bluuebunny/crossref_metadata_2025_split huggingface dataset:
Restoring assembly and activity of cystathionine β‐synthase mutants by ligands and chemical chaperones
Authors: Kopecká Jana , Krijt Jakub , Raková Kateřina , Kožich Viktor 
Published: May 2010

AbstractMisfolding and aggregation of mutant enzymes have been proposed to play role in the pathogenesis of homocystinuria due to cystathionine β‐synthase (CBS) deficiency. Chemical chaperones have been recently shown to facilitate proper assembly of several CBS mutants. To asses the number of patients that may respond to chaperone therapy, we examined the effect of selected CBS ligands and osmolytes on assembly and activity of 27 CBS mutants that represent 70% of known CBS alleles. The mutant enzymes were expressed in a bacterial system, and their properties were assessed by native Western blotting and sensitive liquid chromatography tandem mass spectrometry (LC‐MS/MS) assay, respectively. We studied the chaperoning activity of δ‐aminolevulinic acid (δ‐ALA)—a heme precursor—and of three osmolytes betaine, 2‐aminoethanesulfonic acid (taurine), and glycerol. Fourteen mutants responded by at least 30% increase in the amount of correctly assembled tetramers and enzymatic activity to the coexpressional presence of either 0.5 mM δ‐ALA, 100 mM betaine, and/or 750 mM glycerol. Eight of these mutants (p.R266K, p.P49L, p.R125Q, p.K102N, p.R369C, p.V180A, p.P78R, p.S466L) were rescuable by all of these three substances. Four mutants showed increased formation of tetramers that was not accompanied by changes in activity. Topology of mutations appeared to determine the chaperone responsiveness, as 11 of 14 solvent‐exposed mutations were substantially more responsive than three of 13 buried mutations. This study identified chaperone‐responsive mutants that represent 56 of 713 known patient‐derived CBS alleles and may serve as a basis for exploring pharmacological approaches aimed at correcting misfolding in homocystinuria.
The birth prevalence of lysosomal storage disorders in the Czech Republic: comparison with data in different populations
Authors: Poupětová Helena , Ledvinová Jana , Berná Linda , Dvořáková Lenka , Kožich Viktor , Elleder Milan 
Published: May 2010

AbstractThe aim of this retrospective study was to determine the prevalence of lysosomal storage disorders (LSDs) in the Czech Republic. The data on cases diagnosed between 1975 and 2008 were collected and analyzed. The overall prevalence of LSDs in the Czech population (12.25 per 100,000) is comparable to that reported for the countries with well‐established and advanced diagnostics of LSDs such as the Netherlands (14 per 100,000), Australia (12.9 per 100,000) and Italy (12.1 per 100,000). Relatively higher prevalence of LSDs was reported in the north of Portugal (25 per 100,000). Thirty‐four different LSDs were diagnosed in a total of 478 individuals. Gaucher disease was the most frequent LSD with a birth prevalence of 1.13 per 100,000 births. The most frequent LSD groups were lipidoses, mucopolysaccharidoses, and neuronal ceroid lipofuscinoses, with combined prevalences of 5.0, 3.72, and 2.29 per 100,000 live births, respectively. Glycoproteinoses (0.57 per 100,000 live births), glycogenosis type II (0.37), and mucolipidoses (0.31) rarely occur in the Czech population, and a range of other LSDs have not been detected at all over the past three decades. Knowledge of the birth prevalence and carrier frequency of particular disorders is important in genetic counselling for calculation of the risk for the disorder in the other members of affected families. Earlier diagnosis of these disorders will permit timely intervention and may also result in lowering of the number of newborns with LSDs.
Clone lineages of grape phylloxera differ in their performance onVitis vinifera
Authors: Herbert K.S. , Umina P.A. , Mitrovski P.J. , Powell K.S. , Viduka K. , Hoffmann A.A. 
Published: May 2010

AbstractGrape phylloxera,Daktulosphaira vitifoliaeFitch, is an important pest of grapevines (Vitis viniferaL.) (Vitaceae). The distribution and frequency of phylloxera clone lineages vary within infested regions of Australia, suggesting the introduction of separate lineages ofD. vitifoliaewith host associations. Virulence levels of particular phylloxera clones may vary onV. vinifera, but much of this evidence is indirect. In this study, we directly tested the performance of phylloxera clones onV. viniferausing an established excised root assay and a new glasshouse vine assessment. In the root assay, grape phylloxera clones differed in egg production and egg to adult survivorship. In the vine assay, clones differed in the number of immature and adult life stages on roots. In addition vine characteristics, including mean stem weight, root weight, leaf chlorophyll and leaf area, were affected by different phylloxera clones. The two most widespread clones displayed high levels of virulence. These results point to only some phylloxera clones being highly virulent onV. vinifera, helping to explain patterns of field damage, phylloxera distributions and continued survival and production ofV. viniferavines in some infested areas.
‘The Kenyas we don't want’: popular thought over constitutional review in Kenya, 2002
Authors: Diepeveen Stephanie 
Published: May 2010

ABSTRACTEvidence of political instability in Kenya emerged through the organised and spontaneous violence following the 2007 elections. Understanding the underlying reasons for popular contestations of power and involvement in violence depends on understanding Kenyans' perceptions of existing and desirable political institutions. By analysing recommendations made in the Constitution of Kenya Review Commission public hearings in each province in 2002, this article explores the viewpoints of those outside high politics in Kenya, teasing out the continuities and divergences in popular thought about political relations in Kenya at the end of the Moi years, and thus providing insights into how Kenyans understand their relationship with those in power, revealing that amidst diverse experiences, disillusionment pervades Kenyans' perceptions of political relations.
The chief, the youth and the plantation: communal politics in southern Nigeria
Authors: von Hellermann Pauline 
Published: May 2010

ABSTRACTIn August 2006 the chief of Udo, a small town in Edo State, Nigeria, was deposed and the town taken over by the ‘youth’. This event presents the classic fall of a ‘big man’ who had lost support, but also involved long-standing chieftaincy rivalries, electoral competition in the run up to the 2007 elections, and conflict over a nearby oil palm and rubber plantation. Through an examination of Udo's crisis, this paper engages with three key questions concerning contemporary communal politics in southern Nigeria: the manifestations of patrimonial power and resistance to it; the meaning and role of ‘youth’; and the impact of expatriate capital.
Dust ion-acoustic solitary waves in a magnetized dusty electronegative plasma
Authors: ANOWAR M. G. M. , ASHRAFI K. S. , MAMUN A. A. 
Published: May 2010

AbstractThe basic features of obliquely propagating dust ion-acoustic (DIA) solitary waves in an adiabatic magnetized dusty electronegative plasma (containing Boltzmann electrons, Boltzmann negative ions, adiabatic positive ions, and negatively charged stationary dust) have been investigated. The reductive perturbation method has been employed to derive the Korteweg–de Vries (KdV) equation which admits a solitary wave solution. The combined effects of ion adiabaticity and external magnetic field (obliqueness), which are found to significantly modify the basic features of the small but finite-amplitude DIA solitary waves, are explicitly examined. The implications of our results in space and laboratory dusty plasmas are briefly discussed.
Adolescent development of psychosis as an outcome of hearing impairment: a 10-year longitudinal study
Authors: van der Werf M. , Thewissen V. , Dominguez M. D. , Lieb R. , Wittchen H. , van Os J. 
Published: May 2010

BackgroundIt has long been acknowledged that hearing impairment may increase the risk for psychotic experiences. Recent work suggests that young people in particular may be at risk, indicating a possible developmental mechanism.MethodThe hypothesis that individuals exposed to hearing impairment in early adolescence would display the highest risk for psychotic symptoms was examined in a prospective cohort study of a population sample of originally 3021 adolescents and young adults aged 14–24 years at baseline, in Munich, Germany (Early Developmental Stages of Psychopathology Study). The expression of psychosis was assessed at multiple time points over a period of up to 10 years, using a diagnostic interview (Munich Composite International Diagnostic Interview; CIDI) administered by clinical psychologists.ResultsHearing impairment was associated with CIDI psychotic symptoms [odds ratio (OR) 2.04, 95% confidence interval (CI) 1.10–3.81], particularly more severe psychotic symptoms (OR 5.66, 95% CI 1.64–19.49). The association between hearing impairment and CIDI psychotic symptoms was much stronger in the youngest group aged 14–17 years at baseline (OR 3.28, 95% CI 1.54–7.01) than in the older group aged 18–24 years at baseline (OR 0.82, 95% CI 0.24–2.84).ConclusionsThe finding of an age-specific association between hearing impairment and psychotic experiences suggests that disruption of development at a critical adolescent phase, in interaction with other personal and social vulnerabilities, may increase the risk for psychotic symptoms.
Evolution of neuropsychological dysfunction during the course of schizophrenia and bipolar disorder
Authors: Lewandowski K. E. , Cohen B. M. , Öngur D. 
Published: May 2010

BackgroundNeurocognitive dysfunction in schizophrenia (SZ), bipolar (BD) and related disorders represents a core feature of these illnesses, possibly a marker of underlying pathophysiology. Substantial overlap in domains of neuropsychological deficits has been reported among these disorders after illness onset. However, it is unclear whether deficits follow the same longitudinal pre- and post-morbid course across diagnoses. We examine evidence for neurocognitive dysfunction as a core feature of all idiopathic psychotic illnesses, and trace its evolution from pre-morbid and prodromal states through the emergence of overt psychosis and into chronic illness in patients with SZ, BD and related disorders.MethodArticles reporting on neuropsychological functioning in patients with SZ, BD and related disorders before and after illness onset were reviewed. Given the vast literature on these topics and the present focus on cross-diagnostic comparisons, priority was given to primary data papers that assessed cross-diagnostic samples and recent meta-analyses.ResultsPatients with SZ exhibit dysfunction preceding the onset of illness, which becomes more pronounced in the prodrome and early years following diagnosis, then settles into a stable pattern. Patients with BD generally exhibit typical cognitive development pre-morbidly, but demonstrate deficits by first episode that are amplified with worsening symptoms and exacerbations.ConclusionsNeuropsychological deficits represent a core feature of SZ and BD; however, their onset and progression differ between diagnostic groups. A lifetime perspective on the evolution of neurocognitive deficits in SZ and BD reveals distinct patterns, and may provide a useful guide to the examination of the pathophysiological processes underpinning these functions across disorders.
Pathways between early visual processing and functional outcome in schizophrenia
Authors: Rassovsky Y. , Horan W. P. , Lee J. , Sergi M. J. , Green M. F. 
Published: May 2010

BackgroundEarly visual processing deficits are reliably detected in schizophrenia and show relationships to poor real-world functioning. However, the nature of this relationship is complex. Theoretical models and recent studies using statistical modeling approaches suggest that multiple intervening factors are involved. We previously reported that a direct and significant association between visual processing and functional status was mediated by a measure of social perception. The present study examined the contribution of negative symptoms to this model.MethodWe employed structural equation modeling (sem) to test several models of outcome, using data from 174 schizophrenia out-patients. Specifically, we examined the direct and indirect relative contributions of early visual processing, social perception and negative symptoms to functional outcome.ResultsFirst, we found that, similar to social perception, a measure of negative symptoms mediated the association between visual information processing and functional status. Second, we found that the inclusion of negative symptoms substantially enhanced the explanatory power of the model. Notably, it was the experiential aspect of negative symptoms (avolition and anhedonia) more than the expressive aspect (affective flattening and alogia) that accounted for significant variance in functional outcome, especially in the social component of the construct of functional outcome.ConclusionsSocial perception and negative symptoms play relevant roles in functional impairment in schizophrenia. Both social perception and negative symptoms statistically mediate the connection between visual processing and functional outcome. However, given the lack of association between social perception and negative symptoms, these constructs appear to have an impact on functioning through separate pathways.
Psychophysiological responses to different levels of cognitive and physical workload in haptic interaction
Authors: Novak Domen , Mihelj Matjaž , Munih Marko 
Published: May 2010

SUMMARYPsychophysiological measurements, which serve as objective indicators of psychological state, have recently been introduced into human–robot interaction. However, their usefulness in haptic interaction is uncertain, since they are influenced by physical workload. This study analyses psychophysiological responses to a haptic task with three different difficulty levels and two different levels of physical load. Four physiological responses were recorded: heart rate, skin conductance, respiratory rate and skin temperature. Results show that mean respiratory rate, respiratory rate variability and skin temperature show significant differences between difficulty levels regardless of physical load and can be used to estimate cognitive workload in haptic interaction.
Democratic decentralization in sub-Saharan Africa: its contribution to forest management, livelihoods, and enfranchisement
Authors: RIBOT J. C. , LUND J. F. , TREUE T. 
Published: May 2010

SUMMARYEfforts to promote popular participation in forest management in Sub-Saharan Africa have faced many obstacles and disappointments. Although promises of improvements in relation to forest management, rural livelihoods and local enfranchisement have been achieved in some cases, accounts of frustration outnumber those of success. Focusing on participation through democratic decentralization (namely the transfer of meaningful discretionary powers to local representative authorities), this paper reviews recent empirical studies on the outcomes of popular participation in forest management. The implementation of decentralization of forest management, and ecological, livelihood and democracy outcomes are examined, and misconceptions in analyses of decentralized forestry are explored. The expected benefits of democratic decentralization within forestry are rarely realized because democratic decentralization is rarely established. In most cases, local authorities do not represent the local population or their space of discretion is so narrow that they have little effect on management. There is little official local management taking place, even under so-called decentralized or participatory management arrangements. If ever significant space for local discretion under democratic authorities is created, researchers will have the opportunity to study whether democratic decentralization can deliver the theoretically promised positive outcomes. Nevertheless, some cases shed light on effects of local decision making. Three general observations are made on effects of decentralization. First, environmental, livelihood and democracy objectives are not always mutually reinforcing, and under some circumstances they may be at odds. Second, environmental effects of improved forest management often result in benefits accruing to distant or higher-scale aggregate populations, while local communities carry the costs. Third, poor peoples' use of natural resources to maintain their livelihoods often conflicts with profit and revenue interests of local elites, national commercial interests and governments. A negotiated minimum social and environmental standards approach to decentralization of forest management may safeguard essential ecological functions and at the same time protect essential livelihood and economic values of forests at all scales of society. The remainder of decisions, such as how forests are used, by whom and for what, could then be safely placed at the discretion of responsive local representatives.
Avian biology, the human influence on global avian influenza transmission, and performing surveillance in wild birds
Authors: Gibbs Samantha E. J. 
Published: May 2010

AbstractThis paper takes a closer look at three interrelated areas of study: avian host biology, the role of human activities in virus transmission, and the surveillance activities centered on avian influenza in wild birds. There are few ecosystems in which birds are not found. Correspondingly, avian influenza viruses are equally global in distribution, relying on competent avian hosts. The immune systems, annual cycles, feeding behaviors, and migration patterns of these hosts influence the ecology of the disease. Decreased biodiversity has also been linked to heightened disease transmission in several disease systems, and it is evident that active destruction and modification of wetland environments for human use is impacting avian populations drastically. Legal and illegal trade in wild birds present a significant risk for introduction and maintenance of exotic diseases. After the emergence of HPAI H5N1 in Hong Kong in 1996 and the ensuing geographic spread of outbreaks after 2003, both infected countries and those at risk of introduction began intensifying avian influenza surveillance efforts. Several techniques for sampling wild birds for influenza viruses have been applied. Benefits, problems, and biases exist for each method. The wild bird avian influenza surveillance programs taking place across the continents are now scaling back due to the rise of other spending priorities; hopefully the lessons learned from this work will be preserved and will inform future research and disease outbreak response priorities.
Study of putative microfossils in space dust from the stratosphere
Authors: Rauf Kani , Hann Anthony , Wallis Max , Wickramasinghe Chandra 
Published: May 2010

AbstractInterplanetary dust particles (IDPs) were recovered from the stratosphere by a cryosampler flown below a balloon flying at altitudes of 20–41 km. The present study uses high-resolution scanning electron microscopy (SEM) and ultraviolet-visible (UV-Vis) spectrophotometry to examine fresh samples collected at 38–41 km. The SEM observations confirm the presence of 7–32 μm sized clusters of coccoidal (0.4–1.3 μm in diameter) and rod-shaped (0.6–2.5 μm in length) objects as components of the IDP complex. Many single globules (1.6–9.0 μm in diameter) are also observed, some of which exhibit a rough surface with filamentous features of variable lengths. The spectrophotometry of the particles in aggregate reveals a prominent peak centred at 216 nm, which is remarkably similar to that of diatoms and close to the UV astronomical feature of 217.5 nm that has been identified as the spectral characteristic of aromatic hydrocarbons. The evidence presented here suggests that the stratospheric particles are IDPs comprising an assortment of materials among which are included microfossil-like features in variable sizes and forms, such as coccoids, rods and filaments.
Towards evidence-based clinical supervision: the development and evaluation of four CBT guidelines
Authors: Milne Derek , Dunkerley Chris 
Published: May 2010

AbstractClinical supervision is central to evidence-based practice (EBP) and continuing professional development (CPD), but the evidence base has made little impact on supervision, a major form of CPD. We unite the two by developing four evidence-based guidelines for cognitive behavioural therapy (CBT) supervision. The guidelines were designed to address the supervision cycle (i.e. collaborative goal-setting; methods of facilitating learning; evaluation and feedback) within the context of the supervision alliance. Guideline development followed the National Institute for Clinical Excellence approach, including a representative stakeholder working group (with local service users and supervisees), a national group of supervisors and supervisor trainers, plus an expert reference group. A total of 106 such participants completed anad-hocguideline evaluation tool, designed to provide a multi-dimensional reaction evaluation of the guidelines. The guidelines were all rated favourably, satisfying the key initial criteria of accuracy and acceptability, and were judged to represent a CBT approach to supervision. It is concluded that the use of the guidelines might help CBT supervisors to better meet demands for CPD (including specialization in supervision) and EBP.
Open Source Kinesin-1 Stochastic Simulation and Gliding Assay Tracking Software
Authors: Herskowitz Lawrence , Salvagno Anthony , Josey Brian , Maloney Andy , Koch Steven 
Published: May 2010

AbstractKinesin-1 (conventional kinesin) is a homodimeric motor protein important for axonal transport. It has been well studied through ensemble and single-molecule assays. However, the enzymatic stepping cycle is complex, with many rate constants that are modulated by interaction of the two motor domains. This makes it difficult to predict how changes in a given rate constant may affect observable properties such as processivity, velocity, or stall force. We have written a simulation of kinesin walking using a Stochastic Simulation Algorithm. The model allows for interactions between the heads, and includes states that are not considered part of the normal cycle. This adds to the complexity of the model but also allows for probing rare events, such as those that lead to a finite processivity. Also included are rate constant dependencies on force and concentrations of ATP, ADP, and Pi, which may provide insight into other processes under investigation, such as kinesin backstepping. We intend to use the simulation to aid in interpreting our own gliding motility assay results and to place upper and lower limits on values for rate constants. Our source and executable codes will be freely available.
Acknowledgements:
This work was supported by the DTRA CB Basic Research Program under Grant No. HDTRA1-09-1-008.