Influence of Sodium Dodecyl Sulfate on the Protolytic Properties of N,N-Dimethyl-N′-(2-Hydroxybenzyl) Ethylenediamine and Its Complexation with Copper(II)

To investigate the effect of sodium dodecyl sulfate (concentration Cs= 5 and 10 mM) on the acid-base properties of N,N-dimethyl-N′-(2-hydroxybenzyl) ethylenediamine and its complexation with copper(II) (Cs= 10 mM) was studied using the methods of potentiometry, spectrophotometry (at 298 K), and mathematical simulation of equilibria in solutions. The sodium dodecyl sulfate contributes to the formation of a monomeric form of N,N-dimethyl-N′-(2-hydroxybenzyl)ethylenediamine, while this compound exists in 40% isopropyl alcohol predominantly in the form of a dimer. The acidic properties of protonated monomeric and dimeric species are weaker than those in an aqueous alcohol solution. Sodium dodecyl sulfate facilitates the coordination of the deprotonated form of ligand. The region of the predominant accumulation of an uncharged complex of 1 : 2 composition is shifted to lower pH values (11.0 and 7.5, respectively), whereas the molar absorption coefficient of the complex increases by approximately 1.7 times. The apparent stability constants of complexes of the same type increase

Influence of Sodium Dodecyl Sulfate on the Protolytic Properties of N,N-Dimethyl-N′-(2-Hydroxybenzyl) Ethylenediamine and Its Complexation with Copper(II)

To investigate the effect of sodium dodecyl sulfate (concentration Cs= 5 and 10 mM) on the acid-base properties of N,N-dimethyl-N′-(2-hydroxybenzyl) ethylenediamine and its complexation with copper(II) (Cs= 10 mM) was studied using the methods of potentiometry, spectrophotometry (at 298 K), and mathematical simulation of equilibria in solutions. The sodium dodecyl sulfate contributes to the formation of a monomeric form of N,N-dimethyl-N′-(2-hydroxybenzyl)ethylenediamine, while this compound exists in 40% isopropyl alcohol predominantly in the form of a dimer. The acidic properties of protonated monomeric and dimeric species are weaker than those in an aqueous alcohol solution. Sodium dodecyl sulfate facilitates the coordination of the deprotonated form of ligand. The region of the predominant accumulation of an uncharged complex of 1 : 2 composition is shifted to lower pH values (11.0 and 7.5, respectively), whereas the molar absorption coefficient of the complex increases by approximately 1.7 times. The apparent stability constants of complexes of the same type increase

Model of high school students professional education

The main idea of the article is to develop a model of high school students professional education, which includes target, content, technology, criterion-estimation and result components contributing to ensure the efficiency and the sequence of scientific-technique provision of educational process in High School. The aim of the developed model is the future competent specialist educating on the basis of his academic and extracurricular activities integration. This objective fulfillment is specified in the complex of interrelated tasks: students civil and patriotic education system forming; student self-government system improving on the basis of corporate culture and human individuality basic principles; conditions and prerequisites providing for the students world outlook forming in integrity of which his attitude to human values of social life is expressed; conditions providing for students tolerance development and the their common culture educating; innovative environment making with the aim to develop students creative abilities; students and University graduates social and professional adaptation and socialization organizational processes; organization of students legal, social and psychological protection system. This model provides the future competent specialists self-determination; the creation of psycho-pedagogical support of personal senses development in students' academic and extra-curricular activities in new forms of professional education building: from academic - to practical-oriented one; each student interest designing in extra-curricular activities

Properties of N,N-dimethyl-N′-(2-hydroxybenzyl)ethylenediamine as a ligand to copper(II)

The acid-basic and complexing properties of N,N-dimethyl-N′-(2- hydroxybenzyl)ethylenediamine (HL) in aqueous propan-2-ol were characterized by spectrophotometry, pH-metry, and mathematical simulation of equilibria in solutions (T = 25 ± 0.1°C, μ = 0.1 M KNO 3). Dimer H 2L 2 was found to predominate in solution at c HL = 0.01 mol/l. Three protonated dimeric (H 3L 2 +, H 4L 2 2+, and H 5L 2 3+), diprotonated monomeric (H 3L 2+), and triprotonated tetrameric forms (H 7L 4 3+) were detected in the system, depending on pH. At lower ligand concentrations (c HL = 0.0015 mol/l), the solution contains both dimers and monomers of this compound. The higher dentate number of HL compared to 2-alkylaminomethylphenols allows it to form more number of both mono- and binuclear complexes ([Cu(HL)] 2+, [Cu(HL) 2] 2+, [CuL 2], [CuL 2OH] -, [Cu 2(HL) 2] 4+, and [Cu 2(HL) 2L 2] 2+), making them more stable

The peculiarities of the advanced training of the future specialists for the competitive high-tech industry in the process of integration of education, science and industry

© 2015, Mediterranean Center of Social and Educational Research. All rights reserved. The urgency of the advanced professional training of the future competitive specialists for a high-tech industry is conditioned by the fact that one of the strategic objectives of modern production is to provide a new generation with the manpower, the leaders of production who can not only purchase and adapt foreign models, but also but have the qualification to develop and introduce new home-produced samples, and also be able to quickly adapt to new social and economic conditions. Therefore, this article aims to identify the features of the advanced training of the future specialists for high-tech industry in the process of integration of education, science and industry. The authors have found the distinctive features of the institution providing the advanced training of the future competitive specialists; proposed measures for the development of advanced training; set the requirements to the syllabus and technology of advanced training; discussed the conditions for the implementation of the advanced training of the future competitive specialists in the condition of education, science and industry integration. The issues of this article are of both theoretical and practical value for the management and teaching staff of the vocational education institutions

Structure and content of higher professional school lecturer education competence

© 2015, Review of European Studies. All right reserved. Article aims to develop the structure and content of higher professional school lecturer education competence as a lecturer professionalism should be evaluated not only on the quality of the studies, but also for his participation in education activities in higher school. It is needed to rely on a stating that education competence is an integral feature of professional and educational activities aimed at the effectiveness of the educational process in higher professional school in developing its structure and content. In the structure of the lecturer education competence there are four components: humanologic, socio-pedagogical, organizational and methodical, professional personal. Their content is disclosed in knowledge, skills, abilities and personal qualities as necessary for the implementation of the education activities in higher professional school. The presented structure of high school lecturer education competence facilitates the efficient evaluation of the competences, the identification of the gaps in the competencies development levels and their correction with additional measures and means aimed to their effective formation

Bi-allelic <em>ACBD6</em> variants lead to a neurodevelopmental syndrome with progressive and complex movement disorders

\ua9 The Author(s) 2023. Published by Oxford University Press on behalf of the Guarantors of Brain. The acyl-CoA-binding domain-containing protein 6 (ACBD6) is ubiquitously expressed, plays a role in the acylation of lipids and proteins and regulates the N-myristoylation of proteins via N-myristoyltransferase enzymes (NMTs). However, its precise function in cells is still unclear, as is the consequence of ACBD6 defects on human pathophysiology. Using exome sequencing and extensive international data sharing efforts, we identified 45 affected individuals from 28 unrelated families (consanguinity 93%) with bi-allelic pathogenic, predominantly loss-of-function (18/20) variants in ACBD6. We generated zebrafish and Xenopus tropicalis acbd6 knockouts by CRISPR/Cas9 and characterized the role of ACBD6 on protein N-myristoylation with myristic acid alkyne (YnMyr) chemical proteomics in the model organisms and human cells, with the latter also being subjected further to ACBD6 peroxisomal localization studies. The affected individuals (23 males and 22 females), aged 1-50 years, typically present with a complex and progressive disease involving moderate-to-severe global developmental delay/intellectual disability (100%) with significant expressive language impairment (98%), movement disorders (97%), facial dysmorphism (95%) and mild cerebellar ataxia (85%) associated with gait impairment (94%), limb spasticity/hypertonia (76%), oculomotor (71%) and behavioural abnormalities (65%), overweight (59%), microcephaly (39%) and epilepsy (33%). The most conspicuous and common movement disorder was dystonia (94%), frequently leading to early-onset progressive postural deformities (97%), limb dystonia (55%) and cervical dystonia (31%). A jerky tremor in the upper limbs (63%), a mild head tremor (59%), parkinsonism/hypokinesia developing with advancing age (32%) and simple motor and vocal tics were among other frequent movement disorders. Midline brain malformations including corpus callosum abnormalities (70%), hypoplasia/agenesis of the anterior commissure (66%), short midbrain and small inferior cerebellar vermis (38% each) as well as hypertrophy of the clava (24%) were common neuroimaging findings. Acbd6-deficient zebrafish and Xenopus models effectively recapitulated many clinical phenotypes reported in patients including movement disorders, progressive neuromotor impairment, seizures, microcephaly, craniofacial dysmorphism and midbrain defects accompanied by developmental delay with increased mortality over time. Unlike ACBD5, ACBD6 did not show a peroxisomal localization and ACBD6-deficiency was not associated with altered peroxisomal parameters in patient fibroblasts. Significant differences in YnMyr-labelling were observed for 68 co- and 18 post-translationally N-myristoylated proteins in patient-derived fibroblasts. N-myristoylation was similarly affected in acbd6-deficient zebrafish and X. tropicalis models, including Fus, Marcks and Chchd-related proteins implicated in neurological diseases. The present study provides evidence that bi-allelic pathogenic variants in ACBD6 lead to a distinct neurodevelopmental syndrome accompanied by complex and progressive cognitive and movement disorders

AMPA receptor GluA2 subunit defects are a cause of neurodevelopmental disorders.

AMPA receptors (AMPARs) are tetrameric ligand-gated channels made up of combinations of GluA1-4 subunits encoded by GRIA1-4 genes. GluA2 has an especially important role because, following post-transcriptional editing at the Q607 site, it renders heteromultimeric AMPARs Ca2+-impermeable, with a linear relationship between current and trans-membrane voltage. Here, we report heterozygous de novo GRIA2 mutations in 28 unrelated patients with intellectual disability (ID) and neurodevelopmental abnormalities including autism spectrum disorder (ASD), Rett syndrome-like features, and seizures or developmental epileptic encephalopathy (DEE). In functional expression studies, mutations lead to a decrease in agonist-evoked current mediated by mutant subunits compared to wild-type channels. When GluA2 subunits are co-expressed with GluA1, most GRIA2 mutations cause a decreased current amplitude and some also affect voltage rectification. Our results show that de-novo variants in GRIA2 can cause neurodevelopmental disorders, complementing evidence that other genetic causes of ID, ASD and DEE also disrupt glutamatergic synaptic transmission

Bi-allelic ACBD6 variants lead to a neurodevelopmental syndrome with progressive and complex movement disorders

This is the author accepted manuscript. The final version is available from Oxford University Press via the DOI in this recordData availability: The data that support the findings of this study are available from the corresponding author, upon reasonable request. The mass spectrometry proteomics data have been deposited to the ProteomeXchange Consortium via the PRIDE24 partner repository with the dataset identifiers PXD024957 (YnMyr chemical proteomics in human cells), PXD043676 (YnMyr chemical proteomics in zebrafish), PXD043679 (zebrafish whole proteome), PXD043677 (YnMyr chemical proteomics in X. tropicalis) and PXD043680 (X. tropicalis whole proteome).The acyl-CoA-binding domain-containing protein 6 (ACBD6) is ubiquitously expressed, plays a role in the acylation of lipids and proteins, and regulates the N-myristoylation of proteins via N-myristoyltransferase enzymes (NMTs). However, its precise function in cells is still unclear, as is the consequence of ACBD6 defects on human pathophysiology. Utilizing exome sequencing and extensive international data sharing efforts, we identified 45 affected individuals from 28 unrelated families (consanguinity 93%) with bi-allelic pathogenic, predominantly loss-of-function (18/20) variants in ACBD6. We generated zebrafish and Xenopus tropicalis acbd6 knockouts by CRISPR/Cas9 and characterized the role of ACBD6 on protein N-myristoylation with YnMyr chemical proteomics in the model organisms and human cells, with the latter also being subjected further to ACBD6 peroxisomal localization studies. The affected individuals (23 males and 22 females), with ages ranging from 1 to 50 years old, typically present with a complex and progressive disease involving moderate-to-severe global developmental delay/intellectual disability (100%) with significant expressive language impairment (98%), movement disorders (97%), facial dysmorphism (95%), and mild cerebellar ataxia (85%) associated with gait impairment (94%), limb spasticity/hypertonia (76%), oculomotor (71%) and behavioural abnormalities (65%), overweight (59%), microcephaly (39%) and epilepsy (33%). The most conspicuous and common movement disorder was dystonia (94%), frequently leading to early-onset progressive postural deformities (97%), limb dystonia (55%), and cervical dystonia (31%). A jerky tremor in the upper limbs (63%), a mild head tremor (59%), parkinsonism/hypokinesia developing with advancing age (32%), and simple motor and vocal tics were among other frequent movement disorders. Midline brain malformations including corpus callosum abnormalities (70%), hypoplasia/agenesis of the anterior commissure (66%), short midbrain and small inferior cerebellar vermis (38% each), as well as hypertrophy of the clava (24%) were common neuroimaging findings. acbd6-deficient zebrafish and Xenopus models effectively recapitulated many clinical phenotypes reported in patients including movement disorders, progressive neuromotor impairment, seizures, microcephaly, craniofacial dysmorphism, and midbrain defects accompanied by developmental delay with increased mortality over time. Unlike ACBD5, ACBD6 did not show a peroxisomal localisation and ACBD6-deficiency was not associated with altered peroxisomal parameters in patient fibroblasts. Significant differences in YnMyr-labelling were observed for 68 co- and 18 post-translationally N-myristoylated proteins in patient-derived fibroblasts. N-Myristoylation was similarly affected in acbd6-deficient zebrafish and Xenopus tropicalis models, including Fus, Marcks, and Chchd-related proteins implicated in neurological diseases. The present study provides evidence that bi-allelic pathogenic variants in ACBD6 lead to a distinct neurodevelopmental syndrome accompanied by complex and progressive cognitive and movement disorders

Influence of Sodium Dodecyl Sulfate on the Protolytic Properties of N,N-Dimethyl-N′-(2-Hydroxybenzyl) Ethylenediamine and Its Complexation with Copper(II)

To investigate the effect of sodium dodecyl sulfate (concentration Cs= 5 and 10 mM) on the acid-base properties of N,N-dimethyl-N′-(2-hydroxybenzyl) ethylenediamine and its complexation with copper(II) (Cs= 10 mM) was studied using the methods of potentiometry, spectrophotometry (at 298 K), and mathematical simulation of equilibria in solutions. The sodium dodecyl sulfate contributes to the formation of a monomeric form of N,N-dimethyl-N′-(2-hydroxybenzyl)ethylenediamine, while this compound exists in 40% isopropyl alcohol predominantly in the form of a dimer. The acidic properties of protonated monomeric and dimeric species are weaker than those in an aqueous alcohol solution. Sodium dodecyl sulfate facilitates the coordination of the deprotonated form of ligand. The region of the predominant accumulation of an uncharged complex of 1 : 2 composition is shifted to lower pH values (11.0 and 7.5, respectively), whereas the molar absorption coefficient of the complex increases by approximately 1.7 times. The apparent stability constants of complexes of the same type increase