An item response analysis of the motor and behavioral subscales of the unified Huntington's disease rating scale in huntington disease gene expansion carriers

Although the Unified Huntington's Disease Rating Scale (UHDRS) is widely used in the assessment of Huntington disease (HD), the ability of individual items to discriminate individual differences in motor or behavioral manifestations has not been extensively studied in HD gene expansion carriers without a motor-defined clinical diagnosis (ie, prodromal-HD or prHD). To elucidate the relationship between scores on individual motor and behavioral UHDRS items and total score for each subscale, a nonparametric item response analysis was performed on retrospective data from 2 multicenter longitudinal studies. Motor and behavioral assessments were supplied for 737 prHD individuals with data from 2114 visits (PREDICT-HD) and 686 HD individuals with data from 1482 visits (REGISTRY). Option characteristic curves were generated for UHDRS subscale items in relation to their subscale score. In prHD, overall severity of motor signs was low, and participants had scores of 2 or above on very few items. In HD, motor items that assessed ocular pursuit, saccade initiation, finger tapping, tandem walking, and to a lesser extent, saccade velocity, dysarthria, tongue protrusion, pronation/supination, Luria, bradykinesia, choreas, gait, and balance on the retropulsion test were found to discriminate individual differences across a broad range of motor severity. In prHD, depressed mood, anxiety, and irritable behavior demonstrated good discriminative properties. In HD, depressed mood demonstrated a good relationship with the overall behavioral score. These data suggest that at least some UHDRS items appear to have utility across a broad range of severity, although many items demonstrate problematic features.

Ethylene and senescence processes

Senescence is a vitally important sequence of events in the latter phase of the life cycle of a plant that determines yield and reproductive success. In many species, and in different plant organs, ethylene is a key regulator of senescence and an increased understanding of the way the hormone functions will enable the timing and location of senescence to be manipulated in order to improve yield, quality and longevity. This chapter examines the physiological and molecular regulation of senescence in different plant organs and introduces the concept of the ‘senescence window’ in which plant organs are receptive to ethylene-mediated senescence cues. Several studies have attempted to elucidate global patterns of the regulation of senescence, which have enabled the function of ethylene to be placed in the context of the involvement of other, often antagonistic, hormones in the execution of senescence and downstream processes. Finally, we examine the consequences of senescence for post-harvest biology, an area where the control of ethylene action has been actively sought in order to control precisely the timing of senescence and ripening processes so that crop quality can be enhanced and maintained.

Economic and political influences on urban planning: a comparison of London, Paris and Berlin

This paper addressed the degree of autonomy experienced by the planning regimes of London, Paris and Berlin. What variation exists in the governance of these cities and how do national, local, political, business and community interests effect planning decisions? The discussion is placed in the context of the literature on world cities and growth coalitions and the debate over whether economic forces compel cities to follow similar strategies. The paper concludes that in the case of the three cities examined there is considerable variation of planning approach due to different historical, cultural and political factors.

Evoked prior learning experience and approach to learning as predictors of academic achievement

Background. In separate studies and research from different perspectives, five factors are found to be among those related to higher quality outcomes of student learning (academic achievement). Those factors are higher self-efficacy, deeper approaches to learning, higher quality teaching, students’ perceptions that their workload is appropriate, and greater learning motivation. University learning improvement strategies have been built on these research results. Aim. To investigate how students’ evoked prior experience, perceptions of their learning environment, and their approaches to learning collectively contribute to academic achievement. This is the first study to investigate motivation and self-efficacy in the same educational context as conceptions of learning, approaches to learning and perceptions of the learning environment. Sample. Undergraduate students (773) from the full range of disciplines were part of a group of over 2,300 students who volunteered to complete a survey of their learning experience. On completing their degrees 6 and 18 months later, their academic achievement was matched with their learning experience survey data. Method. A 77-item questionnaire was used to gather students’ self-report of their evoked prior experience (self-efficacy, learning motivation, and conceptions of learning), perceptions of learning context (teaching quality and appropriate workload), and approaches to learning (deep and surface). Academic achievement was measured using the English honours degree classification system. Analyses were conducted using correlational and multi-variable (structural equation modelling) methods. Results. The results from the correlation methods confirmed those found in numerous earlier studies. The results from the multi-variable analyses indicated that surface approach to learning was the strongest predictor of academic achievement, with self-efficacy and motivation also found to be directly related. In contrast to the correlation results, a deep approach to learning was not related to academic achievement, and teaching quality and conceptions of learning were only indirectly related to achievement. Conclusions. Research aimed at understanding how students experience their learning environment and how that experience relates to the quality of their learning needs to be conducted using a wider range of variables and more sophisticated analytical methods. In this study of one context, some of the relations found in earlier bivariate studies, and on which learning intervention strategies have been built, are not confirmed when more holistic teaching–learning contexts are analysed using multi-variable methods.

Online customer experience in e-retailing: an empirical model of antecedents and outcomes

The theoretical understanding of online shopping behavior has received much attention. Less focus has been given to the formation of the customer experience (CE) that results from online shopper interactions with e-retailers. This study develops and empirically tests a model of the relationship between antecedents and outcomes of online customer experience (OCE) within Internet shopping websites using an international sample. The study identifies and provides operational measures of these variables plus the cognitive and affective components of OCE. The paper makes contributions towards new knowledge and understanding of how e-

Gap junctions and connexin hemichannels underpin hemostasis and thrombosis

BACKGROUND: Connexins are a widespread family of membrane proteins that assemble into hexameric hemichannels, also known as connexons. Connexons regulate membrane permeability in individual cells or couple between adjacent cells to form gap junctions and thereby provide a pathway for regulated intercellular communication. We have now examined the role of connexins in platelets, blood cells that circulate in isolation, but upon tissue injury adhere to each other and the vessel wall to prevent blood loss and facilitate wound repair. METHODS AND RESULTS: We report the presence of connexins in platelets, notably connexin37, and that the formation of gap junctions within platelet thrombi is required for the control of clot retraction. Inhibition of connexin function modulated a range of platelet functional responses prior to platelet-platelet contact, and reduced laser induced thrombosis in vivo in mice. Deletion of the Cx37 gene (Gja4) in transgenic mice reduced platelet aggregation, fibrinogen binding, granule secretion and clot retraction indicating an important role for Cx37 hemichannels and gap junctions in platelet thrombus function. CONCLUSIONS: Together, these data demonstrate that platelet gap junctions and hemichannels underpin the control of haemostasis and thrombosis and represent potential therapeutic targets.

Physical interpretation of the spectral radiative signature in the transition zone between cloud-free and cloudy regions

One-second-resolution zenith radiance measure- ments from the Atmospheric Radiation Measurement pro- gram’s new shortwave spectrometer (SWS) provide a unique opportunity to analyze the transition zone between cloudy and cloud-free air, which has considerable bearing on the aerosol indirect effect. In the transition zone, we find a re- markable linear relationship between the sum and difference of radiances at 870 and 1640 nm wavelengths. The intercept of the relationship is determined primarily by aerosol prop- erties, and the slope by cloud properties. We then show that this linearity can be predicted from simple theoretical con- siderations and furthermore that it supports the hypothesis of inhomogeneous mixing, whereby optical depth increases as a cloud is approached but the effective drop size remains un- changed.

Co-variation of temperature and precipitation in CMIP5 models and satellite observations

Current variability of precipitation (P) and its response to surface temperature (T) are analysed using coupled(CMIP5) and atmosphere-only (AMIP5) climate model simulations and compared with observational estimates. There is striking agreement between Global Precipitation Climatology Project (GPCP) observed and AMIP5 simulated P anomalies over land both globally and in the tropics suggesting that prescribed sea surface temperature and realistic radiative forcings are sufficient for simulating the interannual variability in continental P. Differences between the observed and simulated P variability over the ocean, originate primarily from the wet tropical regions, in particular the western Pacific, but are reduced slightly after 1995. All datasets show positive responses of P to T globally of around 2 %/K for simulations and 3-4 %/K in GPCP observations but model responses over the tropical oceans are around 3 times smaller than GPCP over the period 1988-2005. The observed anticorrelation between land and ocean P, linked with El Niño Southern Oscillation, is captured by the simulations. All data sets over the tropical ocean show a tendency for wet regions to become wetter and dry regions drier with warming. Over the wet region (75% precipitation percentile), the precipitation response is ~13-15%/K for GPCP and ~5%/K for models while trends in P are 2.4%/decade for GPCP, 0.6% /decade for CMIP5 and 0.9%/decade for AMIP5 suggesting that models are underestimating the precipitation responses or a deficiency exists in the satellite datasets.

Bells and whistles of convection parameterization

The present workshop constitutes the 5th in the annual series on “Concepts for Convective Parameterizations in Large-Scale Models”. The purpose of the workshop series has been to discuss the fundamental theoretical issues of convection parameterization with a small number of European scientists. The workshop series has been funded by European Cooperation in the Field of Scientific and Technical Research (COST) Action ES0905. The theme of the workshop for the year 2012 was decided from a main conclusion of the previous workshop, which focused on the convective organization problem, seeking a means for implementing such effects into convection parameterizations (Yano et al. 2012). As it turned out, in order to discuss this implementation issue in any concrete manner, we have first to know very well the bells and whistles of convection parameterizations. This was the purpose of the 5th workshop. The title of the workshop is rather metaphorically tagged as “Bulk or Spectrum?”, because this is a typical decision we have to face at the outset of any parameterization development. The following report discusses selected issues of bells and whistles addressed during the meeting.

EEG activation differences in the pre-motor cortex and supplementary motor area between normal individuals with high and low traits of autism

The human mirror neuron system (hMNS) is believed to provide a basic mechanism for social cognition. Event-related desynchronization (ERD) in alpha (8–12 Hz) and low beta band (12–20 Hz) over sensori-motor cortex has been suggested to index mirror neurons' activity. We tested whether autistic traits revealed by high and low scores on the Autistic Quotient (AQ) in the normal population are linked to variations in the electroencephalogram (EEG) over motor, pre-motor cortex and supplementary motor area (SMA) during action observation. Results revealed that in the low AQ group, the pre-motor cortex and SMA were more active during hand action than static hand observation whereas in the high AQ group the same areas were active both during static and hand action observation. In fact participants with high traits of autism showed greater low beta ERD while observing the static hand than those with low traits and this low beta ERD was not significantly different when they watched hand actions. Over primary motor cortex, the classical alpha and low beta ERD during hand actions relative to static hand observation was found across all participants. These findings suggest that the observation–execution matching system works differently according to the degree of autism traits in the normal population and that this is differentiated in terms of the EEG according to scalp site and bandwidth.

Inner plasma structure of the low-latitude reconnection layer

We report a clear transition through a reconnection layer at the low-latitude magnetopause which shows a complete traversal across all reconnected field lines during northwestward interplanetary magnetic field (IMF) conditions. The associated plasma populations confirm details of the electron and ion mixing and the time history and acceleration through the current layer. This case has low magnetic shear with a strong guide field and the reconnection layer contains a single density depletion layer on the magnetosheath side which we suggest results from nearly field-aligned magnetosheath flows. Within the reconnection boundary layer, there are two plasma boundaries, close to the inferred separatrices on the magnetosphere and magnetosheath sides (Ssp and Ssh) and two boundaries associated with the Alfvén waves (or Rotational Discontinuities, RDsp and RDsh). The data are consistent with these being launched from the reconnection site and the plasma distributions are well ordered and suggestive of the time elapsed since reconnection of the field lines observed. In each sub-layer between the boundaries the plasma distribution is different and is centered around the current sheet, responsible for magnetosheath acceleration. We show evidence for a velocity dispersion effect in the electron anisotropy that is consistent with the time elapsed since reconnection. In addition, new evidence is presented for the occurrence of partial reflection of magnetosheath electrons at the magnetopause current layer.