MODERN STABLE MATHEMATICAL AND SOFTWARE-BASED METHODS FOR DISTORTED SPECTRA RESTORATION

The paper presents analysis and comparison of various methods and algorithms for restoration of the spectra fine structure smoothed by the instrumental spectrometer function and/or having the overlapping of close spectral lines. Continuous and discrete spectra are considered. Successful spectra restoration enhances mathematically the resolution of spectrometers. In the case of a continuous spectrum smoothing by the instrumental function, the problem of restoration is reduced to solving integral equations of the first kind. This problem is ill-posed (essentially unstable). Therefore, to obtain a stable solution of integral equations, the Tikhonov regularization, Wiener filtering, Kalman–Bucy and other methods are used. However, in the case of close lines overlapping in the spectrum, these methods make it possible to restore only the total spectrum, but not the profiles of each line. To separate line profiles, the desired lines are modeled by the Gaussians or Lorentzians; the total spectrum is differentiated using smoothing splines; the number and parameters of the lines are estimated from the results of differentiation. To refine the line parameters, minimization of the discrepancy functional by the coordinate descent method and for comparison by the Nelder–Mead method is performed. A comparison is also made with the Fourier-self-deconvolution method, in which the line widths are artificially reduced due to apodization (the interferogram truncation), and, as a result, the true line profiles are distorted for their resolution. In the original convolution method, the parameters of lines (peaks) are determined from convolutions of experimental spectrum with model spectrum derivatives. If a discrete spectrum is smoothed by the instrumental function, then the problem of spectrum restoration is described by a system of linear-non-linear equations (SLNE) and solved by the integral approximation algorithm that is more efficient than the Prony method, the Golub–Mullen–Hegland variable projection method, and other methods. Based on the results of the review of various mathematical methods, it is proposed to create a new complex algorithm for distorted spectra restoration, which makes it possible to remove the effect of instrumental function, noise, lines overlapping and other effects. The software in MATLAB is developed and the processing of a number of spectra is performed. The stated technique can be used to enhance the spectrometer resolution via mathematical and computer processing of spectra

Pairing and Density Correlations of Stripe Electrons in a Two-Dimensional Antiferromagnet

We study a one-dimensional electron liquid embedded in a 2D antiferromagnetic insulator, and coupled to it via a weak antiferromagnetic spin exchange interaction. We argue that this model may qualitatively capture the physics of a single charge stripe in the cuprates on length- and time scales shorter than those set by its fluctuation dynamics. Using a local mean-field approach we identify the low-energy effective theory that describes the electronic spin sector of the stripe as that of a sine-Gordon model. We determine its phases via a perturbative renormalization group analysis. For realistic values of the model parameters we obtain a phase characterized by enhanced spin density and composite charge density wave correlations, coexisting with subleading triplet and composite singlet pairing correlations. This result is shown to be independent of the spatial orientation of the stripe on the square lattice. Slow transverse fluctuations of the stripes tend to suppress the density correlations, thus promoting the pairing instabilities. The largest amplitudes for the composite instabilities appear when the stripe forms an antiphase domain wall in the antiferromagnet. For twisted spin alignments the amplitudes decrease and leave room for a new type of composite pairing correlation, breaking parity but preserving time reversal symmetry.Comment: Revtex, 28 pages incl. 5 figure

Перспективы этиотропного лечения дисферлинопатий

Dysferlinopathies belong to a phenotypically heterogeneous group of neuromuscular diseases caused by mutations in the DYSF gene, which disrupt the expression of dysferlin protein in human skeletal muscle cells. These pathologies are of an autosomal recessive inheritance pattern, their prevalence is 1: 200000. Dysferlinopathies include diseases such as Miyoshi myopathy with primary lesion of the distal fragments of the lower extremities and limb-gridle muscular dystrophy type 2B with primary lesion of the proximal fragments of both the lower and upper limbs, also distal myopathy with anterior tibial onset (DMAT). Nowdays, there are various pathogenetic and symptomatic treatments for hereditary muscular dystrophies but there are very few registered drugs for the etiological treatment of these diseases. This review discusses the main modern methods of gene therapy that can be used to treat dysferlinopathies, such as stop-codon passing, exon skipping, overexpression of other genes, gene transfer, splicosome-mediated trans-splicing, and also describes the latest experimental studies using these methods. In conclusion, exon-skipping and trans-splicing have been identified as the most optimal approaches in the treatment of muscular dystrophies, in particular dysferlinopathies.Дисферлинопатии относятся к фенотипически гетерогенной группе нервно-мышечных заболеваний, причиной которых являются мутации в гене DYSF, вследствие которых нарушается экспрессия белка дисферлина в клетках скелетной мышечной ткани человека. Патологии носят аутосомно-рецессивный характер наследования, распространенность составляет 1:200 000. К дисферлинопатиям относятся такие заболевания, как миопатия Миоши с первичным поражением дистальных фрагментов нижних конечностей и поясно-конечностная мышечная дистрофия типа 2Б с первичным поражением проксимальных фрагментов и нижних, и верхних конечностей, а также дистальная миопатия переднего ложа голени (ДМПЛГ). На сегодняшний день существуют различные патогенетические и симптоматические способы терапии наследственных мышечных дистрофий, однако очень мало зарегистрированных препаратов для этиологического лечения этих заболеваний. В настоящем обзоре рассмотрены основные современные методы генной терапии, которые могут быть применены в целях лечения дисферлинопатий, такие как прохождение стоп-кодона, пропуск экзонов, оверэкспрессия других генов, перенос гена, сплайсосомо-опосредованный транссплайсинг, а также описаны последние экспериментальные исследования с использованием этих методов. В заключение экзон-скиппинг и транс-сплайсинг выделены как наиболее оптимальные подходы в терапии миодистрофий, в частности дисферлинопатий

Integrative taxonomic re-description of halisarca magellanica and description of a new species of Halisarca (Porifera, Demospongiae) from Chilean Patagonia

A series of recent expeditions in fjords and canals of Southern Chilean Patagonia allowed the re-collection of Halisarca magellanica Topsent, 1901 and the discovery of a new species, Halisarca desqueyrouxae sp. nov. The material studied was collected at depths ranging from 3 to 30 m at latitudes comprised between 42° and 49°S. Both species share the same habitat and show a morphological plasticity, but differ in their colour. Halisarca magellanica is bright pink to whitish with three morphs whereas H. desqueyrouxae sp. nov. is light brown to beige with two morphs. An extensive investigation in TEM and SEM reveals several differences among cell types with inclusions between both species. Three distinct spherulous cells occur. Type 1 is shared by both species, Type 2 is occasional in H. magellanica but absent from H. desqueyrouxae sp. nov. Type 3 is rare in H. magellanica and occurs abundantly in half of the specimens of H. desqueyrouxae sp. nov. Granular cells are shared by both species but do not occur in all specimens. Microgranular cells are characteristic of H. magellanica. Both species also clearly differ by their endobiotic bacteria. Phylogenetic analysis of cox1 sequences places H. magellanica as a sister group to all other previously published Halisarca species sequences (9.1-9.7% difference) except H. harmelini, while H. desqueyrouxae sp. nov. is placed as a sister group to H. dujardini (2.3% difference).SCOPUS: ar.jinfo:eu-repo/semantics/publishe

FACTOR ANALYSIS AND GROWTH PROSPECTS OF POTABLE WATER LOCAL MARKET

Currently, the clean potable water is globally the restricted economic benefit. In highly urbanized and environmentally unfavorable regions, including the Kemerovo region, development of food plants to fill drinking water is the most promising way to solve the problem of potable water availability. Factors and conditions of the drinking water market formation are studied by integral evaluation of drinking water availability in all municipal districts of the region, using the criteria of availability in terms of geographic location, management, technological process, economic value and quality. The volume of supply of bottled drinking water is also analyzed in view of its availability. As a result, the data on the level of availability of drinking water is first obtained for residents of all municipal districts of the Kemerovo region, on the potential of the population to pay for the pure water delivery and on prospects to expand the bottled water production market. The most population was identified to live in conditions with low technological, economic and environmental access to drinking water. The residents of big and medium-sized cities live in conditions of low environmental availability and high potential to pay for the drinking water delivery. The residents of peripheral municipalities live in conditions with low access to potable water due to management, technology and economic restriction but within the high geographic availability. Thus, the analysis of the drinking water availability and volume of its production suggest the possibility of the local market considerable capacity and its growth in future

Кардиомиопатии, ассоциированные с мутациями гена десмина: молекулярный патогенез и генотерапевтические подходы

Cardiomyopathy (CMP) is a common group of cardiovascular disorders. Genetic (primary) cardiomyopathies are related to abnormalities in more than 100 genes, including the DES gene encoding desmin protein. Desmin is an essential member of the intermediate filaments, ensuring the structural and functional integrity of myocytes. Mutations in the DES gene result in desmin-related cardiomyopathy with progressive course and poor prognosis. By now, specific therapy for cardiomyopathy has not been developed. Existing conservative and surgical treatment modalities target the rate of heart failure progression and sudden cardiac death prevention but have limited efficacy. The development of gene therapy and genome editing could allow for creating effective and specific methods of gene-based therapy for desminopathies. A  number of studies have been published on the use of gene therapy for various genetic cardiomyopathies including those caused by the DES gene mutations, while genome editing has not been used yet. However, promising results have been obtained with CRISPR/Cas9 and TALEN editing systems to correct for “gain-of-function mutations” in some other genes, such as MYBPC3 and PLN. There is also evidence of the possibility to reduce the symptoms of desmin-related cardiomyopathy up to the normal function by knocking out the mutant DES allele, and preserved protein function provided by expression of the normal allele. We believe that genome editing approaches have an open perspective into the development of specific and effective methods to treat desminopathies.Кардиомиопатия – широко распространенная группа заболеваний сердечно-сосудистой системы. Генетически обусловленные кардиомиопатии связывают с  нарушениями более чем в 100 различных генах, в том числе в гене DES, кодирующем белок десмин  – один из основных белков промежуточных филаментов, обеспечивающих структурную и функциональную целостность миоцитов. Мутации в  гене DES приводят к  развитию десминзависимых кардиомиопатий, характеризующихся высокой степенью тяжести течения и неблагоприятным прогнозом. До настоящего времени специфического лечения кардиомиопатии не разработано. Имеющиеся консервативные и хирургические подходы направлены на замедление темпов прогрессирования сердечной недостаточности и  профилактику внезапной сердечной смерти, но их эффективность ограничена. Развитие методов генотерапии и  геномного редактирования может способствовать созданию эффективных методов этиотропной терапии десминопатий. Опубликован ряд работ, посвященных применению методов генотерапии при кардиомиопатиях различной генетической природы, включая ассоциированные с мутациями в гене DES. В области терапии десминопатий методы геномного редактирования пока не используются. Тем не менее многообещающие результаты получены при использовании систем редактирования CRISPR/Cas9 и  TALEN для коррекции “gain-of-function” мутаций в  некоторых других генах, таких как MYBPC3 и PLN. Имеются данные, указывающие на возможность улучшения симптоматики десминзависимой кардиомиопатии, вплоть до бессимптомного течения после нокаута мутантного аллеля с сохранением функции белка за счет экспрессии только нормального аллеля. Мы считаем, что подходы, основанные на технологии геномного редактирования, представляют собой перспективное направление для разработки эффективных специфических методов лечения десминопатий