Comparison of estimates calculated on the energy flow problem basis and using testing equations method

The article is devoted to the electrical energy measurements estimates calculation. Automatic Meter Reading systems are the source of measurements. Two methods are compared. The first one is based on the energy flow problem, which allows to receive the estimates of the electrical energy flows and losses on all elements of the electrical network. Calculations using the first method were made using the software product "Balance". The second technique is simplified and uses a system of testing equations to calculate the measurements estimates. Comparison showed the use of a simplified procedure gives adequate accuracy on condition of sufficient redundancy level. © 2018 The Authors, published by EDP Sciences.The reported study was funded by the Ministry of Education and Science of the Russian Federation under Federal Targeted Programme according to the agreement № 14.578.21.0226 (Project identifier: RFMEFI57817X0226)

Testing Equation Method Modification for Demanding Energy Measurements Verification

The paper is devoted to the mathematical approaches of the measurements received from Automatic Meter Reading Systems verification. Reliability of metering data can be improved by application of the new issue named Energy Flow Problem. The paper considers demanding energy measurements verification method based on verification expressions groups analysis. Bad data detection and estimates accuracy calculation is presented using the Automatic Meter Reading system data from the Russian power system fragment. © 2016 The Authors, published by EDP Sciences

Ubiquitin Ligases of the N-End Rule Pathway: Assessment of Mutations in UBR1 That Cause the Johanson-Blizzard Syndrome

Background: Johanson-Blizzard syndrome (JBS; OMIM 243800) is an autosomal recessive disorder that includes congenital exocrine pancreatic insufficiency, facial dysmorphism with the characteristic nasal wing hypoplasia, multiple malformations, and frequent mental retardation. Our previous work has shown that JBS is caused by mutations in human UBR1, which encodes one of the E3 ubiquitin ligases of the N-end rule pathway. The N-end rule relates the regulation of the in vivo half-life of a protein to the identity of its N-terminal residue. One class of degradation signals (degrons) recognized by UBR1 are destabilizing N-terminal residues of protein substrates. Methodology/Principal Findings: Most JBS-causing alterations of UBR1 are nonsense, frameshift or splice-site mutations that abolish UBR1 activity. We report here missense mutations of human UBR1 in patients with milder variants of JBS. These single-residue changes, including a previously reported missense mutation, involve positions in the RING-H2 and UBR domains of UBR1 that are conserved among eukaryotes. Taking advantage of this conservation, we constructed alleles of the yeast Saccharomyces cerevisiae UBR1 that were counterparts of missense JBS-UBR1 alleles. Among these yeast Ubr1 mutants, one of them (H160R) was inactive in yeast-based activity assays, the other one (Q1224E) had a detectable but weak activity, and the third one (V146L) exhibited a decreased but significant activity, in agreement with manifestations of JBS in the corresponding JBS patients. Conclusions/Significance: These results, made possible by modeling defects of a human ubiquitin ligase in its yeast counterpart, verified and confirmed the relevance of specific missense UBR1 alleles to JBS, and suggested that a residual activity of a missense allele is causally associated with milder variants of JBS

Arbeitszeiten mobil Beschäftigter: welche Auswirkungen haben auswärtige Übernachtungen?

The following article presents results of working time surveys in the context of mobile work that have been analysed in the research project prentimo (prevention-oriented organization of mobile work) on the basis of three companies (in two ones by means of qualitative surveys and in one by means of the analysis of concrete working hours). The central question is whether overnight-staying workers and not overnight-staying workers differ in terms of possible impacts of various working time features (duration of daily working hours and resting periods, reliability of the end of shift, weekly working hours as well as weekend work). The focus is hereby on mobile work that is accompanied by spatial mobility that occurs when employees have to travel to a customer’s location and work on site. Mobility is hence a necessary precondition to accomplish the job. As a result, excessive working hours, reduced resting periods, an end of shift that is hard to calculate, increased weekly working hours, travel times, on-call duty and insufficient route planning have emerged as derivative stress factors.Im vorliegenden Beitrag werden Ergebnisse von Arbeitszeiterhebungen bei mobiler Arbeit vorgestellt, die im Rahmen des Forschungsprojektes prentimo (Präventionsorientierte Gestaltung mobiler Arbeit) anhand von drei Unternehmen (in zwei Unternehmen durch qualitative Erhebungen, in einem durch die Analyse von konkreten Arbeitszeiten) analysiert wurden. Im Fokus steht dabei mobiler Arbeit, die mit räumlicher Mobilität einhergeht. Mobilität ist zur Erledigung der Arbeitsaufgabe notwendig, wenn beispielsweise direkt beim Kunden gearbeitet wird. Zentrale Fragestellung des Beitrages ist, ob sich bei ÜbernachterInnen bzw. und Nicht-ÜbernachterInnen unterschiedliche Auswirkungen hinsichtlich verschiedener Arbeitszeitmerkmale (Dauer der täglichen Arbeits- und Ruhezeiten, Verlässlichkeit des Arbeitsendes, Pausen, Wochenarbeitszeiten sowie Wochenendarbeit) ergeben. Als differenzierende Belastungsfaktoren zeigen sich überlange tägliche Arbeitszeiten, verkürzte Ruhezeiten, ein schwer kalkulierbares Arbeitsende, eine erhöhte Wochenarbeitszeit, Reisezeiten, Rufbereitschaften sowie eine unzureichende Routenplanung

СТЕПЕНЬ КОЛОНИЗАЦИИ ПАТОГЕННЫМИ И УСЛОВНО-ПАТОГЕННЫМИ МИКРООРГАНИЗМАМИ КАК ПРОГНОСТИЧЕСКИЙ ФАКТОР РАЗВИТИЯ СЕПСИСА

The development of sepsis in newborns with congenital malformations of the gastrointestinal tract in the postoperative period depends on the colonization of various biotopes. The starting etiological factors are: the presence of opportunistic and pathogenic microflora in certain biotopes, as well as a quantitative content of microorganisms (the “degree of microbial load”). It has been established that colonization of biotopes with a quantitative assessment of CFU of 104 /g (colony forming unit) and more is one of the factors of the sepsis development in newborns with this pathology. Развитие сепсиса у новорожденных с врожденными пороками развития желудочно-кишечного тракта в послеоперационном периоде зависит от колонизации различных биотопов. Пусковыми этиологическими факторами являются наличие в определенных биотопах условно-патогенной и патогенной микрофлоры, а также количественное содержание микроорганизмов («степень микробной нагрузки»). Установлено, что колонизация биотопов с количественной оценкой КОЕ 104 /г (колоний образующая единица) и более является одним из факторов развития сепсиса у новорожденных с данной патологией.

Haploinsufficiency of the NOTCH1 Receptor as a Cause of Adams-Oliver Syndrome With Variable Cardiac Anomalies.

BACKGROUND: Adams-Oliver syndrome (AOS) is a rare disorder characterized by congenital limb defects and scalp cutis aplasia. In a proportion of cases, notable cardiac involvement is also apparent. Despite recent advances in the understanding of the genetic basis of AOS, for the majority of affected subjects, the underlying molecular defect remains unresolved. This study aimed to identify novel genetic determinants of AOS. METHODS AND RESULTS: Whole-exome sequencing was performed for 12 probands, each with a clinical diagnosis of AOS. Analyses led to the identification of novel heterozygous truncating NOTCH1 mutations (c.1649dupA and c.6049_6050delTC) in 2 kindreds in which AOS was segregating as an autosomal dominant trait. Screening a cohort of 52 unrelated AOS subjects, we detected 8 additional unique NOTCH1 mutations, including 3 de novo amino acid substitutions, all within the ligand-binding domain. Congenital heart anomalies were noted in 47% (8/17) of NOTCH1-positive probands and affected family members. In leukocyte-derived RNA from subjects harboring NOTCH1 extracellular domain mutations, we observed significant reduction of NOTCH1 expression, suggesting instability and degradation of mutant mRNA transcripts by the cellular machinery. Transient transfection of mutagenized NOTCH1 missense constructs also revealed significant reduction in gene expression. Mutant NOTCH1 expression was associated with downregulation of the Notch target genes HEY1 and HES1, indicating that NOTCH1-related AOS arises through dysregulation of the Notch signaling pathway. CONCLUSIONS: These findings highlight a key role for NOTCH1 across a range of developmental anomalies that include cardiac defects and implicate NOTCH1 haploinsufficiency as a likely molecular mechanism for this group of disorders

DOCK6 Mutations Are Responsible for a Distinct Autosomal-Recessive Variant of Adams-Oliver Syndrome Associated with Brain and Eye Anomalies

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