Abstract
The Earth is bombarded by ultrarelativistic particles, known as cosmic rays (CRs). CRs with energies up to a few PeV (=10
15
eV), the knee in the particle spectrum, are believed to have a ...Galactic origin. One or more factories of PeV CRs, or PeVatrons, must thus be active within our Galaxy. The direct detection of PeV protons from their sources is not possible since they are deflected in the Galactic magnetic fields. Hundred TeV
γ
-rays from decaying
π
0
, produced when PeV CRs collide with the ambient gas, can provide the decisive evidence of proton acceleration up to the knee. Here we report the discovery by the High Altitude Water Cerenkov (HAWC) observatory of the
γ
-ray source, HAWC J1825-134, whose energy spectrum extends well beyond 200 TeV without a break or cutoff. The source is found to be coincident with a giant molecular cloud. The ambient gas density is as high as 700 protons cm
−3
. While the nature of this extreme accelerator remains unclear, CRs accelerated to energies of several PeV colliding with the ambient gas likely produce the observed radiation.
The PHENIX Forward Silicon Vertex Detector Aidala, C.; Anaya, L.; Anderssen, E. ...
Nuclear instruments & methods in physics research. Section A, Accelerators, spectrometers, detectors and associated equipment,
08/2014, Volume:
755
Journal Article
Peer reviewed
Open access
A new silicon detector has been developed to provide the PHENIX experiment with precise charged particle tracking at forward and backward rapidity. The Forward Silicon Vertex Tracker (FVTX) was ...installed in PHENIX prior to the 2012 run period of the Relativistic Heavy Ion Collider (RHIC). The FVTX is composed of two annular endcaps, each with four stations of silicon mini-strip sensors, covering a rapidity range of 1.2<|η|<2.2 that closely matches the two existing PHENIX muon arms. Each station consists of 48 individual silicon sensors, each of which contains two columns of mini-strips with 75μm pitch in the radial direction and lengths in the ϕ direction varying from 3.4mm at the inner radius to 11.5mm at the outer radius. The FVTX has approximately 0.54million strips in each endcap. These are read out with FPHX chips, developed in collaboration with Fermilab, which are wire bonded directly to the mini-strips. The maximum strip occupancy reached in central Au–Au collisions is approximately 2.8%. The precision tracking provided by this device makes the identification of muons from secondary vertices away from the primary event vertex possible. The expected distance of closest approach (DCA) resolution of 200μm or better for particles with a transverse momentum of 5 GeV/c will allow identification of muons from relatively long-lived particles, such as D and B mesons, through their broader DCA distributions.
Abstract
Galactic gamma-ray diffuse emission (GDE) is emitted by cosmic rays (CRs), ultra-relativistic protons, and electrons, interacting with gas and electromagnetic radiation fields in the ...interstellar medium. Here we present the analysis of teraelectronvolt diffuse emission from a region of the Galactic plane over the range in longitude of
l
∈ 43°, 73°, using data collected with the High Altitude Water Cherenkov (HAWC) detector. Spectral, longitudinal, and latitudinal distributions of the teraelectronvolt diffuse emission are shown. The radiation spectrum is compatible with the spectrum of the emission arising from a CR population with an
index
similar to that of the observed CRs. When comparing with the
DRAGON
base model
, the HAWC GDE flux is higher by about a factor of 2. Unresolved sources such as pulsar wind nebulae and teraelectronvolt halos could explain the excess emission. Finally, deviations of the Galactic CR flux from the locally measured CR flux may additionally explain the difference between the predicted and measured diffuse fluxes.
Summary
Background
Autosomal recessive congenital ichthyosis (ARCI) is a genetically heterogeneous group of rare Mendelian skin disorders characterized by cornification and differentiation defects of ...keratinocytes. Mutations in nine genes including PNPLA1 are known to cause nonsyndromic forms of ARCI. To date, only 10 distinct pathogenic mutations in PNPLA1 have been reported.
Objectives
To identify new causative PNPLA1 mutations.
Methods
We screened genetically unresolved cases, including our ARCI collection, comprising more than 700 families. Screening for mutations was performed either by direct Sanger sequencing or in combination with a multigene panel, followed by sequence and mutation analysis.
Results
Here we report on 16 novel mutations present in patients from 17 families. While all previously reported mutations and most of our novel mutations are located within the core patatin domain, we report five novel PNPLA1 mutations that are downstream of this domain. Thus, as recently described for PNPLA2, we hypothesize that a region larger than the core domain is required for full enzymatic activity of PNPLA1 in human skin barrier formation.
Conclusions
We estimate the frequency of PNPLA1 mutations among patients with ARCI to be around 3%. Most of our patients were born as collodion babies and showed a relatively mild ichthyosis phenotype. In four unrelated patients we observed a cyclic scaling course, which seems to be a potential phenotypic variation in a small percentage of patients with PNPLA1 mutations. The variability of the clinical manifestations and the lack of typical clinical features are specific for patients with PNPLA1 mutations, and emphasize the importance of DNA sequencing for differential diagnosis of ARCIs.
What's already known about this topic?
Only five reports with 10 distinct PNPLA1 mutations causing autosomal recessive congenital ichthyosis (ARCI) have been described.
Relatively little is known about the type and localization of mutations in PNPLA1 that cause ARCI.
What does this study add?
This is the first comprehensive series of PNPLA1 mutations, from 18 patients with autosomal recessive congenital ichthyosis.
The results of this study provide important conclusions about the localization of disease‐causing mutations in PNPLA1 and the resulting phenotype, including clinical variations.
What is the translational message?
Multigene panels and knowledge about causative PNPLA1 mutations will lead to progress in deciphering the function of PNPLA1.
This might facilitate diagnosis and provide a basis for novel therapeutic strategies in patients with PNPLA1 mutations.
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Linked Comment: Uitto et al. Br J Dermatol 2017; 177:342–343
Plain language summary available online
We report the ground-level detection of a Galactic cosmic-ray (GCR) flux enhancement lasting ∼17 hr and associated with the passage of a magnetic flux rope (MFR) over the Earth. The MFR was ...associated with a slow coronal mass ejection (CME) caused by the eruption of a filament on 2016 October 9. Due to the quiet conditions during the eruption and the lack of interactions during the interplanetary CME transport to the Earth, the associated MFR preserved its configuration and reached the Earth with a strong magnetic field, low density, and a very low turbulence level compared to local background, thus generating the ideal conditions to redirect and guide GCRs (in the ∼8-60 GV rigidity range) along the magnetic field of the MFR. An important negative BZ component inside the MFR caused large disturbances in the geomagnetic field and a relatively strong geomagnetic storm. However, these disturbances are not the main factors behind the GCR enhancement. Instead, we found that the major factor was the alignment between the MFR axis and the asymptotic direction of the observer.
The High Altitude Water Cherenkov (HAWC) gamma-ray observatory is located close to the equator (latitude
18
∘
N), at an altitude of 4100 m above sea level. HAWC has 295 water Cherenkov detectors ...(WCD), each containing four photomultiplier tubes (PMT). The main purpose of HAWC is the determination of the energy and arrival direction of very high energy gamma rays produced by energetic processes in the universe, HAWC also has a scaler system which counts the arrival of secondary particles to the detector. In this work we show that the scaler system of HAWC is an ideal instrument for solar modulation and space-weather studies due to its large area and high sensitivity. In order to prepare the scaler system for low energy heliospheric studies, we model and correct the efficiency variation of each PMT of the array, which result in a capability to measure variations
>
0.01
%
with high accuracy. Using the singular value decomposition method, we correct the rate deviations of all PMTs of the array, due to changes in efficiency, gain and operational voltage. We isolate and remove the atmospheric modulations of the PMTs count rates measured by the TDC-scaler data acquisition system. In particular, the atmospheric pressure at the HAWC site exhibits an oscillating behavior with a period of ∼12 hours and we make use of this periodic property to estimate the pressure coefficients for the HAWC TDC-scaler system. These corrections performed on the TDC-scaler system make the HAWC TDC-scaler system an ideal instrument for solar modulation and space-weather studies. As examples of this capability, we present the preliminary analysis of the solar modulation of cosmic rays at three time scales observed by HAWC, with an unprecedented accuracy.
Temperatures obtained from early Cassini infrared observations of Titan show a stratopause at an altitude of 310 kilometers (and 186 kelvin at$15\textdegree S$). Stratospheric temperatures are ...coldest in the winter northern hemisphere, with zonal winds reaching 160 meters per second. The concentrations of several stratospheric organic compounds are enhanced at mid- and high northern latitudes, and the strong zonal winds may inhibit mixing between these latitudes and the rest of Titan. Above the south pole, temperatures in the stratosphere are 4 to 5 kelvin cooler than at the equator. The stratospheric mole fractions of methane and carbon monoxide are ($1.6 \pm 0.5) \times 10^{-2}$and$(4.5 \pm 1.5) \times 10^{-5}$, respectively.
Hard-scattered parton probes produced in collisions of large nuclei indicate large partonic energy loss, possibly with collective produced-medium response to the lost energy. We present measurements ...of π^{0} trigger particles at transverse momenta p{T}{t}=4-12 GeV/c and associated charged hadrons (p{T}{a}=0.5-7 GeV/c) vs relative azimuthal angle Δϕ in Au+Au and p+p collisions at sqrts{NN}=200 GeV. The Au+Au distribution at low p{T}{a}, whose shape has been interpreted as a medium effect, is modified for p{T}{t}<7 GeV/c. At higher p{T}{t}, the data are consistent with unmodified or very weakly modified shapes, even for the lowest measured p{T}{a}, which quantitatively challenges some medium response models. The associated yield of hadrons opposing the trigger particle in Au+Au relative to p+p (I{AA}) is suppressed at high p{T} (I{AA}≈0.35-0.5), but less than for inclusive suppression (R{AA}≈0.2).
Abstract A flavour-tagged time-dependent angular analysis of $${{B} ^0_{s}} \!\rightarrow {{J /\psi }} \phi $$ B s 0 → J / ψ ϕ decays is presented where the $${J /\psi }$$ J / ψ meson is ...reconstructed through its decay to an $$e ^+e ^-$$ e + e - pair. The analysis uses a sample of pp collision data recorded with the LHCb experiment at centre-of-mass energies of 7 and $$8\text {\,Te V} $$ 8 \,Te V , corresponding to an integrated luminosity of $$3 \text {\,fb} ^{-1} $$ 3 \,fb - 1 . The $$C\!P$$ C P -violating phase and lifetime parameters of the $${B} ^0_{s} $$ B s 0 system are measured to be $${\phi _{{s}}} =0.00\pm 0.28\pm 0.07\text {\,rad}$$ ϕ s = 0.00 ± 0.28 ± 0.07 \,rad , $${\Delta \Gamma _{{s}}} =0.115\pm 0.045\pm 0.011\text {\,ps} ^{-1} $$ Δ Γ s = 0.115 ± 0.045 ± 0.011 \,ps - 1 and $${\Gamma _{{s}}} =0.608\pm 0.018\pm 0.012\text {\,ps} ^{-1} $$ Γ s = 0.608 ± 0.018 ± 0.012 \,ps - 1 where the first uncertainty is statistical and the second systematic. This is the first time that $$C\!P$$ C P -violating parameters are measured in the $${{B} ^0_{s}} \!\rightarrow {{J /\psi }} \phi $$ B s 0 → J / ψ ϕ decay with an $$e ^+e ^-$$ e + e - pair in the final state. The results are consistent with previous measurements in other channels and with the Standard Model predictions.
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