A C-Terminal Protease-Resistant Prion Fragment Distinguishes Ovine “CH1641-Like” Scrapie from Bovine Classical and L-Type BSE in Ovine Transgenic Mice

The protease-resistant prion protein (PrPres) of a few natural scrapie isolates identified in sheep, reminiscent of the experimental isolate CH1641 derived from a British natural scrapie case, showed partial molecular similarities to ovine bovine spongiform encephalopathy (BSE). Recent discovery of an atypical form of BSE in cattle, L-type BSE or BASE, suggests that also this form of BSE might have been transmitted to sheep. We studied by Western blot the molecular features of PrPres in four “CH1641-like” natural scrapie isolates after transmission in an ovine transgenic model (TgOvPrP4), to see if “CH1641-like” isolates might be linked to L-type BSE. We found less diglycosylated PrPres than in classical BSE, but similar glycoform proportions and apparent molecular masses of the usual PrPres form (PrPres #1) to L-type BSE. However, the “CH1641-like” isolates differed from both L-type and classical BSE by an abundant, C-terminally cleaved PrPres product (PrPres #2) specifically recognised by a C-terminal antibody (SAF84). Differential immunoprecipitation of PrPres #1 and PrPres #2 resulted in enrichment in PrPres #2, and demonstrated the presence of mono- and diglycosylated PrPres products. PrPres #2 could not be obtained from several experimental scrapie sources (SSBP1, 79A, Chandler, C506M3) in TgOvPrP4 mice, but was identified in the 87V scrapie strain and, in lower and variable proportions, in 5 of 5 natural scrapie isolates with different molecular features to CH1641. PrPres #2 identification provides an additional method for the molecular discrimination of prion strains, and demonstrates differences between “CH1641-like” ovine scrapie and bovine L-type BSE transmitted in an ovine transgenic mouse model

A behavioral comparison of male and female adults with high functioning autism spectrum conditions

Autism spectrum conditions (ASC) affect more males than females in the general population. However, within ASC it is unclear if there are phenotypic sex differences. Testing for similarities and differences between the sexes is important not only for clinical assessment but also has implications for theories of typical sex differences and of autism. Using cognitive and behavioral measures, we investigated similarities and differences between the sexes in age- and IQ-matched adults with ASC (high-functioning autism or Asperger syndrome). Of the 83 (45 males and 38 females) participants, 62 (33 males and 29 females) met Autism Diagnostic Interview-Revised (ADI-R) cut-off criteria for autism in childhood and were included in all subsequent analyses. The severity of childhood core autism symptoms did not differ between the sexes. Males and females also did not differ in self-reported empathy, systemizing, anxiety, depression, and obsessive-compulsive traits/symptoms or mentalizing performance. However, adult females with ASC showed more lifetime sensory symptoms (p = 0.036), fewer current socio-communication difficulties (p = 0.001), and more self-reported autistic traits (p = 0.012) than males. In addition, females with ASC who also had developmental language delay had lower current performance IQ than those without developmental language delay (p<0.001), a pattern not seen in males. The absence of typical sex differences in empathizing-systemizing profiles within the autism spectrum confirms a prediction from the extreme male brain theory. Behavioral sex differences within ASC may also reflect different developmental mechanisms between males and females with ASC. We discuss the importance of the superficially better socio-communication ability in adult females with ASC in terms of why females with ASC may more often go under-recognized, and receive their diagnosis later, than males

Emergence of Classical BSE Strain Properties during Serial Passages of H-BSE in Wild-Type Mice

BACKGROUND: Two distinct forms of atypical spongiform encephalopathies (H-BSE and L-BSE) have recently been identified in cattle. Transmission studies in several wild-type or transgenic mouse models showed that these forms were associated with two distinct major strains of infectious agents, which also differed from the unique strain that had been isolated from cases of classical BSE during the food-borne epizootic disease. METHODOLOGY/PRINCIPAL FINDINGS: H-BSE was monitored during three serial passages in C57BL/6 mice. On second passage, most of the inoculated mice showed molecular features of the abnormal prion protein (PrP(d)) and brain lesions similar to those observed at first passage, but clearly distinct from those of classical BSE in this mouse model. These features were similarly maintained during a third passage. However, on second passage, some of the mice exhibited distinctly different molecular and lesion characteristics, reminiscent of classical BSE in C57Bl/6 mice. These similarities were confirmed on third passage from such mice, for which the same survival time was also observed as with classical BSE adapted to C57Bl/6 mice. Lymphotropism was rarely detected in mice with H-BSE features. In contrast, PrP(d) was detectable, on third passage, in the spleens of most mice exhibiting classical BSE features, the pattern being indistinguishable from that found in C57Bl/6 mice infected with classical BSE. CONCLUSION/SIGNIFICANCE: Our data demonstrate the emergence of a prion strain with features similar to classical BSE during serial passages of H-BSE in wild-type mice. Such findings might help to explain the origin of the classical BSE epizootic disease, which could have originated from a putatively sporadic form of BSE

Study of Envelope Velocity Evolution of Type Ib-c Core-Collapse Supernovae from Observations of XRF 080109 / SN 2008D and GRB 060218 / SN 2006aj with BTA

Results of modeling the spectra of two supernovae SN 2008D and SN 2006aj related to the X-ray flash XRF 080109 and gamma-ray burst GRB / XRF 060218, respectively, are studied. The spectra were obtained with the 6-meter BTA telescope of the Special Astrophysical Observatory of the Russian Academy of Sciences in 6.48 and 27.61 days after the explosion of SN 2008D, and in 2.55 and 3.55 days after the explosion of SN 2006aj. The spectra were interpreted in the Sobolev approximation with the SYNOW code. An assumption about the presence of envelopes around the progenitor stars is confirmed by an agreement between the velocities of lines interpreted as hydrogen and helium, and the empiric power-law velocity drop with time for the envelopes of classic core-collapse supernovae. Detection of a P Cyg profile of the H-beta line in the spectra of optical afterglows of GRBs can be a determinative argument in favor of this hypothesis.Comment: 12 pages, 6 figures, accepted for publication in Astrophysical Bulletin

Radiation hydrodynamics of SN 1987A: I. Global analysis of the light curve for the first 4 months

The optical/UV light curves of SN 1987A are analyzed with the multi-energy group radiation hydrodynamics code STELLA. The calculated monochromatic and bolometric light curves are compared with observations shortly after shock breakout, during the early plateau, through the broad second maximum, and during the earliest phase of the radioactive tail. We have concentrated on a progenitor model calculated by Nomoto & Hashimoto and Saio, Nomoto, & Kato, which assumes that 14 solar masses of the stellar mass is ejected. Using this model, we have updated constraints on the explosion energy and the extent of mixing in the ejecta. In particular, we determine the most likely range of E/M (explosion energy over ejecta mass) and R_0 (radius of the progenitor). In general, our best models have energies in the range E = (1.1 +/- 0.3) x 10^{51} ergs, and the agreement is better than in earlier, flux-limited diffusion calculations for the same explosion energy. Our modeled B and V fluxes compare well with observations, while the flux in U undershoots after about 10 days by a factor of a few, presumably due to NLTE and line transfer effects. We also compare our results with IUE observations, and a very good quantitative agreement is found for the first days, and for one IUE band (2500-3000 A) as long as for 3 months. We point out that the V flux estimated by McNaught & Zoltowski should probably be revised to a lower value.Comment: 27 pages AASTeX v.4.0 + 35 postscript figures. ApJ, accepte

Interferometric radii of bright Kepler stars with the CHARA Array: {\theta} Cygni and 16 Cygni A and B

We present the results of long-baseline optical interferometry observations using the Precision Astronomical Visual Observations (PAVO) beam combiner at the Center for High Angular Resolution Astronomy (CHARA) Array to measure the angular sizes of three bright Kepler stars: {\theta} Cygni, and both components of the binary system 16 Cygni. Supporting infrared observations were made with the Michigan Infrared Combiner (MIRC) and Classic beam combiner, also at the CHARA Array. We find limb-darkened angular diameters of 0.753+/-0.009 mas for {\theta} Cyg, 0.539+/-0.007 mas for 16 Cyg A and 0.490+/-0.006 mas for 16 Cyg B. The Kepler Mission has observed these stars with outstanding photometric precision, revealing the presence of solar-like oscillations. Due to the brightness of these stars the oscillations have exceptional signal-to-noise, allowing for detailed study through asteroseismology, and are well constrained by other observations. We have combined our interferometric diameters with Hipparcos parallaxes, spectrophotometric bolometric fluxes and the asteroseismic large frequency separation to measure linear radii ({\theta} Cyg: 1.48+/-0.02 Rsun, 16 Cyg A: 1.22+/-0.02 Rsun, 16 Cyg B: 1.12+/-0.02 Rsun), effective temperatures ({\theta} Cyg: 6749+/-44 K, 16 Cyg A: 5839+/-42 K, 16 Cyg B: 5809+/-39 K), and masses ({\theta} Cyg: 1.37+/-0.04 Msun, 16 Cyg A: 1.07+/-0.05 Msun, 16 Cyg B: 1.05+/-0.04 Msun) for each star with very little model dependence. The measurements presented here will provide strong constraints for future stellar modelling efforts.Comment: 9 pages, 4 figures, and 5 tables, accepted for publication in Monthly Notices of the Royal Astronomical Societ