95 research outputs found
Large Faraday effect of borate glasses with high Tb³⁺ content prepared by containerless processing
Borate glasses containing a large amount of Tb³⁺ ions have been prepared by containerless processing. The content of Tb₂O₃ reached 60 mol%. The glass bearing the highest content of Tb³⁺ ions showed a large Faraday effect; the Verdet constant was 234 rad/T m. Annealing of the glasses in H₂/N₂ atmosphere resulted in a low optical absorption coefficient, leading to an extremely large magneto-optical figure of merit that was ∼1.7 times higher than that of Tb₃Ga₅O₁₂ single crystal
Increased CaV1.2 late current by a CACNA1C p.R412M variant causes an atypical Timothy syndrome without syndactyly
Timothy syndrome (TS) is a rare pleiotropic disorder associated with long QT syndrome, syndactyly, dysmorphic features, and neurological symptoms. Several variants in exon 8 or 8a of CACNA1C, a gene encoding the α-subunit of voltage-gated Ca2+ channels (Cav1.2), are known to cause classical TS. We identified a p.R412M (exon 9) variant in an atypical TS case. The aim of this study was to examine the functional effects of CACNA1C p.R412M on CaV1.2 in comparison with those of p.G406R. The index patient was a 2-month-old female infant who suffered from a cardio-pulmonary arrest in association with prolonged QT intervals. She showed dysmorphic facial features and developmental delay, but not syndactyly. Interestingly, she also presented recurrent seizures from 4 months. Genetic tests identified a novel heterozygous CACNA1C variant, p.R412M. Using heterologous expression system with HEK-293 cells, analyses with whole-cell patch-clamp technique revealed that p.R412M caused late Ca2+ currents by significantly delaying CaV1.2 channel inactivation, consistent with the underlying mechanisms of classical TS. A novel CACNA1C variant, p.R412M, was found to be associated with atypical TS through the same mechanism as p.G406R, the variant responsible for classical TS
Belediye Müzesi
Taha Toros Arşivi, Dosya No: 114-Müzelerİstanbul Kalkınma Ajansı (TR10/14/YEN/0033) İstanbul Development Agency (TR10/14/YEN/0033
Combined Effects of Summer Water Temperature and Current Velocity on the Distribution of a Cold-Water-Adapted Sculpin (Cottus nozawae)
Clarifying the combined effects of water temperature and other environmental factors on the species distributions of cold-water fishes is the first step toward obtaining a better understanding of the complex impacts of climate warming on these species. In the present study, we examined the abundance and occurrence of the fluvial sculpin, Cottus nozawae, in response to water temperature along environmental gradients in northern Japan. The abundance survey was conducted in the Sorachi River catchment with two-pass electrofishing with a backpack electrofisher. For the occurrence survey, we carried out one-pass electrofishing in the Sorachi, Chitose, and Tokachi River catchments. Fish sampling was conducted once from July to August 2018 in the Sorachi River catchment, from May to June 2011 in the Chitose River catchment, and from July to September 2012 in the Tokachi River catchment. Generalized linear mixed models (GLMMs) and generalized linear models (GLMs) were used for the abundance and occurrence analyses, respectively. We found that the mean summer water temperature was the most influential factor on the distribution of C. nozawae; the abundance and occurrence were both negatively affected by increased water temperatures. In the occurrence model, occurrence probabilities of 0.9 and 0.5 for C. nozawae corresponded to mean summer temperatures of 12.0 and 16.1 degrees C, respectively. Furthermore, we identified a combined effect of water temperature and current velocity on the abundance of C. nozawae. The increased mean summer water temperature had a stronger negative effect on C. nozawae abundance under gentle flow conditions. While the precise mechanisms of this combined effect could not be determined in this study, stressors associated with low current velocities may increase their vulnerability to higher water temperatures. Our findings indicate that flow disturbances caused by human activities such as excessive water abstraction may exacerbate the negative impacts of climate warming on populations of C. nozawae in the future
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