Objectives: To report two novel DNA2 gene mutations causing early onset myopathy with cardiac involvement and late onset mitochondriopathy with rhabdomyolysis.Methods: We performed detailed clinical, muscle histopathology and molecular studies including mitochondrial gene NGS analysis in two patients (Patient 1 and 2), a mother and her son, belonging to a Mexican family, and a third sporadic French patient.Results: Patient 1 and 2 presented with an early onset myopathy associated with ptosis, velopharyngeal weakness, and cardiac involvement. Patient 3 presented rhabdomyolysis unmasking a mitochondrial disease characterized by a sensorineural hearing loss, ptosis, and lipomas. Muscle biopsies performed in all patients showed variable mitochondrial alterations. Patient 3 had multiple mtDNA deletion in his muscle. Genetic studies revealed a novel heterozygous frameshift mutation in DNA2 gene (c.2346delT p.Phe782Leufs*3) in P1 and P2, and a novel heterozygous missense mutation in DNA2 gene (c.578T>C p.Leu193Ser) in the P3.Conclusions: To date only few AD cases presenting either missense or truncating DNA2 variants have been reported. None of them presented with a cardiac involvement or rhabdomyolysis. Here we enlarge the genetic and phenotypic spectrum of DNA2-related mitochondrial disorders.

The aim of the work is to conduct a comparative analysis of the national goals and objectives in the national project “Ecology” and the global SDGs. This article considers he main vectors of environmental discourse, their compliance with the international agenda and relevance for various regions of RussiaThe work presents the course results designed according to the classical model ADDIE. An educational strategy and a public environmental information model have been established.. Small groups of students (12) were set up to work on researching selected federal projects from the national project. The research was conducted according to the unified plan and was designed with different content for each group of students, which allowed a comprehensive evaluation of the “Ecology” project. Each group of researchers analyzed the main goals and objectives of the federal projects, conducted a comparative analysis between the global SDGs and features of the national plan to achieve them. The analysis showed that the goals should be adjusted as they are achieved. The technique tested allows to implement a widespread application in an environmental education and investigate the large management models in the natural resource management.

Воскресенская Н.А., Казакова Е.В.Национальный исследовательский Нижегородский государственный университет им. Н.И. Лобачевского 603950, г. Нижний Новгород, пр. Гагарина, 23, Российская Федерация. Аннотация. В статье рассматриваются проблемы перевода в художественных текстах имён собственных на материале французских переводов цикла И.С. Тургенева «Записки охотника» XIX-XX вв. Сравнительно-сопоставительный анализ текстов показал, что суть переводческой проблемы сводится к сохранению смысла внутренней формы оригиналь-ного имени собственного при переводе. Передача имён текста И. Тургенева на француз-ский язык осложняется тем, что имена в цикле «Записки охотника» являются важной составляющей в авторской системе представления русского национального характера.Ключевые слова: имена собственные, переводческая рецепция, французский, художе-ственный текст, Тургенев, «Записки охотника». PECULIARITIES OF THE TRANSLATION OF PROPER NAMES IN A LITERARY TEXT (ON THE MATERIAL OF FRENCH TRANSLATIONS OF "THE HUNTING SKETCHES" BY I. TURGENEV) N. Voskresenskaya, E. Kazakova Lobachevsky State University of Nizhni Novgorod 23, Gagarina ave., Nizhni Novgorod, 603950, Russian FederationAbstract. The article presents problems of translating proper names in literary texts on the material of French translations of I. Turgenev's cycle "The Hunting Sketches" in 19-20 th centuries. The comparative-contrastive analysis of texts shows that the essence of a translation problem comes down to maintaining the meaning of the internal form of an original proper name when translating. The transfer of names in I. Turgenev's texts into French is complicated by the fact that names in the cycle "The Hunting Sketches" are an important component in the author's system of representation of the Russian national character.

The study assesses the effect of low-frequency ultrasonic pre-treatment of slightly saturated 3% brine on colour attributes of chilled pork after 24 h salting. The results obtained are used to formulate recommendations to stabilise surface and intramuscular colouration in normally autolysed, PSE- and DFD-defective pork by cavitational disintegration of liquid sodium chloride-based salting media. Cavitational pre-activation of 3% brine using a submersible low-frequency sonication technique is recommended as a method to optimise colour attributes in normally autolysed pork and meat with inner and surface DFD defects. Prior cavitational activation of 3% brine was shown undesirable with PSE meat due to a reduced steadiness of the component a* (degree of redness) in the CIELab colour system.

In this paper, we present a methodology for integrated assessment of food quality as affected by various processes. In particular, the method of high-frequency acoustic cavitation, which is used for making Circassian cheese, is considered. The following measurement techniques were used: sample deformation profile test, relaxation depth test by penetrating to 36 mm with a cylinder, cheese hardness when penetrated with a ∅2 mm cylinder, and cutting force. An integrated metric for assessing food quality is proposed, which describes its rheological properties (the depth of relaxation). In addition, a diagram of the structural and mechanical classes of food, which combines multiple organoleptic and rheological food properties, is proposed. This diagram can be used for estimating the effect of a specific treatment process on food production.