26 research outputs found

    Ultraviolet Observations of Super-Chandrasekhar Mass Type Ia Supernova Candidates with Swift UVOT

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    Among Type Ia supernovae (SNe~Ia) exist a class of overluminous objects whose ejecta mass is inferred to be larger than the canonical Chandrasekhar mass. We present and discuss the UV/optical photometric light curves, colors, absolute magnitudes, and spectra of three candidate Super-Chandrasekhar mass SNe--2009dc, 2011aa, and 2012dn--observed with the Swift Ultraviolet/Optical Telescope. The light curves are at the broad end for SNe Ia, with the light curves of SN~2011aa being amongst the broadest ever observed. We find all three to have very blue colors which may provide a means of excluding these overluminous SNe from cosmological analysis, though there is some overlap with the bluest of "normal" SNe Ia. All three are overluminous in their UV absolute magnitudes compared to normal and broad SNe Ia, but SNe 2011aa and 2012dn are not optically overluminous compared to normal SNe Ia. The integrated luminosity curves of SNe 2011aa and 2012dn in the UVOT range (1600-6000 Angstroms) are only half as bright as SN~2009dc, implying a smaller 56Ni yield. While not enough to strongly affect the bolometric flux, the early time mid-UV flux makes a significant contribution at early times. The strong spectral features in the mid-UV spectra of SNe 2009dc and 2012dn suggest a higher temperature and lower opacity to be the cause of the UV excess rather than a hot, smooth blackbody from shock interaction. Further work is needed to determine the ejecta and 56Ni masses of SNe 2011aa and 2012dn and fully explain their high UV luminosities.Comment: 12 pages, 8 figures Accepted for publication in the Astrophysical Journal Data available on the Swift supernova website http://swift.gsfc.nasa.gov/docs/swift/sne/swift_sn.htm

    THE 1999aa-LIKE TYPE Ia SUPERNOVA IPTF14BDN IN THE ULTRAVIOLET AND OPTICAL

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    We present ultraviolet (UV) and optical photometry and spectra of the 1999aa-like supernova (SN) iPTF14bdn. The UV data were observed using the Swift Ultraviolet/Optical Telescope (UVOT) and constitute the first UV spectral series of a 1999aa-like SN. From the photometry we measure Δm15(B)=0.84±0.05\Delta m_{15}({\it B})\,=\,0.84 \pm0.05 mag and blue UV colors at epochs earlier than 5-5 days. The spectra show that the early-time blue colors are the result of less absorption between 28003200A˚ 2800 - 3200 \,\AA~ than is present in normal SNe Ia. Using model spectra fits of the data at 10-10 and +10+10 days, we identify the origin of this spectral feature to be a temperature effect in which doubly ionized iron group elements create an opacity 'window'. We determine that the detection of high temperatures and large quantities of iron group elements at early epochs imply the mixing of a high Ni mass into the outer layers of the SN ejecta. We also identify the source of the I-band secondary maximum in iPTF14bdn to be the decay of Fe III to Fe II, as is seen in normal SNe Ia.Comment: 10 pages, 11 figures, 4 tables. Accepted to Ap

    The clinical and genetic spectrum of autosomal-recessive TOR1A-related disorders.

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    In the field of rare diseases, progress in molecular diagnostics led to the recognition that variants linked to autosomal-dominant neurodegenerative diseases of later onset can, in the context of biallelic inheritance, cause devastating neurodevelopmental disorders and infantile or childhood-onset neurodegeneration. TOR1A-associated arthrogryposis multiplex congenita 5 (AMC5) is a rare neurodevelopmental disorder arising from biallelic variants in TOR1A, a gene that in the heterozygous state is associated to torsion dystonia-1 (DYT1 or DYT-TOR1A), an early-onset dystonia with reduced penetrance. While 15 individuals with TOR1A-AMC5 have been reported (less than 10 in detail), a systematic investigation of the full disease-associated spectrum has not been conducted. Here, we assess the clinical, radiological and molecular characteristics of 57 individuals from 40 families with biallelic variants in TOR1A. Median age at last follow-up was 3 years (0-24 years). Most individuals presented with severe congenital flexion contractures (95%) and variable developmental delay (79%). Motor symptoms were reported in 79% and included lower limb spasticity and pyramidal signs, as well as gait disturbances. Facial dysmorphism was an integral part of the phenotype, with key features being a broad/full nasal tip, narrowing of the forehead and full cheeks. Analysis of disease-associated manifestations delineated a phenotypic spectrum ranging from normal cognition and mild gait disturbance to congenital arthrogryposis, global developmental delay, intellectual disability, absent speech and inability to walk. In a subset, the presentation was consistent with fetal akinesia deformation sequence with severe intrauterine abnormalities. Survival was 71% with higher mortality in males. Death occurred at a median age of 1.2 months (1 week - 9 years) due to respiratory failure, cardiac arrest, or sepsis. Analysis of brain MRI studies identified non-specific neuroimaging features, including a hypoplastic corpus callosum (72%), foci of signal abnormality in the subcortical and periventricular white matter (55%), diffuse white matter volume loss (45%), mega cisterna magna (36%) and arachnoid cysts (27%). The molecular spectrum included 22 distinct variants, defining a mutational hotspot in the C-terminal domain of the Torsin-1A protein. Genotype-phenotype analysis revealed an association of missense variants in the 3-helix bundle domain to an attenuated phenotype, while missense variants near the Walker A/B motif as well as biallelic truncating variants were linked to early death. In summary, this systematic cross-sectional analysis of a large cohort of individuals with biallelic TOR1A variants across a wide age-range delineates the clinical and genetic spectrum of TOR1A-related autosomal-recessive disease and highlights potential predictors for disease severity and survival

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