Together forever? Explaining exclusivity in party-firm relations

Parties and firms are the key actors of representative democracy and capitalism respectively and the dynamic of attachment between them is a central feature of any political economy. This is the first article to systematically analyse the exclusivity of party-firm relations. We consider exclusivity at a point in time and exclusivity over time. Does a firm have a relationship with only one party at a given point in time, or is it close to more than one party? Does a firm maintain a relationship with only one party over time, or does it switch between parties? Most important, how do patterns of exclusivity impact on a firm’s ability to lobby successfully? We propose a general theory, which explains patterns of party-firm relations by reference to the division of institutions and the type of party competition in a political system. A preliminary test of our theory with Polish survey data confirms our predictions, establishing a promising hypothesis for future research

The Sub-State Politics of Welfare in Italy: Assessing the Effect of Territorial Mobilization on the Development of Region-Specific Social Governance

This article demonstrates that the political mobilization of regional identities through the creation of regionalist parties has positively impacted on the development of region-specific models of welfare governance in Italy. This means that, in a decentralized country, the ‘centre-periphery’ cleavage may significantly influence the sub-state politics of welfare

Treatment of Fabry Disease: Outcome of a Comparative Trial with Agalsidase Alfa or Beta at a Dose of 0.2 mg/kg

Two different enzyme preparations, agalsidase alfa (Replagal(TM), Shire) and beta (Fabrazyme(TM), Genzyme), are registered for treatment of Fabry disease. We compared the efficacy of and tolerability towards the two agalsidase preparations administered at identical protein dose in a randomized controlled open label trial.Thirty-four Fabry disease patients were treated with either agalsidase alfa or agalsidase beta at equal dose of 0.2 mg/kg biweekly. Primary endpoint was reduction in left ventricular mass after 12 and 24 months of treatment. Other endpoints included occurrence of treatment failure (defined as progression of cardiac, renal or cerebral disease), glomerular filtration rate, pain, anti-agalsidase antibodies, and globotriaosylceramide levels in plasma and urine. After 12 and 24 months of treatment no reduction in left ventricular mass was seen, which was not different between the two treatment groups. Also, no differences in glomerular filtration rate, pain and decline in globotriaosylceramide levels were found. Antibodies developed only in males (4/8 in the agalsidase alfa group and 6/8 in the agalsidase beta group). Treatment failure within 24 months of therapy was seen in 8/34 patients: 6 male patients (3 in each treatment group) and 2 female patients (both agalsidase alfa). The occurrence of treatment failures did not differ between the two treatment groups; chi(2) = 0.38 p = 0.54.Our study revealed no difference in reduction of left ventricular mass or other disease parameters after 12 and 24 months of treatment with either agalsidase alfa or beta at a dose of 0.2 mg/kg biweekly. Treatment failure occurred frequently in both groups and seems related to age and severe pre-treatment disease.International Standard Randomized Clinical Trial ISRCTN45178534 [http://www.controlled-trials.com/ISRCTN45178534]

Inconsistent impacts of decomposer diversity on the stability of aboveground and belowground ecosystem functions

The intensive discussion on the importance of biodiversity for the stability of essential processes in ecosystems has prompted a multitude of studies since the middle of the last century. Nevertheless, research has been extremely biased by focusing on the producer level, while studies on the impacts of decomposer diversity on the stability of ecosystem functions are lacking. Here, we investigate the impacts of decomposer diversity on the stability (reliability) of three important aboveground and belowground ecosystem functions: primary productivity (shoot and root biomass), litter decomposition, and herbivore infestation. For this, we analyzed the results of three laboratory experiments manipulating decomposer diversity (1–3 species) in comparison to decomposer-free treatments in terms of variability of the measured variables. Decomposer diversity often significantly but inconsistently affected the stability of all aboveground and belowground ecosystem functions investigated in the present study. While primary productivity was mainly destabilized, litter decomposition and aphid infestation were essentially stabilized by increasing decomposer diversity. However, impacts of decomposer diversity varied between plant community and fertility treatments. There was no general effect of the presence of decomposers on stability and no trend toward weaker effects in fertilized communities and legume communities. This indicates that impacts of decomposers are based on more than effects on nutrient availability. Although inconsistent impacts complicate the estimation of consequences of belowground diversity loss, underpinning mechanisms of the observed patterns are discussed. Impacts of decomposer diversity on the stability of essential ecosystem functions differed between plant communities of varying composition and fertility, implicating that human-induced changes of biodiversity and land-use management might have unpredictable effects on the processes mankind relies on. This study therefore points to the necessity of also considering soil feedback mechanisms in order to gain a comprehensive and holistic understanding of the impacts of current global change phenomena on the stability of essential ecosystem functions

Biodiversity and structure of spider communities along a metal pollution gradient

The objective of the study was to determine whether long-term metal pollution affects communities of epigeal spiders (Aranea), studied at three taxonomic levels: species, genera, and families. Biodiversity was defined by three indices: the Hierarchical Richness Index (HRI), Margalef index (DM) and Pielou evenness index (J). In different ways the indices describe taxa richness and the distribution of individuals among taxa. The dominance pattern of the communities was described with four measures: number of dominant species at a site, percentage of dominant species at a site, average dominant species abundance at a site, and the share of the most numerous species (Alopecosa cuneata) at a site. Spiders were collected along a metal pollution gradient in southern Poland, extending ca. 33 km from zinc and lead smelter to an uncontaminated area. The zinc concentration in soil was used as the pollution index.The study revealed a significant effect of metal pollution on spider biodiversity as described by HRI for species (p = 0.039), genera (p = 0.0041) and families (p = 0.0147), and by DM for genera (p = 0.0259) and families (p = 0.0028). HRI correlated negatively with pollution level, while DM correlated positively. This means that although broadly described HRI diversity decreased with increasing pollution level, species richness increased with increasing contamination. Mesophilic meadows were generally richer. Pielou (J) did not show any significant correlations. There were a few evidences for the intermediate disturbance hypothesis: certain indices reached their highest values at moderate pollution levels rather than at the cleanest or most polluted sites

The Enigma of Soil Animal Species Diversity Revisited: The Role of Small-Scale Heterogeneity

Peer reviewedPublisher PD

Cynnydd dysgu yn y dyniaethau: canfod tensiynau wrth gyfleu cynnydd yn y dyniaethau yng Nghymru

Mae’r papur hwn yn edrych ar y tensiynau sy’n codi wrth gyfleu cynnydd mewn dysgu yn nisgyblaethau’r Dyniaethau. Ar sail ein hadolygiad o ymchwil yn nisgyblaethau’r Dyniaethau, cwricwla rhyngwladol ar gynnydd yn y meysydd hyn a myfyrdodau o weithgarwch proffesiynol ym ‘Maes Dysgu a Phrofiad’ y Dyniaethau, sydd newydd gael ei ddiffinio yn y Cwricwlwm i Gymru newydd, mae’r papur hwn yn disgrifio sut mae cynnydd dysgu yn y Dyniaethau wedi’i gysyniadoli yn y cwricwlwm newydd ac wedyn yn amlinellu ac yn adolygu’n feirniadol bedair her a gododd wrth ganfod a disgrifio’r cynnydd dysgu yng nghwricwlwm newydd y Dyniaethau. Mae’r tensiynau’n cynnwys y berthynas rhwng disgyblaethau; y cydbwysedd rhwng gwybodaeth, sgiliau a gwerthoedd; y gwahaniaethau rhwng modelau cynnydd sylfaenol yn y Dyniaethau; a chydbwyso cymhlethdod y dysgu ag ystyriaethau ymarferol ar gyfer cwricwlwm cenedlaethol. Drwy ddefnyddio’r Model Newid Integredd, mae’r papur hwn yn cyfrannu drwy gynnig mewnwelediadau newydd i agweddau eang ar gynnydd dysgu yn y Dyniaethau a thrwy dynnu sylw at y buddion a heriau dichonol o wneud penderfyniadau penodol mewn perthynas â phob un o’r tensiynau hyn. Trafodir goblygiadau sy’n codi mewn perthynas â chynllunio’r cwricwlwm ac ymchwil yn y dyfodol, yn cynnwys y rôl sylfaenol sydd gan ddysgu proffesiynol wrth ddatblygu a gweithredu’r cwricwlwm

Mechanisms of stretch-mediated skin expansion at single-cell resolution.

The ability of the skin to grow in response to stretching has been exploited in reconstructive surgery1. Although the response of epidermal cells to stretching has been studied in vitro2,3, it remains unclear how mechanical forces affect their behaviour in vivo. Here we develop a mouse model in which the consequences of stretching on skin epidermis can be studied at single-cell resolution. Using a multidisciplinary approach that combines clonal analysis with quantitative modelling and single-cell RNA sequencing, we show that stretching induces skin expansion by creating a transient bias in the renewal activity of epidermal stem cells, while a second subpopulation of basal progenitors remains committed to differentiation. Transcriptional and chromatin profiling identifies how cell states and gene-regulatory networks are modulated by stretching. Using pharmacological inhibitors and mouse mutants, we define the step-by-step mechanisms that control stretch-mediated tissue expansion at single-cell resolution in vivo.Wellcome Trust Royal Societ

CTCF variants in 39 individuals with a variable neurodevelopmental disorder broaden the mutational and clinical spectrum

Purpose: Pathogenic variants in the chromatin organizer CTCF were previously reported in seven individuals with a neurodevelopmental disorder (NDD). Methods: Through international collaboration we collected data from 39 subjects with variants in CTCF. We performed transcriptome analysis on RNA from blood samples and utilized Drosophila melanogaster to investigate the impact of Ctcf dosage alteration on nervous system development and function. Results: The individuals in our cohort carried 2 deletions, 8 likely gene-disruptive, 2 splice-site, and 20 different missense variants, most of them de novo. Two cases were familial. The associated phenotype was of variable severity extending from mild developmental delay or normal IQ to severe intellectual disability. Feeding difficulties and behavioral abnormalities were common, and variable other findings including growth restriction and cardiac defects were observed. RNA-sequencing in five individuals identified 3828 deregulated genes enriched for known NDD genes and biological processes such as transcriptional regulation. Ctcf dosage alteration in Drosophila resulted in impaired gross neurological functioning and learning and memory deficits. Conclusion: We significantly broaden the mutational and clinical spectrum of CTCF-associated NDDs. Our data shed light onto the functional role of CTCF by identifying deregulated genes and show that Ctcf alterations result in nervous system defects in Drosophila.Peer reviewe