Effect of low-temperature neutron irradiation on the properties of titanium beryllide

Beryllium-based intermetallic compounds, such as Be12Ti, are increasingly being considered as a material capable of replacing pure beryllium in structural elements of fusion reactors. Be12Ti is considered as a neutron breeder material, a structural part of the Helium Cooled Pebble Bed of the DEMO reactor. It is expected that the replacement of beryllium by Be12Ti will make it possible to reduce the capture of tritium in the blanket without a significant decrease in the neutronic characteristics. Unlike beryllium, beryllides have relatively recently begun to be considered for use in nuclear and thermonuclear facilities, so the radiation resistance of these compounds remains little studied. This paper presents the experimental results on effect of low temperature neutron irradiation to properties of titanium beryllide samples manufactured by industrial technology in the Ulba Metallurgical Plant (UMP, Kazakhstan). The manufactured samples before and after irradiation were analyzed by scanning electron microscopy (SEM), X-Ray diffraction (XRD), hydrostatic weighing method, dimension method and microhardness measurement by Vickers method

Kctd7 deficiency induces myoclonic seizures associated with Purkinje cell death and microvascular defects

Mutations in the potassium channel tetramerization domain-containing 7 (KCTD7) gene are associated with a severe neurodegenerative phenotype characterized by childhood onset of progressive and intractable myoclonic seizures accompanied by developmental regression. KCTD7-driven disease is part of a large family of progressive myoclonic epilepsy syndromes displaying a broad spectrum of clinical severity. Animal models of KCTD7-related disease are lacking, and little is known regarding how KCTD7 protein defects lead to epilepsy and cognitive dysfunction. We characterized Kctd7 expression patterns in the mouse brain during development and show that it is selectively enriched in specific regions as the brain matures. We further demonstrate that Kctd7-deficient mice develop seizures and locomotor defects with features similar to those observed in human KCTD7-associated diseases. We also show that Kctd7 is required for Purkinje cell survival in the cerebellum and that selective degeneration of these neurons is accompanied by defects in cerebellar microvascular organization and patterning. Taken together, these results define a new model for KCTD7-associated epilepsy and identify Kctd7 as a modulator of neuron survival and excitability linked to microvascular alterations in vulnerable regions