Dielectric Characteristics, Electrical Conductivity and Solvation of Ions in Electrolyte Solutions.

Solvation and association of ions in solutions largely depend on the dielectric properties of the solvent, the distance between ions in solutions, and temperature. This paper considers the effect of temperature on static dielectric constant (DC), dipole dielectric relaxation (DR) time, and limiting (ultimate) high frequency (HF) electrical conductivity (EC) of water and some polar solvents. In the investigated temperature range (0-370 °C), the static DC and DR time of water decrease, and limiting HF EC passes through a maximum at 250-300 °C with temperature growth. The dielectric characteristics of methanol, ethanol, and propanol behave in a similar way. It is shown that the existence of an HF EC temperature maximum is due to the different nature of the temperature dependences of DC and DR time. It is suggested that the same dependences are responsible for the presence of a maximum in the temperature dependences of the dissociation degree and the ionic product of water. The influence of non-electrolytes concentration as well as metal salts on the dielectric properties of their aqueous solutions is considered. The limiting HF EC of water determines the specific EC value of aqueous electrolyte solutions. Analysis of the absorption of microwave energy by polar solvents, as well as aqueous solutions of non-electrolytes and electrolytes, at a frequency of 2455 MHz is carried out. The optimal conditions for high-frequency heating of solutions have been established. The distance between ions in aqueous solutions of inorganic salts and in non-aqueous solutions of ionic liquids is calculated. It is shown that the maximum on the concentration dependence of the specific EC can be related to ions association.

Ataxia with Oculomotor Apraxia Type 4 with PNKP Common "Portuguese" and Novel Mutations in Two Belarusian Families.

Ataxia with oculomotor apraxia type 4 (AOA4) is a rare autosomal recessive, PNKP -related disorder delineated in 2015 in Portugal. We diagnosed AOA4 by next generation sequencing (NGS) followed by Sanger's sequencing in three boys from two unrelated Belarusian families. In both families, one of the heterozygous PNKP mutations was c.1123G>T, common in Portuguese patients; biallelic mutations, c.1270_1283dup14 and c.1029+2T>C, respectively, were novel. These are the first reported AOA4 Slavic cases and the first with a "Portuguese" PNKP mutation outside Portugal. Distinction in two brothers was microcephaly but their disease was not severe in contrast to PNKP -related "microcephaly, seizures, and developmental delay" and reported cases with features of both phenotypes.