Origins of SOPH syndrome: A study of 93 Yakut patients with review of C-terminal phenotype.

Since the first report of SOPH syndrome among the Yakut population in 2010, new clinical data of SOPH-like conditions continue to appear. We expand the phenotypic spectrum of SOPH syndrome and perform a comparative analysis of Yakut SOPH patients' clinical data with SOPH-like conditions reported in the world scientific literature to form a foundation for NBAS pathogenesis discussion. Clinical data from the genetic records of 93 patients with SOPH syndrome and global survey data on patients with pathogenic variants of the C-terminal in the NBAS gene were collected.

Highly Washable and Conductive Cotton E-textiles Based on Electrochemically Exfoliated Graphene.

In this study, cotton e-textiles were obtained using two types of graphene oxide. The first type of graphene oxide was synthesized using the Hummers' method. The second type was obtained by the electrochemical exfoliation of graphite in an ammonium salt solution.

Psychological support and strategies for raising motivation in fresh students.

The goal of this study was to investigate the of fear of starting university training, determine its general characteristics, and test how providing psychological and motivational support for university enrollees may influence the elimination of this type of fear. The study used a random sample of 536 individuals, 268 participants each from one university in the Russian Federation and one from China, who were also randomly selected. Approximately equivalent experimental and control groups (134 people) were formed.

The energy release rate for non-penetrating crack in poroelastic body by fluid-driven fracture.

A new class of constrained variational problems, which describe fluid-driven cracks (that are pressurized fractures created by pumping fracturing fluids), is considered within the nonlinear theory of coupled poroelastic models stated in the incremental form. The two-phase medium is constituted by solid particles and fluid-saturated pores; it contains a crack subjected to non-penetration condition between the opposite crack faces. The inequality-constrained optimization is expressed as a saddle-point problem with respect to the unknown solid phase displacement, pore pressure, and contact force.

Mechanical and Tribological Properties of Polytetrafluoroethylene Modified with Combined Fillers: Carbon Fibers, Zirconium Dioxide, Silicon Dioxide and Boron Nitride.

The introduction of combined fillers can effectively improve the mechanical and tribological properties of polytetrafluoroethylene (PTFE). In this work, three different types of nanosized fillers (zirconium dioxide, silicon dioxide, and boron nitride) were introduced in a carbon fiber-reinforced polymer matrix for the development of polymer composite materials (PCM). Tensile and compressive testing were carried out, and the hardness of created PCM was evaluated.

Role of Lipids of the Evergreen Shrub in Adaptation to Low Temperature in the Cryolithozone.

Lipids are the fundamental components of cell membranes and they play a significant role in their integrity and fluidity. The alteration in lipid composition of membranes has been reported to be a major response to abiotic environmental stresses. Seasonal dynamics of membrane lipids in the shoots of J.

Modeling historical budget for β-Hexachlorocyclohexane (HCH) in the Arctic Ocean: A contrast to α-HCH.

The historical annual loading to, removal from, and cumulative burden in the Arctic Ocean for β-hexachlorocyclohexane (β-HCH), an isomer comprising 5-12% of technical HCH, is investigated using a mass balance box model from 1945 to 2020. Over the 76 years, loading occurred predominantly through ocean currents and river inflow (83%) and only a small portion via atmospheric transport (16%). β-HCH started to accumulate in the Arctic Ocean in the late 1940s, reached a peak of 810 t in 1986, and decreased to 87 t in 2020, when its concentrations in the Arctic water and air were ∼30 ng m and ∼0.

Electrophysiological signatures of resting state networks under new environmental conditions.

It is assumed that cognitive processes are provided by the regulatory interactions of different brain networks. The three most stable resting state networks, among which the default mode network (DMN), the central executive network (CEN) and the salience network (SN) are considered to be the key neurocognitive networks for understanding higher cognitive functions. Peculiarities of changes in the connectivity of resting state networks of an individual entering a new environment and after a year of adaptation in this environment remain poorly studied.

Optical Properties of Tricarboxylic Acid-Derived Carbon Dots.

Herein, we report the characterization of two types of luminescent carbon dots (CDs) synthesized by the hydrothermal treatment of citric acid and -aconitic acid by using ammonia solution as a nitrogen dopant. The lateral size range of nanoparticles for CDs lies in the range of 3-15 nm. The intense blue photoluminescence (PL) was emitted by the CDs at around 409-435 nm under the excitation of 320 nm.

Analysis of SLC26A4, FOXI1, and KCNJ10 Gene Variants in Patients with Incomplete Partition of the Cochlea and Enlarged Vestibular Aqueduct (EVA) Anomalies.

Pathogenic variants in the SLC26A4, FOXI1, and KCNJ10 genes are associated with hearing loss (HL) and specific inner ear abnormalities (DFNB4). In the present study, phenotype analyses, including clinical data collection, computed tomography (CT), and audiometric examination, were performed on deaf individuals from the Sakha Republic of Russia (Eastern Siberia). In cases with cochleovestibular malformations, molecular genetic analysis of the coding regions of the SLC26A4, FOXI1, and KCNJ10 genes associated with DFNB4 was completed.

Plant Pyranocoumarins: Description, Biosynthesis, Application.

This overview article contains information about pyranocoumarins over the last 55 years. The article is based on the authors' phytochemical and physiological studies in vivo and in vitro as well as search and analysis of data in literature available on Google Scholar, Web of Science, PubMed, and ScienceDirect before January 2022. Pyranocoumarins are synthesized in plants of the , families, and one species in each of the , , and families can synthesize this class of compounds.

Morphology Analysis of Friction Surfaces of Composites Based on PTFE and Layered Silicates.

In the present study, the tribological behavior of polytetrafluoroethylene (PTFE) composites filled with natural layered silicates (LS) was investigated. The change in the morphology of the friction surface of composites depending on the content and chemical composition of layered silicates has been shown. The friction surface of PTFE composites with layered silicates was investigated by scanning electron microscopy (SEM).

Ancient DNA Reveals Maternal Philopatry of the Northeast Eurasian Brown Bear () Population during the Holocene.

Significant palaeoecological and paleoclimatic changes that took place during Late Pleistocene-Early Holocene transition are considered important factors that led to megafauna extinctions. Unlike many other species, the brown bear () has survived this geological time. Despite the fact that several mitochondrial DNA clades of brown bears became extinct at the end of the Pleistocene, this species is still widely distributed in Northeast Eurasia.

A combined microbial and biogeochemical dataset from high-latitude ecosystems with respect to methane cycle.

High latitudes are experiencing intense ecosystem changes with climate warming. The underlying methane (CH) cycling dynamics remain unresolved, despite its crucial climatic feedback. Atmospheric CH emissions are heterogeneous, resulting from local geochemical drivers, global climatic factors, and microbial production/consumption balance.

Supersonic Motion of Atoms in an Octahedral Channel of fcc Copper.

In this work, the mass transfer along an octahedral channel in an fcc copper single crystal is studied for the first time using the method of molecular dynamics. It is found that the initial position of the bombarding atom, outside or inside the crystal, does not noticeably affect the dynamics of its motion. The higher the initial velocity of the bombarding atom, the deeper its penetration into the material.

[Efficacy and safety of bulevirtide in patients with chronic hepatitis D and compensated cirrhosis].

Aim: To study the efficacy and safety of bulevirtide, the HBV and HDV entry inhibitor.

Materials And Methods: Analysis of the results of using bulevirtide in randomized controlled open-label comparative studies MYR202 and MYR203 in 56 patients with chronic hepatitis D and compensated cirrhosis, in monotherapy and combination with pegylated interferon alpha-2a (PEG-IFN).

Results: Monotherapy with bulevirtide for 24 weeks in the MYR202 study in 46 patients with compensated liver cirrhosis demonstrated: 1) a high rate of virological (100%) and biochemical response (alanine aminotransferase normalization rate 45.

[Dental caries among 5-7 years old children in a Russian subarctic region].

Objective: The aim of the study is to assess the prevalence and experience of dental caries among preschool children in a Russian Subarctic region.

Material And Methods: We performed a complex dental examination in 1232 randomly selected children aged 5-7 years using the World Health Organization (WHO) methodology (5th Edition) and standard dental forms.

Results: The overall prevalence of caries in primary dentition was 90.

Interactions between climate and COVID-19.

In this Personal View, we explain the ways that climatic risks affect the transmission, perception, response, and lived experience of COVID-19. First, temperature, wind, and humidity influence the transmission of COVID-19 in ways not fully understood, although non-climatic factors appear more important than climatic factors in explaining disease transmission. Second, climatic extremes coinciding with COVID-19 have affected disease exposure, increased susceptibility of people to COVID-19, compromised emergency responses, and reduced health system resilience to multiple stresses.

Equilibrium problem for a thermoelastic Kirchhoff-Love plate with a delaminated flat rigid inclusion.

A new mathematical model describing an equilibrium of a thermoelastic heterogeneous Kirchhoff-Love plate is considered. A corresponding nonlinear variational problem is formulated with respect to a two-dimensional domain with a cut. This cut corresponds to an interfacial crack located on a given part of the boundary of a flat rigid inclusion.

COVID-19 Vaccinating Russian Medical Students-Challenges and Solutions: A Cross-Sectional Study.

: The role of preventive measures increases significantly in the absence of effective specific COVID-19 treatment. Mass population immunization and the achievement of collective immunity are of particular importance. The future development of public attitudes towards SARS-CoV-2 immunization depends significantly on medical students, as future physicians.

Polymeric Compounds of Lingonberry Waste: Characterization of Antioxidant and Hypolipidemic Polysaccharides and Polyphenol-Polysaccharide Conjugates from Press Cake.

Lingonberry ( L.) fruits are important Ericaceous berries to include in a healthy diet of the Northern Hemisphere as a source of bioactive phenolics. The waste generated by the processing industry is hard-skinned press cake that can be a potential source of dietary fiber and has not been studied thus far.

Relationships between Uncoupling Protein Genes , and and Irisin Levels in Residents of the Coldest Region of Siberia.

Currently, it is known that irisin can participate in the processes of thermoregulation and browning of adipose tissue, and, therefore, it is possible that it is involved in the microevolutionary mechanisms of adaptation to a cold. The aim of this study is to investigate the relationship between the uncoupling protein genes (, and the irisin levels in the residents of the coldest region of Siberia. The sample consisted of 279 Yakut people (185 females, 94 males, average age 19.