Dominant mutations in ubiquitously expressed transfer RNA (tRNA) synthetase genes cause axonal peripheral neuropathy, accounting for at least six forms of Charcot-Marie-Tooth (CMT) disease. Genetic ...evidence in mouse and
models suggests a gain-of-function mechanism. In this study, we used in vivo, cell type–specific transcriptional and translational profiling to show that mutant tRNA synthetases activate the integrated stress response (ISR) through the sensor kinase GCN2 (general control nonderepressible 2). The chronic activation of the ISR contributed to the pathophysiology, and genetic deletion or pharmacological inhibition of
alleviated the peripheral neuropathy. The activation of GCN2 suggests that the aberrant activity of the mutant tRNA synthetases is still related to translation and that inhibiting GCN2 or the ISR may represent a therapeutic strategy in CMT.
We present the performances of a 330 g zinc molybdate (ZnMoO
4
) crystal working as scintillating bolometer as a possible candidate for a next generation experiment to search for neutrinoless double ...beta decay of
100
Mo. The energy resolution, evaluated at the 2615 keV
γ
-line of
208
Tl, is 6.3 keV FWHM. The internal radioactive contaminations of the ZnMoO
4
were evaluated as <6 μBq/kg (
228
Th) and 27±6 μBq/kg (
226
Ra). We also present the results of the
α
vs
β
/
γ
discrimination, obtained through the scintillation light as well as through the study of the shape of the thermal signal alone.
The CRESST experiment is a direct dark matter search which aims to measure interactions of potential dark matter particles in an Earth-bound detector. With the current stage, CRESST-III, we focus on ...a low energy threshold for increased sensitivity towards light dark matter particles. In this paper we describe the analysis of one detector operated in the first run of CRESST-III (05/2016–02/2018) achieving a nuclear recoil threshold of 30.1 eV. This result was obtained with a 23.6 g CaWO4 crystal operated as a cryogenic scintillating calorimeter in the CRESST setup at the Laboratori Nazionali del Gran Sasso (LNGS). Both the primary phonon (heat) signal and the simultaneously emitted scintillation light, which is absorbed in a separate silicon-on-sapphire light absorber, are measured with highly sensitive transition edge sensors operated at ∼15 mK. The unique combination of these sensors with the light element oxygen present in our target yields sensitivity to dark matter particle masses as low as 160 MeV/c2.
.
Archaeological Roman lead (Pb) is known to be a suitable material for shielding experimental apparata in rare event searches. In the past years the intrinsic radiopurity of this material was ...investigated using different technologies. In this work we applied the latest advancements in cryogenic techniques to study the bulk radiopurity of a 1cm^3 sample of archaeological Roman Pb. We report the lowest ever measured limit on
210
Pb content in Roman Pb, with a concentration lower than 715μBq/kg. Furthermore, we also studied
238
U and
232
Th impurity concentrations. Our values concur with independent measurements reported in literature.
The suppression of spurious events in the region of interest for neutrinoless double beta decay will play a major role in next generation experiments. The background of detectors based on the ...technology of cryogenic calorimeters is expected to be dominated by
α
particles, that could be disentangled from double beta decay signals by exploiting the difference in the emission of the scintillation light. CUPID-0, an array of enriched Zn
82
Se scintillating calorimeters, is the first large mass demonstrator of this technology. The detector started data-taking in 2017 at the Laboratori Nazionali del Gran Sasso with the aim of proving that dual read-out of light and heat allows for an efficient suppression of the
α
background. In this paper we describe the software tools we developed for the analysis of scintillating calorimeters and we demonstrate that this technology allows to reach an unprecedented background for cryogenic calorimeters.
We present the performances of two 92% enriched 130TeO2 crystals operated as thermal bolometers in view of a next generation experiment to search for neutrinoless double beta decay of 130Te. The ...crystals, 435 g each, show an energy resolution, evaluated at the 2615 keV γ-line of 208Tl, of 6.5 and 4.3 keV FWHM. The only observable internal radioactive contamination arises from 238U (15 and 8 μBq/kg, respectively). The internal activity of the most problematic nuclei for neutrinoless double beta decay, 226Ra and 228Th, are both evaluated as <3.1 μBq/kg for one crystal and <2.3 μBq/kg for the second. Thanks to the readout of the weak Cherenkov light emitted by β/γ particles by means of Neganov–Luke bolometric light detectors we were able to perform an event-by-event identification of β/γ events with a 95% acceptance level, while establishing a rejection factor of 98.21% and 99.99% for α particles.
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