ПРЕЗУМПЦИИ И ФИКЦИИ КАК ЭЛЕМЕНТЫ ТЕКСТА НОРМАТИВНО-ПРАВОВОГО АКТА

В статье рассматриваются презумпции и фикции в аспекте их технико-юридического значения и особенности их закрепления в тексте нормативно-правового акта. На конкретных примерах показано, что они могут быть закреплены как прямым способом, так и косвенным, позволяющим выявить их только путем толкования текста. При этом отмечается, что косвенные презумпции необходимо трансформировать в прямые для их лучшего применения, а косвенные фикции не нуждаются в этом, так как трудностей в их выявлении не возникает, и такой способ закрепления является для них целесообразным. Фикции обязательно нужно отграничивать от негативных фиктивных явлений, так как они, как и презумпции, участвуют в образовании юридических терминов и, благодаря своим уникальным свойствам, оптимизируют не только правовое регулирование, но и правотворческую работу законодателя, способствуя лингвистической экономии, делая текст нормативно-правового акта емким, лаконичным и недвусмысленным, то есть способствуют соблюдению правил юридической техники, предъявляемых к нему. Презумпция в значении предположения, имеющего определенную степень вероятности, обусловленную логически и исторически, используется не только в праве, но и в других науках, а также в повседневной жизни, как и фикция в значении средства, с помощью которого заведомо ложное признается истинным с целью преодоления ситуации неопределенности и нормального развития общественных отношений. Таким образом, презумпции и фикции являются необходимыми элементами текста нормативно-правового акта, позволяющими донести его смысл до адресатов.

Monogenic diabetes associated with PAX4 gene mutations (MODY9): first description in Russia

Maturity-onset diabetes of the young (MODY) is a heterogeneous group of disorders characterised by autosomal dominant type of inheritance and caused by genetic defects leading to dysfunction of pancreatic beta-cells. To date, at least 13 subtypes of MODY have been described in the literature, the most frequent of which are MODY types 13. MODY2 and MODY3 are the most prevalent subtypes, and were previously described in our country, Russia. Several cases of rare MODY subtypes were subsequently described in the Russian literature. The current report is the first in the Russian literature to present clinical and molecular genetic characteristics of two cases of another rare MODY subtypeMODY9. This type of MODY is associated with mutations in the PAX4 gene, which encodes transcription factor PAX4, one of the factors essential for pancreatic beta-cell differentiation. Molecular genetic analysis was performed using next-generation sequencing, a new method recently applied to verify monogenic diseases and, in particular, MODY. This study reports a novel mutation in the PAX4 gene in MODY patients.

Comparison of some indicators of the thyroid gland functional activity in simultaneous testing of capillary and venous blood

Background . In clinical practice it is often impossible to draw blood from peripheral veins for laboratory testing. At the same time the problem of comparison of venous and capillary blood parameters is still insufficiently studied. Aims . To compare 5 laboratory indicators of the thyroid gland function – FT3 (free T3), FT4 (free T4), TgAb (thyroglobulin antibodies), TPOAb (thyroid peroxidase antibodies), and TSH – in order to assess deviations of these parameters in venous against capillary blood. Materials and methods . The study enrolled 22 clinically healthy patients of both sexes who had their venous and capillary blood samples simultaneously collected in compliance with pre-analytical rules. The tests were performed within three hours of sample collection on the analyzer Roche Cobas e601. Mean values were calculated for all parameters using a 95% confidence interval based on bootstrap. Differences between paired values of venous and capillary blood parameters were calculated and expressed in relative units (%). Wilcoxon test and correlation analysis were used to compare dependent samples. Results . A statistically significant increase in capillary against venous samples was shown for FT3 (mean deviation 3.11; p 0.75) with the same parameters of the venous blood: r = 0.971 for FT3; r = 0.993 for FT4; r = 0.958 for TgAb; r = 0.836 for TPOAb; r = 0.995 for TSH. Conclusions . Thus, FT3, FT4 and TSH can be determined in the capillary blood without affecting accuracy and precision of clinical evaluation, while for TgAb and TPOAb these deviations may be significant only when the results are in upper borderline values.

Directional formation of operational properties details in technological environments

HUMAN RIGHT TO LIFE AND ITS IMPLEMENTATION IN THE LABOUR SPHERE

The Insight Phenomenon and the Digital Visualization of the Human Brain Activity

Star formation in shells of colliding multi-SNe bubbles

It is believed that when bubbles formed by multiple supernovae explosions interact with one another, they stimulate star formation in overlapping shells. We consider the evolution of a shocked layer formed by the collision of two identical bubbles each of which originated from OB clusters of ∼ 50 members and ∼ 50 pc. The clusters are separated by 200-400 pc.We found that depending on evolutionary status of colliding bubbles the shocked layer can either be destroyed into diffuse lumps, or be fragmented into dense clumps: the former occurs in collisions of young bubbles with continuing supernovae explosions, and the latter occurs in older bubble interactions.We argue that fragmentation efficiency in shells depends on external heating: for a heating rate <∼ 1.7×10 −24 erg s −1 the number of fragments formed in a collision of two old bubbles reaches several tens at t ∼ 4 Myr, while a heating rate >∼ 7 × 10 −24 erg s −1 prevents fragmentation. The clumps formed in freely expanding parts of bubbles are gradually destroyed and disappear on t <∼ 1 Myr,whereas those formed in the overlapping shells survive much longer. Because of this the number of fragments in an isolated bubble begins to decrease after reaching a maximum, while in collision of two old bubbles it fluctuates around 60-70 until longer than t ∼ 5 Myr.

Transport of gas from disk to halo in starforming galaxies

Using 3-D gas dynamic simulations, we study the supernova (SNe) driven transport of gas from the galactic disk. We assume that SNe are distributed randomly and uniformly in the galactic plane and we consider sufficiently high volume SNe rates that are typical for starforming galaxies: νSN = (0.3 − 3) × 10 −11 pc −3 yr −1 . We found that under such conditions, a major part of gas locked initially in the galactic disk is transported up to ∼ 1 − 5 stellar scale heights within several millions years. As expected gas transport is more efficient in the case of a thinner stellar disk. An decrease/increase of SN rate in the galactic disk with the same stellar scale height leads to an enlarging/shortening of time scale for gas transport. Independent of SN rate, the major fraction of the swept up gas is in the cold phase (T < 10 3 K), though its volume filling factor is rather small, ∼1-3%. Hot gas with T > 106 K is elevated to larger heights than cold gas.

Computer Analysis of Glioma Transcriptome Profiling: Alternative Splicing Events

Here we present the analysis of alternative splicing events on an example of glioblastoma cell culture samples using a set of computer tools in combination with database integration. The gene expression profiles of glioblastoma were obtained from cell culture samples of primary glioblastoma which were isolated and processed for RNA extraction. Transcriptome profiling of normal brain samples and glioblastoma were done by Illumina sequencing. The significant differentially expressed exon-level probes and their corresponding genes were identified using a combination of the splicing index method. Previous studies indicated that tumor-specific alternative splicing is important in the regulation of gene expression and corresponding protein functions during cancer development. Multiple alternative splicing transcripts have been identified as progression markers, including generalized splicing abnormalities and tumor- and stage-specific events. We used a set of computer tools which were recently applied to analysis of gene expression in laboratory animals to study differential splicing events. We found 69 transcripts that are differentially alternatively spliced. Three cancer-associated genes were considered in detail, in particular: APP (amyloid beta precursor protein), CASC4 (cancer susceptibility candidate 4) and TP53. Such alternative splicing opens new perspectives for cancer research.

Thermodynamic Analysis of Perspective AlB12 Synthesis Reactions from Industrially Accessible Oxygen-Free Compounds

Reactions of aluminum dodecaboride synthesis from oxygen free industrially available boron compounds by interaction of condensed and gaseous aluminum with boron nitride and carbide were the subject of thermodynamic analysis in this work. It was shown, that both reactions are thermodynamically advantageous at low temperatures rather than at high and the probability of their occurrence rises significantly for gaseous aluminum in comparison with condensed aluminum. Calculated values of Gibbs’ free energy and equilibrium constants and the analysis of contributions into them clearly demonstrate the advantages of reaction with boron nitride. The probability of polyphase product composition imposes minimum temperature restrictions on the synthesis; it should be carried out at temperatures above 1000 ℃. Hypothetical mechanisms of reactions between aluminum and boron containing compounds differ by the place of interaction — any place on the surface of each layer of BN and only open surface of B4C — and by the transport of reaction participants in the reaction zone. From the results of analysis, we suggest indicative synthesis conditions: vacuum thermal synthesis to provide oxygen free environment and temperature above 1000 ℃ to avoid aluminum diboride formation.