TMEM106B Acts as a Modifier of Cognitive and Motor Functions in Amyotrophic Lateral Sclerosis

The transmembrane protein 106B (TMEM106B) gene is a susceptibility factor and disease modifier of frontotemporal dementia, but few studies have investigated its role in amyotrophic lateral sclerosis. The aim of this work was to assess the impact of the TMEM106B rs1990622 (A–major risk allele; G–minor allele) on phenotypic variability of 865 patients with amyotrophic lateral sclerosis. Demographic and clinical features were compared according to genotypes by additive, dominant, and recessive genetic models. Bulbar onset was overrepresented among carriers of the AA risk genotype, together with enhanced upper motor neuron involvement and poorer functional status in patients harboring at least one major risk allele (A). In a subset of 195 patients, we found that the homozygotes for the minor allele (GG) showed lower scores at the Edinburgh Cognitive and Behavioral Amyotrophic Lateral Sclerosis Screen, indicating a more severe cognitive impairment, mainly involving the amyotrophic lateral sclerosis-specific cognitive functions and memory. Moreover, lower motor neuron burden predominated among patients with at least one minor allele (G). Overall, we found that TMEM106B is a disease modifier of amyotrophic lateral sclerosis, whose phenotypic effects encompass both sites of onset and functional status (major risk allele), motor functions (both major risk and minor alleles), and cognition (minor allele).     

A systematic review of the potential hurdles of interoperability to the emergency services in major incidents: recommendations for solutions and alternatives

This study presents a narrative synthesis of a systematic literature review relating to multi-agency interoperability and major incident decision-making in high risk, high stake environments. The review methodology includes the identification of relevant studies, a critical appraisal of the concepts inherent in the main review question and a narrative synthesis of the central themes that relate to the study as a whole. The review firstly outlines what, currently, appear to be the perceived defining features of successful interoperability by using the SAFE-T phase model of major incident decision-making. It then considers whether these defining features are realistically achievable in major incident practice. Findings suggest that the current definition of an interoperable network is too demanding for the inherent complexity and dynamic nature of the major incident task environment. Individual teams tend to focus on agency-specific behaviour, as opposed to coordinated multi-team functioning, and so collective interoperability is not achieved. Inevitably, this reduces the ability to perform collaborative behaviours, including decision-making and action implementation. The paper concludes that aiming for the current conceptualisation of interoperability along a hierarchical command structure may actually inhibit effective decision-making. Instead, multi-agency systems would do better to work towards an improved understanding of a non-hierarchical and decentralised yet interoperable major incident management network. Recommendations include the need to relate theory and practice in the development of multi-agency decision-making via simulation-based training and to deepen our understanding of interoperability to prevent inertia in high risk, high stake major incident environments.     

Comparative structural and immunochemical properties of leghaemoglobins

Circular dichroism studies on leghaemoglobins from snake bean, lupin, serradella and other plants show that, in common with soybean (reported earlier) they have a similar overall polypeptide chain conformation and haem environment and orientation. Immunochemical studies, on the other hand, suggest that the antigenic determinants on the surface of the leghaemoglobins vary considerably. Thus, firstly the alpha-helix content of the leghaemoglobins as a class is very similar (60-65%) and approaches that of the myoglobins, secondly, the sign, magnitude and shape of their circular dichroism spectra in the near ultraviolet, Soret and visible regions suggest close similarities in the environment and orientation of a structurally important tryptophan residue and of the haem moiety, and thirdly, there is comparatively weak haem-protein interaction. The extent of immuno cross-reactivity was found to be best deomonstrated using the Farr radioimmunoassay procedure. The results were (a) 5 leghaemoglobins from one plant (soybean) crossreacted completely but with varying affinities. (b) The extent of cross reactivity between leghaemoglobins from different plants was compared to that within a single plant; the reaction of antiserum to a soybean leghaemoglobin with a serradella leghaemoglobin was weak, with a snake bean leghaemoglobin still weaker (and incomplete) while lupin leghaemoglobins showed no cross reactivity at all. (c) The "rapid" attenuation of cross reactivity among different plant leghaemoglobins is explicable in terms of the extensive amino acid substitutions which have been demonstrated in the literature and in the present studies. (d) In view of this rapid divergence it is not surprising that sperm whale and horse heart myoglobins showed no cross reactivity with soybean leghaemoglobins. In summary, amino acid substitutions in the leghaemoglobin family are conformationally but not immunochemically conservative.     

Coder gender and potential for hostility ratings.

This study examined the effects of coder gender on Potential for Hostility ratings. Six trained coders (3 men and 3 women) who were unaware of the coder gender effect hypothesis coded 30 male and 30 female undergraduates for Potential for Hostility. Although reliability estimates as calculated by Cronbach's alpha suggested that all coders were consistent, an analysis of variance revealed a significant main effect for coder gender, wherein female coders rated participants as displaying significantly less Potential for Hostility than did male coders. This significant difference was also meaningful, as coder gender accounted for 32% of the variance in Potential for Hostility scores. Thus, future Potential for Hostility investigations need to consider the gender of those coding, as this factor both significantly and substantially influences reported Potential for Hostility ratings. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Feedback controllability assessment and control of particle size distribution in emulsion polymerisation

In this article, the importance of particle size distribution (PSD) control as a means for the inferential control of the rheology of emulsion polymers is illustrated. A controllability assessment is presented to illustrate the attainability or otherwise of bimodal PSD using feedback control through a consideration of the process mechanisms—measurement limitations and process constraints that prevent the implementation of feedback corrections. The suitability of a batch-to-batch iterative feedback PSD control is demonstrated, which could act in addition to any in-batch feedback control, the latter being less feasible in certain cases, as argued in this article. A detailed population balance model is used for the batch-to-batch control, which simplifies model update and feedback correction.     

Intentionality and knowledge in children's judgments of actor's responsibility and recipient's emotional reaction.

Two conflicting developmental accounts of how mental states are used in evaluating actors are tested by varying actors' intentionality, foreknowledge of outcome, and the values of motive and outcome. In Experiment 1, children judged a recipient's emotional reaction to three types of event: intended outcome, foreseen accident, and unforseen accident. Both 6- and 7-year-olds used intentionality and knowledge in their judgments of good and bad outcomes. Three-year-olds did not distinguish between accidents differing in actors' foreknowledge, but discriminated between intended and accidental outcomes when the accident was unforseen. In Experiment 2, children judged actor's responsibility for accidentally caused bad outcomes. Seven-year-olds, but not 5-year-olds, blamed actors for foreseen accidents more than for unforeseen accidents regardless of motive value. The results suggest that children use intentionality before knowledge in judgments of action sequences, and that actor's foreknowledge of an outcome influences children's ability to judge the intended/accidental distinction. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

A discussion of the forward and backward Kolmogorov equations for multiplying systems

A parallel derivation is formulated of the forward Kolmogorov equation (FKE) and of the backward Kolmogorov equation (BKE) from detailed probability balance equations for a population of neutrons, precursors and detected neutrons living in a multiplying medium where processes of capture and leakage, fission, precursor decay, detection and source emission occur.The FKE and BKE are transformed into equations for the generating function of the related state probabilities. The work is characterized by the exposition of several mathematical details and implications. The material has been collected with the purpose of facilitating access and understanding.