Developmental hypomyelination in Wolfram syndrome: New insights from neuroimaging and gene expression analyses

Wolfram syndrome is a rare multisystem disorder caused by mutations in WFS1 or CISD2 genes leading to brain structural abnormalities and neurological symptoms. These abnormalities appear in early stages of the disease. The pathogenesis of Wolfram syndrome involves abnormalities in the endoplasmic reticulum (ER) and mitochondrial dynamics, which are common features in several other neurodegenerative disorders. Mutations in WFS1 are responsible for the majority of Wolfram syndrome cases. WFS1 encodes for an endoplasmic reticulum (ER) protein, wolframin. It is proposed that wolframin deficiency triggers the unfolded protein response (UPR) pathway resulting in an increased ER stress-mediated neuronal loss. Recent neuroimaging studies showed marked alteration in early brain development, primarily characterized by abnormal white matter myelination. Interestingly, ER stress and the UPR pathway are implicated in the pathogenesis of some inherited myelin disorders like Pelizaeus-Merzbacher disease, and Vanishing White Matter disease. In addition, exploratory gene-expression network-based analyses suggest that WFS1 expression occurs preferentially in oligodendrocytes during early brain development. Therefore, we propose that Wolfram syndrome could belong to a category of neurodevelopmental disorders characterized by ER stress-mediated myelination impairment. Further studies of myelination and oligodendrocyte function in Wolfram syndrome could provide new insights into the underlying mechanisms of the Wolfram syndrome-associated brain changes and identify potential connections between neurodevelopmental disorders and neurodegeneration

Comparison of the Land Uses and Sustainable Development in Small Islands: The Case of Skiathos Island, Greece

The Island of Skiathos occupies a total area of 50 km2, accounting for 1.6% of the area of the prefecture of Magnesia and 0.28% of the Region of Thessaly, Greece. The land is hilly and can be divided into farmland, meadows, woodlands, land covered by water and land occupied by settlements and roads. Also, a large part is occupied by burnt areas that resulted from the fire of 2007. The aim of this chapter is to present the evolution of existing land uses at the Island of Skiathos during the past decades. With the contribution of Geographic Information Systems (GIS) and the orthophotomaps, the spatial planning of the land uses can be evaluated for all these years and the total area can also be calculated. Our results are important for understanding the impacts of land uses on ecosystems in the frame of sustainable development. There has been no other research regarding land uses in Skiathos Island in the past, and, also, this is the first digitization of the area. Finally, two sustainable spatial development scenarios for the Island of Skiathos are proposed. The first scenario relates to the results obtained from a prediction (application of the model of automatic cellular) while the second scenario refers to a more realistic model of development with focus on environmental protection and sustainable development

Neuroinflammation and white matter alterations in obesity assessed by Diffusion Basis Spectrum Imaging

Human obesity is associated with low-grade chronic systemic inflammation, alterations in brain structure and function, and cognitive impairment. Rodent models of obesity show that high-calorie diets cause brain inflammation (neuroinflammation) in multiple regions, including the hippocampus, and impairments in hippocampal-dependent memory tasks. To determine if similar effects exist in humans with obesity, we applied Diffusion Basis Spectrum Imaging (DBSI) to evaluate neuroinflammation and axonal integrity. We examined diffusion-weighted magnetic resonance imaging (MRI) data in two independent cohorts of obese and non-obese individuals (Cohort 1: 25 obese/21 non-obese; Cohort 2: 18 obese/41 non-obese). We applied Tract-based Spatial Statistics (TBSS) to allow whole-brain white matter (WM) analyses and compare DBSI-derived isotropic and anisotropic diffusion measures between the obese and non-obese groups. In both cohorts, the obese group had significantly greater DBSI-derived restricted fraction (DBSI-RF; an indicator of neuroinflammation-related cellularity), and significantly lower DBSI-derived fiber fraction (DBSI-FF; an indicator of apparent axonal density) in several WM tracts (all correcte

MALETÍN CLAVE DEL PROFESOR Y LA EVALUACIÓN DE LAS COMPETENCIAS LINGÜÍSTICAS

The purpose of this study is to generate transformations in the learning assessment process applied by Spanish language teachers in secondary education, for the development of language skills in students. For this, a virtual briefcase was created andapplied, designed with interactive links where teachers have easy access to a study plan and useful strategies for this purpose. Framed in a qualitative approach with a procedure organized in three stages (diagnosis, collective construction and evaluation of changes. This was possible, through interviews, surveys, discussion group, training workshops and a focus group. Data analysis was carried out using validated techniques such as: Atlas. Ti, convergence and divergence matrix, class observation processing matrix, check rubrics, questionnaires in Google Forms, among others. The findings establish the need to optimize the teaching evaluation exercise mediated by as a result, it is evident that, when implementing the prototype created, called: The Teacher's Key Briefcase (MCP), the teachers reconsidered their evaluation methods, and included in these the linguistic competencies of the students. great value for its original, effective, creative, and motivating character for both teachers and students.El propósito de este estudio es generar transformaciones en el proceso de evaluación del aprendizaje que aplican los docentes de Lengua Castellana de la educación media, para el desarrollo de competencias lingüísticas en los estudiantes. Para esto, se creó y aplicó un maletín virtual, diseñado con enlaces interactivos donde los docentes tienen fácil acceso a un plan de estudios y estrategias útiles para tal fin. Enmarcado en un enfoque cualitativo con un procedimiento organizado en tres etapas (diagnóstica,construcción colectiva y evaluación de cambios. Esto fue posible, a través de entrevistas, encuestas, grupo de discusión, talleres de formación y un grupo focal. El análisis de los datos se realizó mediante técnicas validadas como: Atlas. Ti, matriz de convergencias ydivergencias, matriz de procesamiento de observación de clases, rúbricas de chequeo; cuestionarios en Google Forms, entre otros. Los hallazgos establecen la necesidad de optimizar el ejercicio evaluativo docente mediado por las TIC. Como resultado se evidencia que, al implementar el prototipo creado, denominado: Maletín Clave Del Profesor (MCP), los docentes replantearon sus métodos de evaluación, e incluyeron en estos las competencias lingüísticas de los estudiantes. Aportando así, una estrategia degran valor por su carácter original, eficaz, creativo y motivador tanto para maestros como para estudiantes

Psychological Aid to Victims of War and the Covid Pandemic - 19 in Ukraine

The article reflects theoretical, methodological and methodical bases of psychological aid to victims of unexpected crisis-military situations on the background of coronovirus pandemics spreading. The essence of psychological self-help as a factor of stress-resistance and internal psychological potential of resisting psychological traumas in the conditions of war and viral pandemics with the propositions of effective methodological means of its conducting is revealed. In providing psychological aid, the cognitive-behavioral approach was deemed effective as the basic one, and it was suggested that integrative psychotherapeutic and comprehensive psychological approaches to crisis counseling in conditions of military conflict be applied, depending on the specifics of survivors' experience of acute emotional reactions and the complexity of the course of posttraumatic stress disorders of resistance to the action of a psychotraumatic factor. Neuropsychological correction deserves special attention in providing psychological aid in conditions of military conflict and pandemics, which allows a combination of an integrated body-oriented psychological approach with neuro-psycho-correctional techniques and psychotherapeutic methods for effective restoration of the tone of the nervous system and stabilization of the psycho-emotional state. The main provisions of practical psychology that in conditions of war and pandemic COVID-19 the adaptive potential of psychological health of each person and psychological self-help is love, faith and hope. Psychological recommendations of educational character for the formation of basic skills to ensure psychological health in conditions of war and pandemic COVID-19 have been developed.</p

Psychological Counseling of Clients in Crisis Situations

The article expands scientific ideas about methods and techniques of dealing with crisis situations in different approaches to psychological counseling of clients who are experiencing current crisis realities in a complex social space. Different psychological approaches to the problem of the impact of psychological counseling on the crisis states of clients at the integrative and systemic levels are analyzed. Significant features of the approaches most often used in the practice of crisis psychological counseling –gestalt-therapeutic, cognitive-behavioral, and existential– are highlighted. Based on the generalization of the theoretical and methodological foundations of crisis counseling, the important role of subject-activity, professionally competent, personality-oriented approaches is highlighted. The importance of maintaining a person’s mental health in a crisis and providing him/her with psychological assistance in counseling and therapeutic practice is emphasized. A theoretical and methodological approach to the requirements for the criteria of consulting practice effectiveness is indicated. The structurization, conditions for organization and providing crisis counseling at different stages (areas) of work is described. The use of a neuropsycho-corrective approach in the practice of crisis counseling is recommended. The significance of the integrative psychological approach to counseling mentioned by foreign and domestic researchers on the basis of a holistic vision of the relationship between the client’s psychological resources and the requirements of the crisis situation is noted. It is emphasized that the management of crisis education should be handled by the personnel services of organizations of extreme profile.</em

Psychological Complications During Military Operations in Ukraine: Neurobiological Effects

The relevance to the investigation stems from the fact that during military operations in Ukraine there are psychological complications both among servicemen and the population. The most common type of psychological complications is fear as one of the most mysterious and contradictory emotions. Formed in the process of evolution as a signal and warning human reaction to danger, fear often misinforms people about the extent of external danger. Fear as a psychological complication is a powerful catalyst of human physical, spiritual strength and energy and, at the same time, it can paralyze his ability to withstand danger. It is reflected in the brain, the brain organ of the central nervous system, which regulates all the most important changes in the human body. In case of stress, it is the brain structures that react first and trigger a response, the purpose of which is adaptation and survival in difficult conditions. The brain detects a threat, recognizes the cause of psychological or physical stress. In response to the influence of a stressor protective mechanisms are triggered, changing the work of the nervous and endocrine systems. The article reveals the concept of horror and its psychotraumatic effect; the most important functions of fear as a psychological complication are defined; the neurobiological basis of stress as a psychological complication during war is investigated.</p

Psychological Correction of Individual Neurotic Problems

The article conducts a theoretical analysis of the features of the neurotic response to uncertain situations as an important component and trigger of individual problems. In this regard, the issue of psychological correction of individual neurotic problems in uncertain situations at the theoretical, methodological and practical levels has been studied in the article. The neurotic experience of psychotraumatic events is due to internal conflicts. Effective methods of corrective work with neurotic reactions as prevention of persistent neurotic states are indicated here too. The neuropsychological content of psychotherapeutic techniques that can prevent neurotic development of an individual is revealed. It is presented the model of psychological correction of individual neurotic problems, which is capable to carry out effective corrective influence of the personal sphere on emotional, cognitive, motivational and behavioral levels. Important principles and conditions are noted regarding the psychological correction of such neurotic nature. The neuropsychological content of the body-oriented model of psychological correction of an individual in combination with psychotherapeutic methods is considered in detail.</em

Russian Old Believers: Genetic Consequences of Their Persecution and Exile, as Shown by Mitochondrial DNA Evidence

In 1653, the Patriarch Nikon modified liturgical practices to bring the Russian Orthodox Church in line with those of the Eastern (Greek) Orthodox Church, from which it had split 200 years earlier. The Old Believers (staroveri) rejected these changes and continued to worship using the earlier practices. These actions resulted in their persecution by the Russian Orthodox Church, which forced them into exile across Siberia. Given their history, we investigate whether populations of Old Believers have diverged genetically from other Slavic populations as a result of their isolation. We also examine whether the three Old Believer populations analyzed in this study are part of a single gene pool (founder population) or are instead derived from heterogeneous sources. As part of this analysis, we survey the mitochondrial DNAs (mtDNAs) of 189 Russian Old Believer individuals from three populations in Siberia and 201 ethnic Russians from different parts of Siberia for phylogenetically informative mutations in the coding and noncoding regions. Our results indicate that the Old Believers have not significantly diverged genetically from other Slavic populations over the 200-300 years of their isolation in Siberia. However, they do show some unique patterns of mtDNA variation relative to other Slavic groups, such as a high frequency of subhaplogroup U4, a surprisingly low frequency of haplogroup H, and low frequencies of the rare East Eurasian subhaplogroup D5

Language learning, language use and the evolution of linguistic variation

Linguistic universals arise from the interaction between the processes of language learning and language use. A test case for the relationship between these factors is linguistic variation, which tends to be conditioned on linguistic or sociolinguistic criteria. How can we explain the scarcity of unpredictable variation in natural language, and to what extent is this property of language a straightforward reflection of biases in statistical learning? We review three strands of experimental work exploring these questions, and introduce a Bayesian model of the learning and transmission of linguistic variation along with a closely matched artificial language learning experiment with adult participants. Our results show that while the biases of language learners can potentially play a role in shaping linguistic systems, the relationship between biases of learners and the structure of languages is not straightforward. Weak biases can have strong effects on language structure as they accumulate over repeated transmission. But the opposite can also be true: strong biases can have weak or no effects. Furthermore, the use of language during interaction can reshape linguistic systems. Combining data and insights from studies of learning, transmission and use is therefore essential if we are to understand how biases in statistical learning interact with language transmission and language use to shape the structural properties of language