Social Portrait of a Foreign Language Teacher at the Turn of XIX-XX Centuries

A social portrait of a gymnasium teacher of ancient and new languages at the turn of the XIX-XX centuries is presented. The mission of the teacher, his study load, the ability to combine the teaching of several subjects of the gymnasium course are shown. Information about the features of work in the position of classes mentors is presented. The forms of work that were paid extra, as well as those for which teachers did not receive any payment, were highlighted. Information is provided on the number of lessons per week and the amount paid. The financial situation of the teacher, in particular, the cost of rent, nutrition, spiritual development, is characterized. The requirements of teachers on increasing salaries and reducing the workload are given. The appeal of the “Society for the mutual assistance of pedagogical rank” Board to teachers of the country with a request to intensify the work in educational institutions to raise funds to help teachers in need is analyzed. It is concluded that the volume of the academic load and material reward did not correspond to the high sociocultural mission that was assigned to the teachers, but during this historical period, the teacher was the standard of education, culture, unquestioned authority, for which he earned respect from students, their parents and public

МОЛЕКУЛЯРНАЯ СТРУКТУРА БОКОВОГО АМИОТРОФИЧЕСКОГО СКЛЕРОЗА В РОССИЙСКОЙ ПОПУЛЯЦИИ

Materials and methods. 285 Russian patients with amyotrophic lateral sclerosis (ALS) including 260 patients with a sporadic form and 25 with a familial form were examined for mutations in SOD1, C9orf72, TARDBP,  ANG and other genes and the presence of associations among polymorphic sites in ATXN2 (polyCAG) and VEGF (-2578С/А) genes.Molecular genetic analysis was performed using direct sequencing, fragment analysis and real-time polymerase chain reaction. On the last stage, rare ALS candidate genes were evaluated using a next generation sequencing (NGS) panel.Results. Total rate of the identified mutations in the examined ALS cohort was 9.5 %. The most frequently observed defects were mutations in the SOD1 (24.0 % in familial ALS and 4.6 % in sporadic ALS) and C9orf72 (pathological hexanucleotide repeat expansion was identified in 1.8 % cases of ALS, all sporadic) genes. The TARDBP gene didn’t contain any mutations, though in the ALS group deletion c.715-126delG located in intron 5 of the TARDBP gene was significantly over-represented – 38.0 % vs. 26.6 % (χ2 = 13.17; р = 0.002). Mutations in the ANG gene were identified in 1.05 % of ALS patients (all cases were sporadic). In 1 (0.35 %) sporadic case a G1082A mutation in the DCTN1 gene was identified. The examined group significantly more frequently carried a risk allele of the ATXN2 gene with an “intermediate” (28–33)  number of CAG repeats – 5.0 % vs. 1.7 % in the control group (χ2 = 3.89; р = 0.0486). In Russian ALS patients, an association between the disease and the presence of a risk А-allele and homozygote genotype А/А of -2578С/А polymorphism in the VEGF gene was identified (χ2 = 7.14; р = 0.008 and χ2 = 13.46; р = 0.001 for the rates in the ALS population and in the control population, respectively), which is confirmed by the odds ratio.Conclusion. In the current article, molecular structure of ALS in the Russian population was examined, rates of individual genetic forms and mutation spectrum were established. This work is of considerable significance for medical genetic counseling and prevention of the disease in the affected families.Материалы и методы. Были обследованы 285 российских пациентов с боковым амиотрофическим склерозом (БАС), включая 260 пациентов со спорадической и 25 с семейной формой, на предмет носительства мутаций в генах SOD1, C9orf72, TARDBP, ANG и др., а также на наличие ассоциаций с полиморфными сайтами в генах ATXN2 (полиCAG) и VEGF (-2578С/А).Молекулярно-генетический анализ выполняли с использованием методов прямого секвенирования, фрагментного анализа и полимеразной цепной реакции в режиме реального времени. На последнем этапе оценивали редкие кандидатные гены БАС с использованием секвенирующей панели нового поколения (nextgenerationsequencing, NGS).Результаты. Суммарная частота выявленных мутаций в обследованной когорте пациентов с БАС составила 9,5 %. Наиболее частыми оказались повреждения в генах SOD1 (24,0 % при семейной форме БАС и 4,6 % при спорадической) и C9orf72 (патологическая экспансия гексануклеотидных повторов в нем обнаружена в 1,8 % случаев БАС, все случаи спорадические). Мутаций в гене TARDBP не обнаружено, однако в группе БАС значимо чаще по сравнению с контролем встречалась делеция c.715-126delG, локализованная в интроне 5 гена TARDBP, – 38,0 % против 26,6 % (χ2 = 13,17; р = 0,002). Мутации в гене ANG выявлены у 1,05 % обследованных больных БАС (все случаи спорадические). В 1 (0,35 %) спорадическом случае выявлена мутация G1082A в гене DCTN1. В обследованной группе значимо чаще по сравнению с контролем встречается носительство рискового аллеля гена ATXN2 с «промежуточным» (28–33) числом копий CAG-повторов – 5,0 % против 1,7 % (χ2 = 3,89; р = 0,0486). У российских пациентов с БАС выявлена ассоциация болезни с носительством рискового А-аллеля и гомозиготного генотипа А/А по полиморфизму -2578С/А в гене VEGF (соответственно χ2 = 7,14; р = 0,008 и χ2 = 13,46; р = 0,001 при сравнении частот у больных БАС и в контроле), что подтверждается анализом отношения шансов. Заключение. В работе раскрыта молекулярная структура БАС в российской популяции, установлены частота отдельных генетических форм и спектр мутаций, что имеет большое значение для медико-генетического консультирования и профилактики заболевания в отягощенных семьях

Оценка метилирования 5’-промоторной области гена C9orf72 у российских пациентов с нейродегенеративными заболеваниями

Background. Hexanucleotide repeat expansion in the C9orf72 gene is the most significant cause of a large number of neurodegenerative diseases: frontotemporal degeneration (FTD), amyotrophic lateral sclerosis (ALS), Parkinson’s disease (PD), etc. Several studies have shown the relationship with the neurodegenerative process for full (>40 GGGGCC copies) and intermediate (13–20) repeats expansion. Methylation of the C9orf72 gene can play an important role in the pathogenesis of FTD and ALS, but the mechanism has not been sufficiently studied.The objective is to investigate the status of methylation of the 5’-promotor region of the C9orf72 gene in patients with neurodegenerative disorders having full or intermediate expansion of GGGGCC-repeats.Materials and methods. We investigated the methylation status of the 5’-promoter region of full C9orf72 expansions in FTD/ALS patients (n = 12), of intermediate expansions in Parkinson’s disease patients (n = 8) and of non-expanded alleles in healthy controls (n = 8). Methylation status was determined via sequencing of amplified fragments of bisulfite-converted DNA.Results. We identified two cases (sibling) with the hypermethylation of the 5’-promoter region in the full C9orf72 expansions group. Patient A. (65 years old, male) had an atypical ALS presentation: an onset with head tremor, a long duration of ALS symptoms (9 years at this time), and cognitive impairments with a temporal lobes atrophy. The patient’s sister had a similar clinical phenotype. There were no cases of the promoter hypermethylation in the intermediate and control groups.Conclusion. This is the first data on the 5’-promoter region C9orf72 gene methylation in Russian population. The frequency of the promoter methylation in this group was 9.1 % that consistent with previous studies in other populations. Atypical clinical presentation may indicate a modifying effect of methylation in this area on the ALS phenotype.Введение. Гексануклеотидная экспансия в гене C9orf72 является одной из наиболее значимых причин большого числа нейродегенеративных заболеваний: лобно-височной деменции (ЛВД), бокового амиотрофического склероза (БАС), болезни Паркинсона и др. При этом показана взаимосвязь с нейродегенеративным процессом как для полной экспансии (число GGGGCC-повторов >40), так и для промежуточной (13–20 повторов). В патогенезе ЛВД и БАС большую роль может играть метилирование гена C9orf72, механизм которого на сегодня недостаточно изучен.Цель исследования – изучить статус метилирования 5’-промоторной области гена C9orf72 у пациентов с нейродегенеративными заболеваниями, имеющих полную или промежуточную экспансию GGGGCC-повторов.Материалы и методы. Мы исследовали метилирование 5’-промоторной области гена C9orf72 в группе пациентов с полной экспансией и фенотипом БАС и/или ЛВД (n = 12), с промежуточной экспансией и фенотипом болезни Паркинсона (n = 8) и в контрольной группе (n = 8). Уровень метилирования определяли методом капиллярного секвенирования амплифицированных фрагментов, прошедших бисульфитную обработку.Результаты. Выявлены 2 случая метилирования 5’-промоторной области гена C9orf72 у сибсов в группе пациентов с полной экспансией. Пациент А. (мужчина), 65 лет, имел атипичную клиническую картину БАС: манифестация с дрожания головы, длительное течение заболевания (на данный момент болен уже 9 лет), когнитивные нарушения, гипотрофия височных долей мозга по данным магнитно-резонансной томографии. У сестры пациента была сходная клиническая симптоматика. Ни одного случая метилирования 5’-промоторной области гена C9orf72 в группе с промежуточной экспансией и контрольной группе не обнаружено. Заключение. Это первые данные о метилировании 5’-промоторной области гена C9orf72, полученные для российской популяции. Частота встречаемости в нашей выборке составила 9,1 %, что соответствует результатам, полученным на других популяциях. Нетипичная клиническая картина у выявленных пациентов может свидетельствовать о модифицирующем влиянии метилирования данной области на фенотип БАС

Culturological Ideas of Teaching a Foreign Language: Concepts and Theories (the End of 20th – at the Beginning of the 21st Century)

The rapidly expanding International cooperation in the late 20th - early 21st centuries raised the question of foreign language learning as well as cultural understanding. Without these essential aspects, intercultural communication cannot be efficient. The high interest in foreign language learning contributed to the search for the most effective ways of teaching it.  The concepts and theories appeared at the turn of two centuries in Russia, remaining relevant today. They became the essential basis for further research on foreign language teaching in the framework of the dialogue of cultures. The cultural idea is focused on learning both the Language and culture. Safonova (1993), developed a socio-cultural approach to teaching a foreign language as a specialty. She has proved the necessity of integration of the culture of the country being studied into the educational process. ? methodical model of foreign language teaching was proposed as a basis for developing the bicultural personality of students

Evaluation of methylation status of the 5’-promoter region of C9orf72 gene in Russian patients with neurodegenerative diseases

Background. Hexanucleotide repeat expansion in the C9orf72 gene is the most significant cause of a large number of neurodegenerative diseases: frontotemporal degeneration (FTD), amyotrophic lateral sclerosis (ALS), Parkinson’s disease (PD), etc. Several studies have shown the relationship with the neurodegenerative process for full (>40 GGGGCC copies) and intermediate (13–20) repeats expansion. Methylation of the C9orf72 gene can play an important role in the pathogenesis of FTD and ALS, but the mechanism has not been sufficiently studied.The objective is to investigate the status of methylation of the 5’-promotor region of the C9orf72 gene in patients with neurodegenerative disorders having full or intermediate expansion of GGGGCC-repeats.Materials and methods. We investigated the methylation status of the 5’-promoter region of full C9orf72 expansions in FTD/ALS patients (n = 12), of intermediate expansions in Parkinson’s disease patients (n = 8) and of non-expanded alleles in healthy controls (n = 8). Methylation status was determined via sequencing of amplified fragments of bisulfite-converted DNA.Results. We identified two cases (sibling) with the hypermethylation of the 5’-promoter region in the full C9orf72 expansions group. Patient A. (65 years old, male) had an atypical ALS presentation: an onset with head tremor, a long duration of ALS symptoms (9 years at this time), and cognitive impairments with a temporal lobes atrophy. The patient’s sister had a similar clinical phenotype. There were no cases of the promoter hypermethylation in the intermediate and control groups.Conclusion. This is the first data on the 5’-promoter region C9orf72 gene methylation in Russian population. The frequency of the promoter methylation in this group was 9.1 % that consistent with previous studies in other populations. Atypical clinical presentation may indicate a modifying effect of methylation in this area on the ALS phenotype