Microstructure and properties of 308LSI steel obtained by deposition of metal wire

Shaped metal deposition (SMD) is a relatively new technology of additive manufacturing, which creates near-net shaped components by joining metallic materials by melting the area of a welding joint in high vacuum in the range from 10-3 to 10-6 mbar. In the present study, the main mechanical properties including micro-hardness and tensile properties were investigated. Single bead walls were deposited. Test pieces were machined from the deposited walls according to the National standard of Russia Federation for the mechanical tests. The tensile properties also showed dependence on the direction of the test carried out. All the examined tensile properties of the as deposited samples are close-matched properties of the as cast material

Proporción diaria de alimento del diablillo antártico (pleuragramma antarcticum boulenger, 1902) en el este del mar de weddell

[EN] The daily ration of Pleuragramma antarcticum in the Eastern Weddell Sea was investigated from midwater and bottom trawl samples collected in the Antarctic in the summer of 1998. Using a gastric evacuation model that takes into account Weddell Sea temperature below zero and information on the prey type daily ration estimates were: 1.133% BW for immature fish of 10-16 cm and 0.484% BW for mature fish of 17-24 cm. The low daily ration intake was influenced by the low temperatures that limited the rate of gastric evacuation. This model seems more realistic than results from the classic Elliot & Persson and Eggers models that are also used in this paper, since their assumptions on feeding regularity are more rigid and they do not consider data of energy density of the prey[ES] La ración diaria de alimento de Pleuragramma antarcticum en el mar de Weddell es investigada en muestras de arrastres de fondo y pelágicas recogidas durante diferentes horas del día en el verano antártico de 1998. Utilizando un modelo de evacuación gástrica que tiene en cuenta la temperatura bajo del Mar de Weddell e información sobre el tipo de presa, las estimaciones de consumo diario fueron: 1.133% BW para peces inmaduros entre 10 -16 cm y 0.484% BW para peces maduros entre 17-24 cm. La baja ración diaria ingerida estuvo influida por las bajas temperaturas que limitan la tasa de evacuación gástrica. Este modelo parece más realista que los resultados de los modelos clásicos de Elliot & Persson and Eggers también empleados, ya que sus asunciones sobre su periodicidad de alimentación son más rígidas y no consideran datos de densidad energética de las presasThis work was conducted under the SCAR EASIZ programme, and was supported by the Alfred Wegener Institute and the Spanish Antarctic ProgrammePeer reviewe

Genetic heterogeneity in Italian families with IgA nephropathy: suggestive linkage for two novel IgA nephropathy loci.

IgA nephropathy (IgAN) is the most common glomerulonephritis worldwide, but its etiologic mechanisms are still poorly understood. Different prevalences among ethnic groups and familial aggregation, together with an increased familial risk, suggest important genetic influences on its pathogenesis. A locus for familial IgAN, called "IGAN1," on chromosome 6q22-23 has been described, without the identification of any responsible gene. The partners of the European IgAN Consortium organized a second genomewide scan in 22 new informative Italian multiplex families. A total of 186 subjects (59 affected and 127 unaffected) were genotyped and were included in a two-stage genomewide linkage analysis. The regions 4q26-31 and 17q12-22 exhibited the strongest evidence of linkage by nonparametric analysis (best P=.0025 and .0045, respectively). These localizations were also supported by multipoint parametric analysis, in which peak LOD scores of 1.83 ( alpha =0.50) and 2.56 ( alpha =0.65) were obtained using the affected-only dominant model, and by allowance for the presence of genetic heterogeneity. Our results provide further evidence for genetic heterogeneity among families with IgAN. Evidence of linkage to multiple chromosomal regions is consistent with both an oligo/polygenic and a multiple-susceptibility-gene model for familial IgAN, with small or moderate effects in determining the pathological phenotype. Although we identified new candidate regions, replication studies are required to confirm the genetic contribution to familial IgA

Mutations in Subunits of the Activating Signal Cointegrator 1 Complex Are Associated with Prenatal Spinal Muscular Atrophy and Congenital Bone Fractures

Transcriptional signal cointegrators associate with transcription factors or nuclear receptors and coregulate tissue-specific gene transcription. We report on recessive loss-of-function mutations in two genes (TRIP4 and ASCC1) that encode subunits of the nuclear activating signal cointegrator 1 (ASC-1) complex. We used autozygosity mapping and whole-exome sequencing to search for pathogenic mutations in four families. Affected individuals presented with prenatal-onset spinal muscular atrophy (SMA), multiple congenital contractures (arthrogryposis multiplex congenita), respiratory distress, and congenital bone fractures. We identified homozygous and compound-heterozygous nonsense and frameshift TRIP4 and ASCC1 mutations that led to a truncation or the entire absence of the respective proteins and cosegregated with the disease phenotype. Trip4 and Ascc1 have identical expression patterns in 17.5-day-old mouse embryos with high expression levels in the spinal cord, brain, paraspinal ganglia, thyroid, and submandibular glands. Antisense morpholino-mediated knockdown of either trip4 or ascc1 in zebrafish disrupted the highly patterned and coordinated process of α-motoneuron outgrowth and formation of myotomes and neuromuscular junctions and led to a swimming defect in the larvae. Immunoprecipitation of the ASC-1 complex consistently copurified cysteine and glycine rich protein 1 (CSRP1), a transcriptional cofactor, which is known to be involved in spinal cord regeneration upon injury in adult zebrafish. ASCC1 mutant fibroblasts downregulated genes associated with neurogenesis, neuronal migration, and pathfinding (SERPINF1, DAB1, SEMA3D, SEMA3A), as well as with bone development (TNFRSF11B, RASSF2, STC1). Our findings indicate that the dysfunction of a transcriptional coactivator complex can result in a clinical syndrome affecting the neuromuscular system

Методы повышения эффективности работы газотурбинных установок

Объект исследования: газотурбинная установка. Цель работы: аналитический обзор современных направлений повышения эффективности работы ГТУ, сравнительный расчет простой и регенеративной ГТУ при заданных равных условиях. Для достижения поставленной цели рассмотрены следующие задачи: проведение литературного обзора современных направлений повышения эффективности работы газотурбинных установок; сравнительный тепловой расчет простой и регенеративной ГТУ при заданных равных условиях; Определение сметной стоимости работ по монтажу регенератора; анализ вредныхThe object of the study: gas turbine plant. The purpose of the work: analytical review of modern directions of increasing the efficiency of GTP operation, comparative calculation of simple and regenerative GTP under given equal conditions. To achieve this goal, the following tasks are considered: conducting a literature review of modern technologies to increase the efficiency of gas turbine plants; comparative heat calculation of simple and regenerative gas turbine plant (efficiency, specific fuel consumption, heat rate) for given equal conditions; determination of the estimated cost of

Разработка технических средств и технологий гравийной обсыпки прифильтровой зоны нефтяных скважин

Борьба с пескопроявлениями в скважинах – процесс, который может потребовать достаточно больших затрат времени и финансов, однако он необходим для правильного функционирования оборудования. Можно выделить два возможных направления решения проблемы: технологические и механические. Технологические методы основаны на изучении механических свойств породы пласта в начальных условиях и их изменений при нарушении равновесного состояния термогидродинамической системы. Механические методы являются наиболее простыми и доступными, поэтому получили наибольшее распространение. К ним относится оборудование нефтяных скважин противопесочными фильтрами различной конструкции.Sand production is undesirable during production of hydrocarbon as it can cause many different problems both topside and downhole. There are several methods available in the industry today to control sand production. In general, sand control methods can be categorized as either mechanical or chemical. The mechanical means hinders formation sand using down-hole filters such as liners, screens or gravel packs

Pan-ethnic carrier screening and prenatal diagnosis for spinal muscular atrophy: clinical laboratory analysis of >72 400 specimens

Spinal muscular atrophy (SMA) is a leading inherited cause of infant death with a reported incidence of ∼1 in 10 000 live births and is second to cystic fibrosis as a common, life-shortening autosomal recessive disorder. The American College of Medical Genetics has recommended population carrier screening for SMA, regardless of race or ethnicity, to facilitate informed reproductive options, although other organizations have cited the need for additional large-scale studies before widespread implementation. We report our data from carrier testing (n=72 453) and prenatal diagnosis (n=121) for this condition. Our analysis of large-scale population carrier screening data (n=68 471) demonstrates the technical feasibility of high throughput testing and provides mutation carrier and allele frequencies at a level of accuracy afforded by large data sets. In our United States pan-ethnic population, the calculated a priori carrier frequency of SMA is 1/54 with a detection rate of 91.2%, and the pan-ethnic disease incidence is calculated to be 1/11 000. Carrier frequency and detection rates provided for six major ethnic groups in the United States range from 1/47 and 94.8% in the Caucasian population to 1/72 and 70.5% in the African American population, respectively. This collective experience can be utilized to facilitate accurate pre- and post-test counseling in the settings of carrier screening and prenatal diagnosis for SMA