21 research outputs found
Electronic structure of NiSSe across the phase transition
We report very highly resolved photoemission spectra of NiS(1-x)Se(x) across
the so-called metal-insulator transition as a function of temperature as well
as composition. The present results convincingly demonstrate that the low
temperature, antiferromagnetic phase is metallic, with a reduced density of
states at E. This decrease is possibly due to the opening of gaps along
specific directions in the Brillouin zone caused by the antiferromagnetic
ordering.Comment: Revtex, 4 pages, 3 postscript figure
Electronic structure of NiS_{1-x}Se_x
We investigate the electronic structure of the metallic NiSSe
system using various electron spectroscopic techniques. The band structure
results do not describe the details of the spectral features in the
experimental spectrum, even for this paramagnetic metallic phase. However, a
parameterized many-body multi-band model is found to be successful in
describing the Ni~2 core level and valence band, within the same model. The
asymmetric line shape as well as the weak intensity feature in the Ni~2 core
level spectrum has been ascribed to extrinsic loss processes in the system. The
presence of satellite features in the valence band spectrum shows the existence
of the lower Hubbard band, deep inside the metallic regime, consistent
with the predictions of the dynamical mean field theory.Comment: To be published in Physical Review B, 18 pages and 5 figure
Primordial Nucleosynthesis for the New Cosmology: Determining Uncertainties and Examining Concordance
Big bang nucleosynthesis (BBN) and the cosmic microwave background (CMB) have
a long history together in the standard cosmology. The general concordance
between the predicted and observed light element abundances provides a direct
probe of the universal baryon density. Recent CMB anisotropy measurements,
particularly the observations performed by the WMAP satellite, examine this
concordance by independently measuring the cosmic baryon density. Key to this
test of concordance is a quantitative understanding of the uncertainties in the
BBN light element abundance predictions. These uncertainties are dominated by
systematic errors in nuclear cross sections. We critically analyze the cross
section data, producing representations that describe this data and its
uncertainties, taking into account the correlations among data, and explicitly
treating the systematic errors between data sets. Using these updated nuclear
inputs, we compute the new BBN abundance predictions, and quantitatively
examine their concordance with observations. Depending on what deuterium
observations are adopted, one gets the following constraints on the baryon
density: OmegaBh^2=0.0229\pm0.0013 or OmegaBh^2 = 0.0216^{+0.0020}_{-0.0021} at
68% confidence, fixing N_{\nu,eff}=3.0. Concerns over systematics in helium and
lithium observations limit the confidence constraints based on this data
provide. With new nuclear cross section data, light element abundance
observations and the ever increasing resolution of the CMB anisotropy, tighter
constraints can be placed on nuclear and particle astrophysics. ABRIDGEDComment: 54 pages, 20 figures, 5 tables v2: reflects PRD version minor changes
to text and reference
Mutations in NGLY1 cause an inherited disorder of the endoplasmic reticulum–associated degradation pathway
PurposeThe endoplasmic reticulum-associated degradation pathway is responsible for the translocation of misfolded proteins across the endoplasmic reticulum membrane into the cytosol for subsequent degradation by the proteasome. To define the phenotype associated with a novel inherited disorder of cytosolic endoplasmic reticulum-associated degradation pathway dysfunction, we studied a series of eight patients with deficiency of N-glycanase 1.MethodsWhole-genome, whole-exome, or standard Sanger sequencing techniques were employed. Retrospective chart reviews were performed in order to obtain clinical data.ResultsAll patients had global developmental delay, a movement disorder, and hypotonia. Other common findings included hypolacrima or alacrima (7/8), elevated liver transaminases (6/7), microcephaly (6/8), diminished reflexes (6/8), hepatocyte cytoplasmic storage material or vacuolization (5/6), and seizures (4/8). The nonsense mutation c.1201A>T (p.R401X) was the most common deleterious allele.ConclusionNGLY1 deficiency is a novel autosomal recessive disorder of the endoplasmic reticulum-associated degradation pathway associated with neurological dysfunction, abnormal tear production, and liver disease. The majority of patients detected to date carry a specific nonsense mutation that appears to be associated with severe disease. The phenotypic spectrum is likely to enlarge as cases with a broader range of mutations are detected