The learners' electronic portfolio system (E-Portfolio) as an element of the information enviroment for managing the educational process

This article is devoted to the methodological and practical approaches to developing and implementing the electronic portfolios for learners as part of the electronic educational environment for managing the learning process. Based on the modern model of education in the pedagogical university, as well as on the federal state standard, the main sections of the system of the students' electronic portfolio have been formulated, which describe the main educational, scientific and other achievements of the students. In addition, the functional capabilities of the software implementation of an example of the electronic portfolio, software architecture and the methodological approaches to working with the system have been substantiated. The application of the students' electronic portfolios allows us to quickly obtain the information about the status of the main parameters of student activities and to stimulate them in different areas of the activities

Платежи за загрязнение окружающей среды и их роль в стимулировании рационального природопользования

Показана актуальность формирования эффективного механизма взаимодействия предприятий и органов власти в решении проблем охраны окружающей среды. Анализ способов использования поступающих в бюджет экологических платежей позволил выявить его особенности, существенные недостатки и сделать вывод о необходимости проведения изменений в системе рационального природопользования. Выделена и проанализирована одна из функций экономического механизма, которая носит стимулирующий характер и направлена на обеспечения заинтересованности как у предприятий, несущих негативное воздействие окружающей среде, так и у экологического законодательства. Определены место и роль экологических платежей в системе экономических возможностей по стимулированию рационального природопользования. Экологические платежи рассмотрены как инструмент повышения эффективности природопользования. Обоснована необходимость совершенствования этого инструмента посредством выработки ряда экономических и организационных мер, при помощи которых можно будет достичь конкретных результатов в решении проблемы природопользования

Специфика русской свадебной приметы на фоне вьетнамской лингвокультуры

Статья посвящена выявлению лингвокультурной специфики русской и вьетнамской свадебной приметы. Данная проблема проецируется в область лексической семантики, лингвокультурологии, без которых она не может быть решена; поэтому в работе обоснованно ставятся такие важные вопросы, как вопрос о своеобразии традиционной культуры, ее символическом метаязыке и средствах языковой материализации, о содержательном наполнении и способах объективации традиционной приметы. Общая цель работы - изучение способов, которыми язык символически объективирует традиционную культуру и в конечном итоге моделирует национальный образ мира. Результатом исследования стало выявление основных формально-семантических характеристик русской и вьетнамской свадебной приметы, описание различий в языковом картировании мира

MIPSPlantsDB—plant database resource for integrative and comparative plant genome research

Genome-oriented plant research delivers rapidly increasing amount of plant genome data. Comprehensive and structured information resources are required to structure and communicate genome and associated analytical data for model organisms as well as for crops. The increase in available plant genomic data enables powerful comparative analysis and integrative approaches. PlantsDB aims to provide data and information resources for individual plant species and in addition to build a platform for integrative and comparative plant genome research. PlantsDB is constituted from genome databases for Arabidopsis, Medicago, Lotus, rice, maize and tomato. Complementary data resources for cis elements, repetive elements and extensive cross-species comparisons are implemented. The PlantsDB portal can be reached at

Разработка ПГУ-ТЭЦ на базе ГТУ SGT5-4000F

Целью работы является разработка парогазового энергоблока ТЭЦ на базе ГТУ. Проводится анализ режимов работы теплофикационных турбин в составе ПГУ, разрабатывается математическая модель поверочного расчета котла-утилизатора, проводится обоснование расчетного режима проектирования КУ, выполняется расчет режимов работы и годовых показателей ПГУ-ТЭЦ.The aim of the work is to develop a combined cycle power plant based on the SGT5-4000F GTU. The analysis of the operating modes of cogeneration turbines as part of a combined cycle power plant

Cardiometabolic risk factor clustering in patients with deficient branched‐chain amino acid catabolism: A case‐control study

AbstractClassical organic acidemias (OAs) result from defective mitochondrial catabolism of branched‐chain amino acids (BCAAs). Abnormal mitochondrial function relates to oxidative stress, ectopic lipids and insulin resistance (IR). We investigated whether genetically impaired function of mitochondrial BCAA catabolism associates with cardiometabolic risk factors, altered liver and muscle energy metabolism, and IR. In this case‐control study, 31 children and young adults with propionic acidemia (PA), methylmalonic acidemia (MMA) or isovaleric acidemia (IVA) were compared with 30 healthy young humans using comprehensive metabolic phenotyping including in vivo 31P/1H magnetic resonance spectroscopy of liver and skeletal muscle. Among all OAs, patients with PA exhibited abdominal adiposity, IR, fasting hyperglycaemia and hypertriglyceridemia as well as increased liver fat accumulation, despite dietary energy intake within recommendations for age and sex. In contrast, patients with MMA more frequently featured higher energy intake than recommended and had a different phenotype including hepatomegaly and mildly lower skeletal muscle ATP content. In skeletal muscle of patients with PA, slightly lower inorganic phosphate levels were found. However, hepatic ATP and inorganic phosphate concentrations were not different between all OA patients and controls. In patients with IVA, no abnormalities were detected. Impaired BCAA catabolism in PA, but not in MMA or IVA, was associated with a previously unrecognised, metabolic syndrome‐like phenotype with abdominal adiposity potentially resulting from ectopic lipid storage. These findings suggest the need for early cardiometabolic risk factor screening in PA

Defining the genotypic and phenotypic spectrum of X-linked MSL3-related disorder

PURPOSE: We sought to delineate the genotypic and phenotypic spectrum of female and male individuals with X-linked, MSL3-related disorder (Basilicata-Akhtar syndrome). METHODS: Twenty-five individuals (15 males, 10 females) with causative variants in MSL3 were ascertained through exome or genome sequencing at ten different sequencing centers. RESULTS: We identified multiple variant types in MSL3 (ten nonsense, six frameshift, four splice site, three missense, one in-frame-deletion, one multi-exon deletion), most proven to be de novo, and clustering in the terminal eight exons suggesting that truncating variants in the first five exons might be compensated by an alternative MSL3 transcript. Three-dimensional modeling of missense and splice variants indicated that these have a deleterious effect. The main clinical findings comprised developmental delay and intellectual disability ranging from mild to severe. Autism spectrum disorder, muscle tone abnormalities, and macrocephaly were common as well as hearing impairment and gastrointestinal problems. Hypoplasia of the cerebellar vermis emerged as a consistent magnetic resonance image (MRI) finding. Females and males were equally affected. Using facial analysis technology, a recognizable facial gestalt was determined. CONCLUSION: Our aggregated data illustrate the genotypic and phenotypic spectrum of X-linked, MSL3-related disorder (Basilicata-Akhtar syndrome). Our cohort improves the understanding of disease related morbidity and allows us to propose detailed surveillance guidelines for affected individuals

Loss-of-function mutations in UDP-Glucose 6-Dehydrogenase cause recessive developmental epileptic encephalopathy

AbstractDevelopmental epileptic encephalopathies are devastating disorders characterized by intractable epileptic seizures and developmental delay. Here, we report an allelic series of germline recessive mutations in UGDH in 36 cases from 25 families presenting with epileptic encephalopathy with developmental delay and hypotonia. UGDH encodes an oxidoreductase that converts UDP-glucose to UDP-glucuronic acid, a key component of specific proteoglycans and glycolipids. Consistent with being loss-of-function alleles, we show using patients’ primary fibroblasts and biochemical assays, that these mutations either impair UGDH stability, oligomerization, or enzymatic activity. In vitro, patient-derived cerebral organoids are smaller with a reduced number of proliferating neuronal progenitors while mutant ugdh zebrafish do not phenocopy the human disease. Our study defines UGDH as a key player for the production of extracellular matrix components that are essential for human brain development. Based on the incidence of variants observed, UGDH mutations are likely to be a frequent cause of recessive epileptic encephalopathy.</jats:p