Исследование влияния давления в реакторах на процесс каталитического риформинга бензинов

Freeman-Sheldon syndrome is defined as a combination of microstomia, deep set eyes, small palpebral fissures, arthrogryposis with ulnar deviation of the hand, talipes equinovarus and generalized muscular hypertension. Respiratory and swallowing problems are frequently encountered in these patients due to small orifices of mouth and nose. Obstruction of the upper airway tract resulting in tracheostomy has only been described twice. The described child manifested the typical dysmorphic features of Freeman-Sheldon syndrome and suffered from serious respiratory distress and swallowing difficulties from birth. The boy died at the age of 7 months after accidental decannulation of the tracheostoma during sleep. He did not show anatomical or histopathological abnormalities in the pharyngeal, laryngeal or tracheal regions. We assume that the only explanation of the repeated obstructive episodes is a functional muscular obstruction. Copyright © 2002 S. Karger AG, Basel

The Watershed Source Book: Watershed-Based Solutions to Natural Resource Problems

1 v. (various pagings) : maps (2 folded) ; 28 cmhttps://scholar.law.colorado.edu/books_reports_studies/1052/thumbnail.jp

The Watershed Source Book: Watershed-Based Solutions to Natural Resource Problems

1 v. (various pagings) : maps (2 folded) ; 28 cmhttps://scholar.law.colorado.edu/books_reports_studies/1052/thumbnail.jp

Density Profiles in Random Quantum Spin Chains

We consider random transverse-field Ising spin chains and study the magnetization and the energy-density profiles by numerically exact calculations in rather large finite systems ($L\le 128$). Using different boundary conditions (free, fixed and mixed) the numerical data collapse to scaling functions, which are very accurately described by simple analytic expressions. The average magnetization profiles satisfy the Fisher-de Gennes scaling conjecture and the corresponding scaling functions are indistinguishable from those predicted by conformal invariance.Comment: 4 pages RevTeX, 4 eps-figures include

Evidence of mild founder LMOD3 mutations causing nemaline myopathy 10 in Germany and Austria

Objective To expand the clinical and genetic spectrum of nemaline myopathy 10 by a series of Austrian and German patients with a milder disease course and missense mutations in LMOD3. Methods We characterized the clinical features and the genetic status of 4 unrelated adolescent or adult patients with nemaline myopathy. Results The 4 patients showed a relatively mild disease course. They all have survived into adulthood, 3 of 4 have remained ambulatory, and all showed marked facial weakness. Muscle biopsy specimens gave evidence of nemaline bodies. All patients were unrelated but originated from Austria (Tyrol and Upper Austria) and Southern Germany (Bavaria). All patients carried the missense variant c.1648C>T, p.(Leu550Phe) in the LMOD3 gene, either on both alleles or in trans with another missense variant (c.1004A>G, p.Gln335Arg). Both variants were not reported previously. Conclusions In 2014, a severe form of congenital nemaline myopathy caused by disrupting mutations in LMOD3 was identified and denoted as NEM10. Unlike the previously reported patients, who had a severe clinical picture with a substantial risk of early death, our patients showed a relatively mild disease course. As the missense variant c.1648C>T is located further downstream compared to all previously published LMOD3 mutations, it might be associated with higher protein expression compared to the reported loss-of-function mutations. The apparent clusters of 2 mild mutations in Germany and Austria in 4 unrelated families may be explained by a founder effect

Influence of alkaline modification on adsorption properties of aluminum oxide

In this work the adsorption capacity of samples A-2-9С, A-2-Na-9С, A-2-K-9С was studied. It was found that alkaline modification leads to an increase in the efficiency of aluminum oxide dehydrators

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

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