| 1. |
- Abdo, A. A., et al.
(författare)
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Gamma-ray flaring activity from the gravitationally lensed blazar PKS 1830-211 observed by Fermi LAT
- 2015
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Ingår i: Astrophysical Journal. - 0004-637X .- 1538-4357. ; 799:2
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Tidskriftsartikel (refereegranskat)abstract
- The Large Area Telescope ( LAT) on board the FermiGamma- ray Space Telescope routinely detects the MeV- peaked flat- spectrum radio quasar PKS 1830- 211 ( z = 2.507). Its apparent isotropic. - ray luminosity ( E > 100 MeV), averaged over 3 years of observations and peaking on 2010 October 14/ 15 at 2.9 x 1050 erg s- 1, makes it among the brightest high- redshift Fermi blazars. No published model with a single lens can account for all of the observed characteristics of this complex system. Based on radio observations, one expects time- delayed variability to follow about 25 days after a primary flare, with flux about a factor of 1.5 less. Two large. - ray flares of PKS 1830- 211 have been detected by the LAT in the considered period, and no substantial evidence for such a delayed activity was found. This allows us to place a lower limit of about 6 on the. - ray flux ratio between the two lensed images. Swift XRT observations from a dedicated Target of Opportunity program indicate a hard spectrum with no significant correlation of X- ray flux with the. - ray variability. The spectral energy distribution can be modeled with inverse Compton scattering of thermal photons from the dusty torus. The implications of the LAT data in terms of variability, the lack of evident delayed flare events, and different radio and. - ray flux ratios are discussed. Microlensing effects, absorption, size and location of the emitting regions, the complex mass distribution of the system, an energy- dependent inner structure of the source, and flux suppression by the lens galaxy for one image path may be considered as hypotheses for understanding our results.
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| 2. |
- Lam, MT, et al.
(författare)
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A novel disorder involving dyshematopoiesis, inflammation, and HLH due to aberrant CDC42 function
- 2019
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Ingår i: The Journal of experimental medicine. - : Rockefeller University Press. - 1540-9538 .- 0022-1007. ; 216:12, s. 2778-2799
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Tidskriftsartikel (refereegranskat)abstract
- Hemophagocytic lymphohistiocytosis (HLH) is characterized by immune dysregulation due to inadequate restraint of overactivated immune cells and is associated with a variable clinical spectrum having overlap with more common pathophysiologies. HLH is difficult to diagnose and can be part of inflammatory syndromes. Here, we identify a novel hematological/autoinflammatory condition (NOCARH syndrome) in four unrelated patients with superimposable features, including neonatal-onset cytopenia with dyshematopoiesis, autoinflammation, rash, and HLH. Patients shared the same de novo CDC42 mutation (Chr1:22417990C>T, p.R186C) and altered hematopoietic compartment, immune dysregulation, and inflammation. CDC42 mutations had been associated with syndromic neurodevelopmental disorders. In vitro and in vivo assays documented unique effects of p.R186C on CDC42 localization and function, correlating with the distinctiveness of the trait. Emapalumab was critical to the survival of one patient, who underwent successful bone marrow transplantation. Early recognition of the disorder and establishment of treatment followed by bone marrow transplant are important to survival.
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