data highlight the importance of proteosome-directed protein degradation in neurodegeneration. This is the first demonstration of a mouse quantitative trait gene that also influences susceptibility to human prion diseases. Characterisation of such genes is key to understanding human risk and the molecular basis of incubation periods

A Large, Uniform Sample of X-Ray-emitting Active Galactic Nuclei from the ROSAT All Sky and Sloan Digital Sky Surveys: The Data Release 5 Sample

We describe further results of a program aimed at yielding ~104 fully characterized optical identifications of ROSAT X-ray sources. Our program employs X-ray data from the ROSAT All Sky Survey (RASS) and both optical imaging and spectroscopic data from the Sloan Digital Sky Survey (SDSS). RASS/SDSS data from 5740 deg² of sky spectroscopically covered in SDSS Data Release 5 provide an expanded catalog of 7000 confirmed quasars and other active galactic nuclei (AGNs) that are probable RASS identifications. Again, in our expanded catalog the identifications as X-ray sources are statistically secure, with only a few percent of the SDSS AGNs likely to be randomly superposed on unrelated RASS X-ray sources. Most identifications continue to be quasars and Seyfert 1 galaxies with 15 < m < 21 and 0.01 < z < 4, but the total sample size has grown to include very substantial numbers of even quite rare AGNs, e.g., it now includes several hundreds of candidate X-ray-emitting BL Lac objects and narrow-line Seyfert 1 galaxies. In addition to exploring rare subpopulations, such a large total sample may be useful when considering correlations between the X-ray and the optical and may also serve as a resource list from which to select the "best" object (e.g., X-ray-brightest AGN of a certain subclass at a preferred redshift or luminosity) for follow-up X-ray spectral or alternate detailed studies

The Ultraviolet-to-Mid-Infrared Spectral Energy Distribution of Weak Emission Line Quasars

We present Spitzer Space Telescope photometry of 18 Sloan Digital Sky Survey (SDSS) quasars at 2.7 <= z <= 5.9 which have weak or undetectable high-ionization emission lines in their rest-frame ultraviolet (UV) spectra (hereafter weak-lined quasars, or WLQs). The Spitzer data are combined with SDSS spectra and ground-based, near-infrared (IR) photometry of these sources to produce a large inventory of spectral energy distributions (SEDs) of WLQs across the rest-frame ~0.1-5 mum spectral band. The SEDs of our sources are inconsistent with those of BL Lacertae objects which are dominated by synchrotron emission due to a jet aligned close to our line-of-sight, but are consistent with the SED of ordinary quasars with similar luminosities and redshifts that exhibit a near-to-mid-IR 'bump', characteristic of hot dust emission. This indicates that broad emission lines in WLQs are intrinsically weak, rather than suffering continuum dilution from a jet, and that such sources cannot be selected efficiently from traditional photometric surveys.Comment: 10 pages (emulateapj), 4 figures. Accepted for publication in Ap