A new technology of treatment of polymeric materials cutting

В статті розроблено новий метод досягнення високої точності та новий метод технологічного досягнення якості обробки полімерних покриттів на великогабаритних нежорстких виробах. Розроблені принципово нові схеми розмірної обробки в’язкопружних полімерних матеріалів, основані на використанні фізичного ефекту ковзного різання, що забезпечує повне виконання основних технологічних вимог до якості та точності обробленої поверхні. Сформульована гіпотеза про механізм ковзного фрезерування в’язкопружних полімерних матеріалів.In this article considered and decided problem of machining of polymeric materials on non-rigid large-dimensioned constructions for achievement of maximal exactness, quality and productivity. The purpose of this work is establishment of features, conformities to law and dependences of cutting of viscoelastic polymeric materials on large-dimensioned non-rigid materials for the increase of exactness of coating thickness, improvement of quality of the machined surface and ecology on a production, and also achievements of more high yield as compared to existent methods. For this purpose developed hypothesis about the increase of exactness of active control at machining of dielectric coverages on hardwares with the use of vortical currents and hypothesis about the mechanism of the sliding cutting of viscoelastic polymeric materials, allowing to manage the process of separation of the cut away layer. The on principle new charts of size machining of viscoelastic polymeric materials, based on the use of physical effect of the sliding cutting, providing complete implementation of the basic technological requirements to quality and exactness of the treated surface, are developed. The new method of machining of viscoelastic polymeric materials is based on the use of cutting chart in two mutually perpendicular directions, allowing to realize the sliding cutting. It will allow substantially to decrease deformation of the processed material as compared to the traditional cutting and, thus, create terms at which energy of deformation will be transformed in work of break of molecular connections on more early stage.В статье разработаны новый метод достижения высокой точности и новый метод технологического достижения качества обработки полимерных покрытий на крупногабаритных нежестких изделиях. Разработаны принципиально новые схемы размерной обработки вязкоупругих полимерных материалов, основанные на использовании физического эффекта скользящего резания, обеспечивающие полное выполнение основных технологических требований к качеству и точности обработанной поверхности. Сформулирована гипотеза о механизме скользящего фрезерования вязкоупругих полимерных материалов

Novel domain-specific POU3F4 mutations are associated with X-linked deafness: examples from different populations

BACKGROUND: Mutations in the POU3F4 gene cause X-linked deafness type 3 (DFN3), which is characterized by inner ear anomalies. METHODS: Three Turkish, one Ecuadorian, and one Nigerian families were included based on either inner ear anomalies detected in probands or X-linked family histories. Exome sequencing and/or Sanger sequencing were performed in order to identify the causative DNA variants in these families. RESULTS: Four novel, c.707A>C (p.(Glu236Ala)), c.772delG (p.(Glu258ArgfsX30)), c.902C>T (p.(Pro301Leu)), c.987T>C (p.(Ile308Thr)), and one previously reported mutation c.346delG (p.(Ala116ProfsX26)) in POU3F4, were identified. All mutations identified are predicted to affect the POU-specific or POU homeo domains of the protein and co-segregated with deafness in all families. CONCLUSIONS: Expanding the spectrum of POU3F4 mutations in different populations along with their associated phenotypes provides better understanding of their clinical importance and will be helpful in clinical evaluation and counseling of the affected individuals. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1186/s12881-015-0149-2) contains supplementary material, which is available to authorized users

A tectorin-based matrix and planar-cell-polarity genes are required for normal collagen-fibril orientation in the developing tectorial membrane

The tectorial membrane is an extracellular structure of the cochlea. It develops on the surface of an epithelium and contains collagen fibrils embedded in a tectorin-based matrix. The collagen fibrils are oriented radially with an apically-directed slant - a feature considered critical for hearing. To determine how this pattern is generated, collagen-fibril formation was examined in mice lacking a tectorin-based matrix, epithelial cilia, or the planar-cell-polarity genes Vangl2 and Ptk7. In wild-type mice, collagen-fibril bundles appear within a tectorin-based matrix at E15.5 and, as fibril-number rapidly increases, become co-aligned and correctly oriented. Epithelial-width measurements and data from Kif3acKO mice suggest, respectively, radial stretch and cilia play little, if any, role in determining normal collagen-fibril orientation, but evidence from tectorin-knockout mice indicates confinement is important. PRICKLE2 distribution reveals the planar-cell-polarity axis in the underlying epithelium is organised along the length of the cochlea and, in mice in which this polarity is disrupted, the apically-directed collagen offset is no longer observed. These results highlight the importance of the tectorin-based matrix and epithelial signals for precise collagen organisation in the tectorial membran

Three deaf mice: mouse models for TECTA-based human hereditary deafness reveal domain-specific structural phenotypes in the tectorial membrane

Tecta is a modular, non-collagenous protein of the tectorial membrane, an extracellular matrix of the cochlea essential for normal hearing. Missense mutations in Tecta cause dominant forms of nonsyndromic deafness and a genotype-phenotype correlation has been reported in humans, with mutations in different Tecta domains causing mid- or high-frequency hearing impairments that are either stable or progressive. Three mutant mice were created as models for human Tecta mutations; the TectaL1820F, G1824D/+ mouse for zona pellucida (ZP) domain mutations causing stable mid-frequency hearing loss in a Belgian family, the TectaC1837G/+ mouse for a ZP-domain mutation underlying progressive mid-frequency hearing loss in a Spanish family, and the TectaC1619S/+ mouse for a zonadhesin-like (ZA) domain mutation responsible for progressive, high-frequency hearing loss in a French family. Mutations in the ZP and ZA domains generate distinctly different changes in the structure of the tectorial membrane. ABR thresholds in the 8-40 kHz range are elevated by 30-40 dB in the ZP-domain mutants, whilst those in the ZA-domain mutant are elevated by 20-30 dB. The phenotypes are stable and no evidence has been found for a progressive deterioration in tectorial membrane structure or auditory function. Despite elevated auditory thresholds, the Tecta mutant mice all exhibit an enhanced tendency to have audiogenic seizures in response to white noise stimuli at low sound pressure levels (≤84 dB SPL), revealing a previously unrecognised consequence of Tecta mutations. These results, together with those from previous studies, establish an allelic series for Tecta unequivocally demonstrating an association between genotype and phenotype

The Chronicles of X-Linked Spinal Muscular Atrophy: The Linkage, The Gene and The SMN Complex

Spinal Muscular Atrophy (SMA) is an autosomal recessive motor neuron disease. SMA is associated with homozygous mutations in the Survival of Motor Neuron gene I (SMN1). SMN protein does not appear to exist in cells in isolation but associates with several proteins to form a large multi-protein complex. The functions of SMN complex include assembly, metabolism and transport of diverse classes of ribonucleoproteins. X- Linked Spinal Muscular Atrophy is a rare congenital disorder characterized by multiple joint contractures. It is associated with hypotonia, areflexia, chest deformities and congenital joint contractures. A candidate interval was defined for XL-SMA in Xp11.3-Xq11.2 in 1995. The purpose of this study was to refine the XL-SMA gene region and discover the XL-SMA gene. In addition to that, the gene product was investigated to delineate the genotype-phenotype correlation. My studies were focused on single nucleotide polymorphism (SNP) analysis. The candidate gene interval was refined by studying 14 SNPs in the three largest families. This analysis revealed a recombination event which allowed elimination of the NDP gene. Significantly positive LOD scores were obtained from these SNP studies. The exons and exon-intron boundaries of 12 genes were screened. No mutations were found in these genes in affected male samples. In late 2006, UBE1 (Ubiquitin activating enzyme 1) was discovered as the XL-SMA gene. UBE1 protein is responsible for the first step of ubiquitination of proteins in a cell. To investigate a possible common molecular mechanism between SMA and XL-SMA, proteins in the SMN Complex in XL-SMA patient cell lines were studied. SMN and Gemin3 protein levels were found to be consistently lower in XL-SMA patient cell lines (lymphoblasts) compared to healthy cell line. These results imply that there may be a common disease mechanism. To understand if the SMN and Gemin3 RNA levels decrease. RNA expression studies were performed. These studies confirmed that there is no difference of RNA expression of SMN and Gemin3 in XL-SMA cell lines when compared to healthy cell lines. As for UBE1, the same experimental procedure for SMN Complex proteins were repeated with antibodies to UBE1 to determine if there is any decline of UBE1 protein levels in XL-SMA patient cell lines compared to a healthy cell line. There was a decline in protein levels of UBE1 in XL-SMA patients. Two possible models are proposed for a molecular mechanism in XL-SMA: 1) UBE1 involves in degradation of a protein which downregulates SMN Complex (or a protein which stabilizes SMN Complex). When UBE1 is mutated, the protein in question is not degraded and this results in excess downregulation of SMN Complex (maybe via a pathway involving SMN-Gemin3 interaction). 2) UBE1 and UBA6 interact with the proteins of SMN Complex as they monoubiquinate them for different cellular processes. When UBE1 is mutated, UBA6 cannot compensate the deficiency of UBE1, which in turn disrupts normal cellular RNA metabolism required for motor neuron development and survival

Підвищення енергоефективності роботи теплового реактора з апаратом зануреного горіння циклічним вводом енергії

We examined the formation of oscillations of contacting phases (gas–fluid) in the thermal reactors, equipped with submerged combustion apparatuses, with the help of the cyclic input of energy for the intensification of heat-mass-exchange processes and improvement in the energy efficiency of their operation. It is established that the cyclic input of external energy increases the mass transfer by 2–2.5 times, as well as energy effectiveness of the process as a whole. A distinctive feature of the thermal reactor with the cyclic input of energy is in the fact that the air, preheated in the thermal installation with SCA that works on the principle of a "vapor pump", enters the upper and lower collectors of SAD. In this case, the air is dispersed depending on the angle of blade rotation, which contributes to the turbulization of phase boundary, which improves energy efficiency of the reactor operation.Based on the conducted studies, we designed a thermal reactor with the built-in submerged combustion apparatuses and the cyclic input of external energy for creating the turbulent pulsations that works on the principle of a "vapor pump". The performance characteristics of the device with the cyclic input of external energy were determined based on the obtained results. The developed thermal reactor makes it possible to control the process of heating and can be easily modified, depending on the technological requirements. The energy-technological indicators of the designed thermal reactor meet the world requirements: the content of nitrogen oxides does not exceed 56.8 mg/m3 at high-temperature water heating – to 70–80 °С. The thermodynamic efficiency of the thermal reactor operation reaches 98.6 % (by direct balance).Исследовано создание колебаний контактирующих фаз (газ – жидкость) в тепловых реакторах, оборудованных аппаратами погружного горения, с помощью циклического ввода энергии для интенсификации тепломассообменных процессов и повышения энергоэффективности их работы. Аналитически и экспериментально определены характеристики процесса и их влияние на интенсивность турбулентных пульсаций. Предложена и исследована конструкция теплового реактора, оборудованная аппаратом погружного горения с циклическим вводом внешней энергииДосліджено утворення коливань контактуючих фаз (газ – рідина) в теплових реакторах, обладнаних апаратами зануреного горіння, за допомогою циклічного вводу енергії для інтенсифікації тепломасообмінних процесів і підвищення енергоефективності їх роботи. Аналітично і експериментально визначено характеристики процесу і їх вплив на інтенсивність турбулентних пульсацій. Запропонована і досліджена конструкція теплового реактора, обладнаного апаратом зануреного горіння з циклічним введенням зовнішньої енергі