40 research outputs found
Gamma-Ray Bursts: The Underlying Model
A pedagogical derivation is presented of the ``fireball'' model of gamma-ray
bursts, according to which the observable effects are due to the dissipation of
the kinetic energy of a relativistically expanding wind, a ``fireball.'' The
main open questions are emphasized, and key afterglow observations, that
provide support for this model, are briefly discussed. The relativistic outflow
is, most likely, driven by the accretion of a fraction of a solar mass onto a
newly born (few) solar mass black hole. The observed radiation is produced once
the plasma has expanded to a scale much larger than that of the underlying
``engine,'' and is therefore largely independent of the details of the
progenitor, whose gravitational collapse leads to fireball formation. Several
progenitor scenarios, and the prospects for discrimination among them using
future observations, are discussed. The production in gamma- ray burst
fireballs of high energy protons and neutrinos, and the implications of burst
neutrino detection by kilometer-scale telescopes under construction, are
briefly discussed.Comment: In "Supernovae and Gamma Ray Bursters", ed. K. W. Weiler, Lecture
Notes in Physics, Springer-Verlag (in press); 26 pages, 2 figure
Clinical spectrum of SIX3-associated mutations in holoprosencephaly: correlation between genotype, phenotype and function
BACKGROUND: Holoprosencephaly (HPE) is the most common structural malformation of the human forebrain. There are several important HPE mutational target genes, including the transcription factor SIX3, which encodes an early regulator of Shh, Wnt, Bmp and Nodal signalling expressed in the developing forebrain and eyes of all vertebrates. OBJECTIVE: To characterise genetic and clinical findings in patients with SIX3 mutations. METHODS: Patients with HPE and their family members were tested for mutations in HPE-associated genes and the genetic and clinical findings, including those for additional cases found in the literature, were analysed. The results were correlated with a mutation-specific functional assay in zebrafish. RESULTS: In a cohort of patients (n = 800) with HPE, SIX3 mutations were found in 4.7% of probands and additional cases were found through testing of relatives. In total, 138 cases of HPE were identified, 59 of whom had not previously been clinically presented. Mutations in SIX3 result in more severe HPE than in other cases of non-chromosomal, non-syndromic HPE. An over-representation of severe HPE was found in patients whose mutations confer greater loss of function, as measured by the functional zebrafish assay. The gender ratio in this combined set of patients was 1.5:1 (F:M) and maternal inheritance was almost twice as common as paternal. About 14% of SIX3 mutations in probands occur de novo. There is a wide intrafamilial clinical range of features and classical penetrance is estimated to be at least 62%. CONCLUSIONS: Our data suggest that SIX3 mutations result in relatively severe HPE and that there is a genotype-phenotype correlation, as shown by functional studies using animal models
The electromagnetic counterpart of the binary neutron star merger LIGO/Virgo GW170817. I. Discovery of the optical counterpart using the Dark Energy Camera
We present the Dark Energy Camera (DECam) discovery of the optical counterpart of the first binary neutron star merger detected through gravitational wave emission, GW170817. Our observations commenced 10.5 hours post-merger, as soon as the localization region became accessible from Chile. We imaged 70 deg2 in the i and z bands, covering 93% of the initial integrated localization probability, to a depth necessary to identify likely optical counterparts (e.g., a kilonova). At 11.4 hours post-merger we detected a bright optical transient located 10:600 from the nucleus of NGC4993 at redshift z = 0:0098, consistent (for H0 = 70 km s-1 Mpc-1) with the distance of 40±8 Mpc reported by the LIGO Scientific Collaboration and the Virgo Collaboration (LVC). At detection the transient had magnitudes i=17.3 and z=17.4, and thus an absolute magnitude of Mi = -15.7, in the luminosity range expected for a kilonova. We identified 1,500 potential transient candidates. Applying simple selection criteria aimed at rejecting background events such as supernovae, we find the transient associated with NGC4993 as the only remaining plausible counterpart, and reject chance coincidence at the 99.5% confidence level. We therefore conclude that the optical counterpart we have identified near NGC4993 is associated with GW170817. This discovery ushers in the era of multi-messenger astronomy with gravitational waves, and demonstrates the power of DECam to identify the optical counterparts of gravitational-wave sources
Catching Element Formation In The Act ; The Case for a New MeV Gamma-Ray Mission: Radionuclide Astronomy in the 2020s
High Energy Astrophysic