856 research outputs found
Congenital macrothrombocytopenia with focal myelofibrosis due to mutations in human G6b-B is rescued in humanized mice.
Unlike primary myelofibrosis (PMF) in adults, myelofibrosis in children is rare. Congenital (inherited) forms of myelofibrosis (cMF) have been described, but the underlying genetic mechanisms remain elusive. Here we describe 4 families with autosomal recessive inherited macrothrombocytopenia with focal myelofibrosis due to germ line loss-of-function mutations in the megakaryocyte-specific immunoreceptor tyrosine-based inhibitory motif (ITIM)-containing receptor G6b-B (G6b, C6orf25, or MPIG6B). Patients presented with a mild-to-moderate bleeding diathesis, macrothrombocytopenia, anemia, leukocytosis and atypical megakaryocytes associated with a distinctive, focal, perimegakaryocytic pattern of bone marrow fibrosis. In addition to identifying the responsible gene, the description of G6b-B as the mutated protein potentially implicates aberrant G6b-B megakaryocytic signaling and activation in the pathogenesis of myelofibrosis. Targeted insertion of human G6b in mice rescued the knockout phenotype and a copy number effect of human G6b-B expression was observed. Homozygous knockin mice expressed 25% of human G6b-B and exhibited a marginal reduction in platelet count and mild alterations in platelet function; these phenotypes were more severe in heterozygous mice that expressed only 12% of human G6b-B. This study establishes G6b-B as a critical regulator of platelet homeostasis in humans and mice. In addition, the humanized G6b mouse will provide an invaluable tool for further investigating the physiological functions of human G6b-B as well as testing the efficacy of drugs targeting this receptor
An improved method for measuring muon energy using the truncated mean of dE/dx
The measurement of muon energy is critical for many analyses in large
Cherenkov detectors, particularly those that involve separating
extraterrestrial neutrinos from the atmospheric neutrino background. Muon
energy has traditionally been determined by measuring the specific energy loss
(dE/dx) along the muon's path and relating the dE/dx to the muon energy.
Because high-energy muons (E_mu > 1 TeV) lose energy randomly, the spread in
dE/dx values is quite large, leading to a typical energy resolution of 0.29 in
log10(E_mu) for a muon observed over a 1 km path length in the IceCube
detector. In this paper, we present an improved method that uses a truncated
mean and other techniques to determine the muon energy. The muon track is
divided into separate segments with individual dE/dx values. The elimination of
segments with the highest dE/dx results in an overall dE/dx that is more
closely correlated to the muon energy. This method results in an energy
resolution of 0.22 in log10(E_mu), which gives a 26% improvement. This
technique is applicable to any large water or ice detector and potentially to
large scintillator or liquid argon detectors.Comment: 12 pages, 16 figure
Demonstration of the temporal matter-wave Talbot effect for trapped matter waves
We demonstrate the temporal Talbot effect for trapped matter waves using
ultracold atoms in an optical lattice. We investigate the phase evolution of an
array of essentially non-interacting matter waves and observe matter-wave
collapse and revival in the form of a Talbot interference pattern. By using
long expansion times, we image momentum space with sub-recoil resolution,
allowing us to observe fractional Talbot fringes up to 10th order.Comment: 17 pages, 7 figure
Azimuthal anisotropy and correlations in p+p, d+Au and Au+Au collisions at 200 GeV
We present the first measurement of directed flow () at RHIC. is
found to be consistent with zero at pseudorapidities from -1.2 to 1.2,
then rises to the level of a couple of percent over the range . The latter observation is similar to data from NA49 if the SPS rapidities
are shifted by the difference in beam rapidity between RHIC and SPS.
Back-to-back jets emitted out-of-plane are found to be suppressed more if
compared to those emitted in-plane, which is consistent with {\it jet
quenching}. Using the scalar product method, we systematically compared
azimuthal correlations from p+p, d+Au and Au+Au collisions. Flow and non-flow
from these three different collision systems are discussed.Comment: Quark Matter 2004 proceeding, 4 pages, 3 figure
Azimuthal anisotropy: the higher harmonics
We report the first observations of the fourth harmonic (v_4) in the
azimuthal distribution of particles at RHIC. The measurement was done taking
advantage of the large elliptic flow generated at RHIC. The integrated v_4 is
about a factor of 10 smaller than v_2. For the sixth (v_6) and eighth (v_8)
harmonics upper limits on the magnitudes are reported.Comment: 4 pages, 6 figures, contribution to the Quark Matter 2004 proceeding
Kaon Production and Kaon to Pion Ratio in Au+Au Collisions at \snn=130 GeV
Mid-rapidity transverse mass spectra and multiplicity densities of charged
and neutral kaons are reported for Au+Au collisions at \snn=130 GeV at RHIC.
The spectra are exponential in transverse mass, with an inverse slope of about
280 MeV in central collisions. The multiplicity densities for these particles
scale with the negative hadron pseudo-rapidity density. The charged kaon to
pion ratios are and
for the most central collisions. The ratio is lower than the same
ratio observed at the SPS while the is higher than the SPS result.
Both ratios are enhanced by about 50% relative to p+p and +p
collision data at similar energies.Comment: 6 pages, 3 figures, 1 tabl
Partonic flow and -meson production in Au+Au collisions at = 200 GeV
We present first measurements of the -meson elliptic flow
() and high statistics distributions for different
centralities from = 200 GeV Au+Au collisions at RHIC. In
minimum bias collisions the of the meson is consistent with the
trend observed for mesons. The ratio of the yields of the to those of
the as a function of transverse momentum is consistent with a model
based on the recombination of thermal quarks up to GeV/,
but disagrees at higher momenta. The nuclear modification factor () of
follows the trend observed in the mesons rather than in
baryons, supporting baryon-meson scaling. Since -mesons are
made via coalescence of seemingly thermalized quarks in central Au+Au
collisions, the observations imply hot and dense matter with partonic
collectivity has been formed at RHIC.Comment: 6 pages, 4 figures, submit to PR
Measurement of the B0-anti-B0-Oscillation Frequency with Inclusive Dilepton Events
The - oscillation frequency has been measured with a sample of
23 million \B\bar B pairs collected with the BABAR detector at the PEP-II
asymmetric B Factory at SLAC. In this sample, we select events in which both B
mesons decay semileptonically and use the charge of the leptons to identify the
flavor of each B meson. A simultaneous fit to the decay time difference
distributions for opposite- and same-sign dilepton events gives ps.Comment: 7 pages, 1 figure, submitted to Physical Review Letter
Plasma Wakefield Acceleration with a Modulated Proton Bunch
The plasma wakefield amplitudes which could be achieved via the modulation of
a long proton bunch are investigated. We find that in the limit of long bunches
compared to the plasma wavelength, the strength of the accelerating fields is
directly proportional to the number of particles in the drive bunch and
inversely proportional to the square of the transverse bunch size. The scaling
laws were tested and verified in detailed simulations using parameters of
existing proton accelerators, and large electric fields were achieved, reaching
1 GV/m for LHC bunches. Energy gains for test electrons beyond 6 TeV were found
in this case.Comment: 9 pages, 7 figure
The energy dependence of angular correlations inferred from mean- fluctuation scale dependence in heavy ion collisions at the SPS and RHIC
We present the first study of the energy dependence of angular
correlations inferred from event-wise mean transverse momentum
fluctuations in heavy ion collisions. We compare our large-acceptance
measurements at CM energies $\sqrt{s_{NN}} =$ 19.6, 62.4, 130 and 200 GeV to
SPS measurements at 12.3 and 17.3 GeV. $p_t$ angular correlation structure
suggests that the principal source of $p_t$ correlations and fluctuations is
minijets (minimum-bias parton fragments). We observe a dramatic increase in
correlations and fluctuations from SPS to RHIC energies, increasing linearly
with $\ln \sqrt{s_{NN}}$ from the onset of observable jet-related
fluctuations near 10 GeV.Comment: 10 pages, 4 figure
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