1,114 research outputs found
Mutations of the ret protooncogene in German multiple endocrine neoplasia families: Relation between genotype and phenotype.
It has been suggested that not only the position but also the nature of the mutations of the ret protooncogene strongly correlate with the clinical manifestation of the multiple endocrine neoplasm type 2 (MEN 2) syndrome. In particular, individuals with a Cys634-Arg substitution should have a greater risk of developing parathyroid disease. We, therefore, analyzed 94 unrelated families from Germany with inherited medullary thyroid carcinoma (MTC) for mutation of the ret protooncogene. In all but 1 of 59 families with MEN 2A, germline mutations in the extracellular domain of the ret protein were found. Some 81% of the MEN 2A mutations affected codon 634. Phenotype-genotype correlations suggested that the prevalence of pheochromocytoma and hyperparathyroidism is significantly higher in families with codon 634 mutations, but there was no correlation with the nature of the mutation. In all but 1 of 27 familial MTC (FMTC) families, mutations were detected in 1 of 4 cysteines in the extracellular domain of the ret protooncogene. Half of the FMTC mutations affected codon 634. Mutations outside of codon 634 occurred more often in FMTC families than in MEN 2A families. In all but 1 of 8 MEN 2B patients, de novo mutations in codon 918 were found. These data confirm the preferential localization of MEN 2-associated mutations and the correlation between disease phenotype and the position of the ret mutation, but there was no correlation between the occurrence of hyperparathyroidism or pheochromocytoma and the nature of the mutation
A new method of reconstructing very-high-energy gamma-ray spectra: the Template Background Spectrum
Very-high-energy (VHE, E>0.1 TeV) gamma-ray emission regions with angular
extents comparable to the field-of-view of current imaging air-Cherenkov
telescopes (IACT) require additional observations of source-free regions to
estimate the background contribution to the energy spectrum. This reduces the
effective observation time and deteriorates the sensitivity. A new method of
reconstructing spectra from IACT data without the need of additional
observations of source-free regions is developed. Its application is not
restricted to any specific IACT or data format. On the basis of the template
background method, which defines the background in air-shower parameter space,
a new spectral reconstruction method from IACT data is developed and studied,
the Template Background Spectrum (TBS); TBS is tested on published H.E.S.S.
data and H.E.S.S. results. Good agreement is found between VHE gamma-ray
spectra reported by the H.E.S.S. collaboration and those re-analysed with TBS.
This includes analyses of point-like sources, sources in crowded regions, and
of very extended sources down to sources with fluxes of a few percent of the
Crab Nebula flux and excess-to-background ratios around 0.1. However, the TBS
background normalisation introduces new statistical and systematic errors which
are accounted for, but may constitute a limiting case for very faint extended
sources. The TBS method enables the spectral reconstruction of data when other
methods are hampered or even fail. It does not need dedicated observations of
VHE gamma-ray-free regions (e.g. as the On/Off background does) and circumvents
known geometrical limitations to which other methods (e.g. the reflected-region
background) for reconstructing spectral information of VHE gamma-ray emission
regions are prone to; TBS would be, in specific cases, the only feasible way to
reconstruct energy spectra.Comment: 18 pages, accepted for publication (Astronomy and Astrophysics
A Spectacular VHE Gamma-Ray Outburst from PKS 2155-304 in 2006
Since 2002 the VHE (>100 GeV) gamma-ray flux of the high-frequency peaked BL
Lac PKS 2155-304 has been monitored with the High Energy Stereoscopic System
(HESS). An extreme gamma-ray outburst was detected in the early hours of July
28, 2006 (MJD 53944). The average flux above 200 GeV observed during this
outburst is ~7 times the flux observed from the Crab Nebula above the same
threshold. Peak fluxes are measured with one-minute time scale resolution at
more than twice this average value. Variability is seen up to ~600 s in the
Fourier power spectrum, and well-resolved bursts varying on time scales of ~200
seconds are observed. There are no strong indications for spectral variability
within the data. Assuming the emission region has a size comparable to the
Schwarzschild radius of a ~10^9 solar mass black hole, Doppler factors greater
than 100 are required to accommodate the observed variability time scales.Comment: 4 pages, 3 figures; To appear in the Proceedings of the 30th ICRC
(Merida, Mexico
Electronic Structure of Transition Metals Fe, Ni and Cu in the GW Approximation
The quasiparticle band structures of 3d transition metals, ferromagnetic Fe,
Ni and paramagnetic Cu, are calculated by the GW approximation. The width of
occupied 3d valence band, which is overestimated in the LSDA, is in good
agreement with experimental observation. However the exchange splitting and
satellite in spectra are not reproduced and it is required to go beyond the GW
approximation. The effects of static screening and dynamical correlation are
discussed in detail in comparison with the results of the static COHSEX
approximation. The dynamical screening effects are important for band width
narrowing.Comment: 4 pages, 3 figure
First detection of a VHE gamma-ray spectral maximum from a Cosmic source: H.E.S.S. discovery of the Vela X nebula
The Vela supernova remnant (SNR) is a complex region containing a number of
sources of non-thermal radiation. The inner section of this SNR, within 2
degrees of the pulsar PSR B0833-45, has been observed by the H.E.S.S. gamma-ray
atmospheric Cherenkov detector in 2004 and 2005. A strong signal is seen from
an extended region to the south of the pulsar, within an integration region of
radius 0.8 deg. around the position (RA = 08h 35m 00s, dec = -45 deg. 36'
J2000.0). The excess coincides with a region of hard X-ray emission seen by the
ROSAT and ASCA satellites. The observed energy spectrum of the source between
550 GeV and 65 TeV is well fit by a power law function with photon index = 1.45
+/- 0.09(stat) +/- 0.2(sys) and an exponential cutoff at an energy of 13.8 +/-
2.3(stat) +/- 4.1(sys) TeV. The integral flux above 1 TeV is (1.28 +/- 0.17
(stat) +/- 0.38(sys)) x 10^{-11} cm^{-2} s^{-1}. This result is the first clear
measurement of a peak in the spectral energy distribution from a VHE gamma-ray
source, likely related to inverse Compton emission. A fit of an Inverse Compton
model to the H.E.S.S. spectral energy distribution gives a total energy in
non-thermal electrons of ~2 x 10^{45} erg between 5 TeV and 100 TeV, assuming a
distance of 290 parsec to the pulsar. The best fit electron power law index is
2.0, with a spectral break at 67 TeV.Comment: 5 pages, 4 figures, accepted for publication in Astronomy and
Astrophysics letter
Observations of Mkn 421 in 2004 with H.E.S.S. at large zenith angles
Mkn 421 was observed during a high flux state for nine nights in April and
May 2004 with the fully operational High Energy Stereoscopic System (H.E.S.S.)
in Namibia. The observations were carried out at zenith angles of
60--65, which result in an average energy threshold of 1.5 TeV
and a collection area reaching 2~km at 10~TeV. Roughly 7000 photons from
Mkn~421 were accumulated with an average gamma-ray rate of 8 photons/min. The
overall significance of the detection exceeds 100 standard deviations. The
light-curve of integrated fluxes above 2~TeV shows changes of the diurnal flux
up to a factor of 4.3. For nights of high flux, intra-night variability is
detected with a decay time of less than 1 hour. The time averaged energy
spectrum is curved and is well described by a power-law with a photon index
\egamm and an exponential cutoff at \ecut~TeV and an average integral flux
above 2~TeV of 3 Crab flux units. Significant variations of the spectral shape
are detected with a spectral hardening as the flux increases. Contemporaneous
multi-wavelength observations at lower energies (X-rays and gamma-rays above
~GeV) indicate smaller relative variability amplitudes than seen
above 2~TeV during high flux state observed in April 2004.Comment: 5 pages, 4 figures, published in A&
Discovery of the Binary Pulsar PSR B1259-63 in Very-High-Energy Gamma Rays around Periastron with H.E.S.S
We report the discovery of very-high-energy (VHE) gamma-ray emission of the
binary system PSR B1259-63/SS 2883 of a radio pulsar orbiting a massive,
luminous Be star in a highly eccentric orbit. The observations around the 2004
periastron passage of the pulsar were performed with the four 13 m Cherenkov
telescopes of the H.E.S.S. experiment, recently installed in Namibia and in
full operation since December 2003. Between February and June 2004, a gamma-ray
signal from the binary system was detected with a total significance above 13
sigma. The flux was found to vary significantly on timescales of days which
makes PSR B1259-63 the first variable galactic source of VHE gamma-rays
observed so far. Strong emission signals were observed in pre- and
post-periastron phases with a flux minimum around periastron, followed by a
gradual flux decrease in the months after. The measured time-averaged energy
spectrum above a mean threshold energy of 380 GeV can be fitted by a simple
power law F_0(E/1 TeV)^-Gamma with a photon index Gamma =
2.7+-0.2_stat+-0.2_sys and flux normalisation F_0 = (1.3+-0.1_stat+-0.3_sys)
10^-12 TeV^-1 cm^-2 s^-1. This detection of VHE gamma-rays provides unambiguous
evidence for particle acceleration to multi-TeV energies in the binary system.
In combination with coeval observations of the X-ray synchrotron emission by
the RXTE and INTEGRAL instruments, and assuming the VHE gamma-ray emission to
be produced by the inverse Compton mechanism, the magnetic field strength can
be directly estimated to be of the order of 1 G.Comment: 10 pages, 8 figures, accepted in Astronomy and Astrophysics on 2 June
2005, replace: document unchanged, replaced author field in astro-ph entry -
authors are all members of the H.E.S.S. collaboration and three additional
authors (99+3, see document
A possible association of the new VHE gamma-ray source HESS J1825--137 with the pulsar wind nebula G18.0--0.7
We report on a possible association of the recently discovered very
high-energy -ray source HESS J1825--137 with the pulsar wind nebula
(commonly referred to as G 18.0--0.7) of the year old
Vela-like pulsar PSR B1823--13. HESS J1825--137 was detected with a
significance of 8.1 in the Galactic Plane survey conducted with the
H.E.S.S. instrument in 2004. The centroid position of HESS J1825--137 is offset
by 11\arcmin south of the pulsar position. \emph{XMM-Newton} observations have
revealed X-ray synchrotron emission of an asymmetric pulsar wind nebula
extending to the south of the pulsar. We argue that the observed morphology and
TeV spectral index suggest that HESS J1825--137 and G 18.0--0.7 may be
associated: the lifetime of TeV emitting electrons is expected to be longer
compared to the {\it XMM-Newton} X-ray emitting electrons, resulting in
electrons from earlier epochs (when the spin-down power was larger)
contributing to the present TeV flux. These electrons are expected to be
synchrotron cooled, which explains the observed photon index of , and
the longer lifetime of TeV emitting electrons naturally explains why the TeV
nebula is larger than the X-ray size. Finally, supernova remnant expansion into
an inhomogeneous medium is expected to create reverse shocks interacting at
different times with the pulsar wind nebula, resulting in the offset X-ray and
TeV -ray morphology.Comment: 5 pages, 3 figures, to appear in Astronomy and Astrophysics Letter
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