Psychopathology in Huntington's disease

Dit proefschrift begint met een overzichtsartikel van oorspronkelijke onderzoek naar psychopathologie bij mutatiedragers voor de ziekte van Huntington. Aansluitend worden de resultaten van een cohortstudie naar de aanwezigheid en ernst van psychopathologie bij mensen met de ziekte van Huntington in verschillende ziektestadia en bij eerstegraads niet-dragers beschreven.UBL - phd migration 201

Оцінювання закону розподілу величини збитків унаслідок реалізації загрози “Відсутність або недостатність технічного обслуговування”

The problem of estimation of size distribution law of damages in the absence or insufficient sample size of the universe and the initial data suggested approach to finding the most expected value of losses due to information security threats

Crystal Structure of 3-Hydroxybenzoate 6-Hydroxylase Uncovers Lipid-assisted Flavoprotein Strategy for Regioselective Aromatic Hydroxylation.

3-Hydroxybenzoate 6-hydroxylase (3HB6H) from Rhodococcus jostii RHA1 is a dimeric flavoprotein that catalyzes the NADH- and oxygen-dependent para-hydroxylation of 3-hydroxybenzoate to 2,5-dihydroxybenzoate. In this study, we report the crystal structure of 3HB6H as expressed in Escherichia coli. The overall fold of 3HB6H is similar to that of p-hydroxybenzoate hydroxylase and other flavoprotein aromatic hydroxylases. Unexpectedly, a lipid ligand is bound to each 3HB6H monomer. Mass spectral analysis identified the ligand as a mixture of phosphatidylglycerol and phosphatidylethanolamine. The fatty acid chains occupy hydrophobic channels that deeply penetrate into the interior of the substrate-binding domain of each subunit, whereas the hydrophilic part is exposed on the protein surface, connecting the dimerization domains via a few interactions. Most remarkably, the terminal part of a phospholipid acyl chain is directly involved in the substrate-binding site. Co-crystallized chloride ion and the crystal structure of the H213S variant with bound 3-hydroxybenzoate provide hints about oxygen activation and substrate hydroxylation. Essential roles are played by His-213 in catalysis and Tyr-105 in substrate binding. This phospholipid-assisted strategy to control regioselective aromatic hydroxylation is of relevance for optimization of flavin-dependent biocatalysts

THE EUROPEAN PHYSICAL JOURNAL D High-resolution spectroscopy on ground-state transitions of samarium-I

Abstract. A high resolution laser-induced fluorescence study has been performed on thirteen ground-state transitions in samarium, using a frequency-doubled Ti:sapphire laser in the wavelength range 350-440 nm. From each spectrum isotope shifts and hyperfine structure constants A and B are derived. The results are used to determine the nuclear parameter λ AA . PACS. 32.30.Jc Visible and ultraviolet spectra -32.10.Fn Fine and hyperfine structure -32.70.Jz Line shapes, widths, and shift

A review on equipment protection and system protection relay in power system

Power system equipment is configured and connected together with multiple voltage levels in existing electrical power system. There are varieties of electrical equipment obtainable in the power system predominantly from generation side up to the distribution side. Consequently, appropriate protections must be apt to prevent inessential disturbances that lead to voltage instability, voltage collapse and sooner a total blackout took place in the power system. The understanding of each component on the system protection is critical. This is due to any abnormal condition and failure can be analyzed and solved effectively due to the rapid changing and development on the power system network. Therefore, the enhancement of power quality can be achieved by sheltering the equipment with protection relay in power system. Moreover, the design of a systematic network is crucial for the system protection itself. Several types of protective equipment and protection techniques are taken into consideration in this paper. Hence, the existing accessible types and methods of system protection in the power system network are reviewed

Modeling Life as Cognitive Info-Computation

This article presents a naturalist approach to cognition understood as a network of info-computational, autopoietic processes in living systems. It provides a conceptual framework for the unified view of cognition as evolved from the simplest to the most complex organisms, based on new empirical and theoretical results. It addresses three fundamental questions: what cognition is, how cognition works and what cognition does at different levels of complexity of living organisms. By explicating the info-computational character of cognition, its evolution, agent-dependency and generative mechanisms we can better understand its life-sustaining and life-propagating role. The info-computational approach contributes to rethinking cognition as a process of natural computation in living beings that can be applied for cognitive computation in artificial systems.Comment: Manuscript submitted to Computability in Europe CiE 201

Incidence of completed suicide and suicide attempts in a global prospective study of Huntington's disease

Background Risk of death from suicide in Huntington's disease is notably elevated relative to that in the general population, although the incidence within HD populations has not been precisely defined. Robust incidence estimates of suicidal behavior can serve as references for HD therapeutic research and post-marketing surveillance to help evaluate the suicidality risk of novel therapeutics. Aims To estimate the incidence rate of completed suicide and suicide attempt in the global, prospective HD cohort study Enroll-HD that records these events per protocol. Method A total of 20 912 participants were available for analysis (HD gene-expansion carriers (HDGECs) n = 15 924; non-HDGECs n = 4988) representing a collective observation period of 53 390 participant-years. Each observed event was subject to clinical review and evaluation. We generated incidence rates (events per 100 000 person-years) for suicides and suicide attempts using all available data, as well as by year of study and geographical region. Proportionate mortality statistics for suicide and respective 95% confidence intervals were also generated. Results The overall incidence rate of suicide in HDGECs was 72 per 100 000 person-years, and 8 per 100 000 person-years in non-HDGECs. Proportionate mortality attributable to suicide in HDGECs was 4.6%. For suicide attempts, the global overall incidence rate observed in HDGECs was 306-375 per 100 000 person-years, and 23-38 per 100 000 person-years in non-HDGECs. Conclusions The incidence estimates calculated here can be used as a reference to help evaluate drug safety and may also be useful in assessing progress in clinical care for HDGECs once therapeutic interventions become widely available.Stress-related psychiatric disorders across the life spa

Factors of specialized mental health care use in the Netherlands: a scoping review applying Andersen-Newman's care utilization model

As mental disorders impact quality of life and result in high costs for society, it is important patients receive timely and adequate care. This scoping review first aims to summarize which factors contribute to specialized mental health care (SMHC) use. Within the Dutch health care system, the general practitioner (GP) is the filter for SMHC and care use costs are relatively low. Second, to organize factors by Andersen and Newman's care utilization model in illness level, predisposing, and enabling factors. Third, to assess equity of access to SMHC in the Netherlands. A health care system is equitable when illness level and the demographic predisposing factors age and gender account for most variation in care use and inequitable when enabling factors and social predisposing factors such as education predominate. We identified 13 cross-sectional and cohort studies in the Netherlands published between 1970 and September 2020 with 20 assessed factors. Illness level factors, disease severity, diagnosis, personality, and comorbidity contributed the most to SMHC use. Predisposing factors related to a more solitary lifestyle contributed to a lesser degree. Enabling factors income and urbanicity contributed the least to SMHC use. These results imply inequity. Factors that did not fit the care utilization model were GP related, for example the ability to recognize mental disorders. This emphasizes their importance in a system where patients are dependent on GPs for access to SMHC. Focus should be on improving recognition of mental disorders by GPs as well as collaboration with mental health care professionals.Pathways through Adolescenc