Party Organisation in a multi-level setting: Spain and the United Kingdom..

Aangezien een talrijke groep van landen vandaag de dag één of andere vorm van decentralisatie heeft aangenomen, zijn vragen rond federalisme, meerlagig beleid en regionalisme belangrijke onderzoeksobjecten geworden. Deze doctoraatsthesis past het meerlagig perspectief toe op de studie van politieke partijen en onderzoekt hoe nationale partijen dit zijn partijen die kandidaten voor nationale en regionale verkiezingen presenteren over het gehele territorium - de interne relaties organiseren tussen het nationale niveau en de regionale partijafdelingen. De nationale partijen worden geconfronteerd met een specifiek dilemma in gedecentraliseerde systemen: aan de ene kant dienen ze zich tijdens nationale verkiezingen met een coherente boodschap voor het hele nationale territorium als één front te presenteren en aan de andere kant moeten ze zich tijdens regionale verkiezingen richten op een regionaal electoraat met haar eigen specifieke belangen. Het bestaan van verschillende beleids- en verkiezingsniveaus stelt de nationale partijen dus voor een probleem van verticale coördinatie.Deze doctoraatsthesis probeert deze vraag te beantwoorden via de voorbeelden van 3 Britse partijen (de Socialistische partij, de Conservatieve partij en de Liberaal-democratische partij) en 2 Spaanse partijen (de Conservatieve partij en de Socialistische partij). De koppeling tussen het nationale en regionale niveau bestaat uit twee dimensies: de integratie van de regionale partijafdelingen in de centrale beslissingsorganen en de autonomie van de regionale partijafdelingen in de regionale beslissingsniveaus. Deze studie concentreert zich op de organisatie van nationale partijen in slechts 3 van de 17 autonome gemeenschappen in Spanje (Catalonië, Baskenland en Galicië) en op de organisatie van de Britse partijen in Schotland en Wales.Deze studie staat toe om kwalitatief een aantal hypotheses te testen die geformuleerd worden in een ruim theoretische neoninstitutioneel kader en die een brede waaier aan variabelen bevat die de manier beïnvloeden waarop de partijen zich organiseren in een meerlagig kader: ten eerste institutionele variabelen (type van staatsstructuur, niveau van regionale bevoegdheden), ten tweede sociale factoren (sociale breuklijnen, regionalisme en regionale identificatie van het kiezerspubliek), vervolgens variabelen die verbonden zijn met de electorale competitie op het nationale en regionale niveau en tenslotte factoren die de eigenheid van de partijen illustreren, zoals de manier waarop ze gevormd werden, het partytype en hun ideologie.De empirische studie toont een verscheidenheid aan organisatievormen. Partijen in eenzelfde land zijn niet noodzakelijkerwijze op dezelfde manier georganiseerd, net zoals regionale afdelingen in eenzelfde regio niet altijd hetzelfde bevoegdheidsniveau hebben. Over het algemeen lijkt het institutionele kader enkel een rol te spelen in de mate dat het de kansen creëert waarbinnen de regionale structuren zich kunnen ontwikkelen te midden van de nationale partijen. Het type van staatsstructuur en het regionale bevoegdheidsniveau worden niet weerspiegeld in de mate van autonomie van de regionale partijafdelingen en ook niet in hun integratie in de centrale partijorganen. De factoren die de eigenheid van de partij bevestigen aan de andere kant, zoals hun ideologie, de manier waarop ze gevormd werden en hun plaats binnen het huidige politieke landschap, op ieder beleidsniveau, als zijnde regeringspartij of in de oppositie blijken bijzonder belangrijk te zijn. Zo blijkt de rol van de nationale en regionale partijleiders cruciaal, en blijkt een regeringspositie een belangrijk hulpmiddel te zijn in de machtverhoudingen tussen de verschillende beleidsniveaus. De doctoraatsthesis toont op deze manier het belang aan van de factoren die de eigenheid van de partij beklemtonen en de bepalende rol van de spelers in het proces van organisationele verandering.

Combined Mutation And Rearrangement Screening by Quantitative PCR High-Resolution Melting: Is It Relevant for Hereditary Recurrent Fever Genes?

The recent identification of genes implicated in hereditary recurrent fevers has allowed their specific diagnosis. So far however, only punctual mutations have been identified and a significant number of patients remain with no genetic confirmation of their disease after routine molecular approaches such as sequencing. The possible involvement of sequence rearrangements in these patients has only been examined in familial Mediterranean fever and was found to be unlikely. To assess the existence of larger genetic alterations in 3 other concerned genes, MVK (Mevalonate kinase), NLRP3 (Nod like receptor family, pyrin domain containing 3) and TNFRSF1A (TNF receptor superfamily 1A), we adapted the qPCR-HRM method to study possible intragenic deletions and duplications. This single-tube approach, combining both qualitative (mutations) and quantitative (rearrangement) screening, has proven effective in Lynch syndrome diagnosis. Using this approach, we studied 113 unselected (prospective group) and 88 selected (retrospective group) patients and identified no intragenic rearrangements in the 3 genes. Only qualitative alterations were found with a sensitivity similar to that obtained using classical molecular techniques for screening punctual mutations. Our results support that deleterious copy number alterations in MVK, NLRP3 and TNFRSF1A are rare or absent from the mutational spectrum of hereditary recurrent fevers, and demonstrate that a routine combined method such as qPCR-HRM provides no further help in genetic diagnosis. However, quantitative approaches such as qPCR or SQF-PCR did prove to be quick and effective and could still be useful after non contributory punctual mutation screening in the presence of clinically evocative signs

Membrane estrogen receptor-α contributes to female protection against high-fat diet-induced metabolic disorders

BackgroundEstrogen Receptor α (ERα) is a significant modulator of energy balance and lipid/glucose metabolisms. Beyond the classical nuclear actions of the receptor, rapid activation of intracellular signaling pathways is mediated by a sub-fraction of ERα localized to the plasma membrane, known as Membrane Initiated Steroid Signaling (MISS). However, whether membrane ERα is involved in the protective metabolic actions of endogenous estrogens in conditions of nutritional challenge, and thus contributes to sex differences in the susceptibility to metabolic diseases, remains to be clarified.MethodsMale and female C451A-ERα mice, harboring a point mutation which results in the abolition of membrane localization and MISS-related effects of the receptor, and their wild-type littermates (WT-ERα) were maintained on a normal chow diet (NCD) or fed a high-fat diet (HFD). Body weight gain, body composition and glucose tolerance were monitored. Insulin sensitivity and energy balance regulation were further investigated in HFD-fed female mice.ResultsC451A-ERα genotype had no influence on body weight gain, adipose tissue accumulation and glucose tolerance in NCD-fed mice of both sexes followed up to 7 months of age, nor male mice fed a HFD for 12 weeks. In contrast, compared to WT-ERα littermates, HFD-fed C451A-ERα female mice exhibited: 1) accelerated fat mass accumulation, liver steatosis and impaired glucose tolerance; 2) whole-body insulin resistance, assessed by hyperinsulinemic-euglycemic clamps, and altered insulin-induced signaling in skeletal muscle and liver; 3) significant decrease in energy expenditure associated with histological and functional abnormalities of brown adipose tissue and a defect in thermogenesis regulation in response to cold exposure.ConclusionBesides the well-characterized role of ERα nuclear actions, membrane-initiated ERα extra-nuclear signaling contributes to female, but not to male, protection against HFD-induced obesity and associated metabolic disorders in mouse

Party rules, party resources, and the politics of parliamentary democracies: how parties organize in the 21st Century

This article introduces the first findings of the Political Party Database (PPDB) project, a major survey of party organizations in parliamentary and semi-presidential democracies. The project’s first round of data covers 122 parties in 19 countries. In this paper we describe the scope of the database, then investigate what it tells us about contemporary party organization in these countries, focussing on parties’ resources, structures and internal decision-making. We examine organizational patterns by country and party family, and where possible we make temporal comparisons with older datasets. Our analyses suggest a remarkable coexistence of uniformity and diversity. In terms of the major organizational resources on which parties can draw, such as members, staff and finance, the new evidence largely confirms the continuation of trends identified in previous research: i.e., declining membership, but enhanced financial resources and more paid staff. We also find remarkable uniformity regarding the core architecture of party organizations. At the same time, however, we find substantial variation between countries and party families in terms of their internal processes, with particular regard to how internally democratic they are, and in the forms that this democratization takes

COVID-19 symptoms at hospital admission vary with age and sex: results from the ISARIC prospective multinational observational study

Background: The ISARIC prospective multinational observational study is the largest cohort of hospitalized patients with COVID-19. We present relationships of age, sex, and nationality to presenting symptoms. Methods: International, prospective observational study of 60 109 hospitalized symptomatic patients with laboratory-confirmed COVID-19 recruited from 43 countries between 30 January and 3 August 2020. Logistic regression was performed to evaluate relationships of age and sex to published COVID-19 case definitions and the most commonly reported symptoms. Results: ‘Typical’ symptoms of fever (69%), cough (68%) and shortness of breath (66%) were the most commonly reported. 92% of patients experienced at least one of these. Prevalence of typical symptoms was greatest in 30- to 60-year-olds (respectively 80, 79, 69%; at least one 95%). They were reported less frequently in children (≤ 18 years: 69, 48, 23; 85%), older adults (≥ 70 years: 61, 62, 65; 90%), and women (66, 66, 64; 90%; vs. men 71, 70, 67; 93%, each P < 0.001). The most common atypical presentations under 60 years of age were nausea and vomiting and abdominal pain, and over 60 years was confusion. Regression models showed significant differences in symptoms with sex, age and country. Interpretation: This international collaboration has allowed us to report reliable symptom data from the largest cohort of patients admitted to hospital with COVID-19. Adults over 60 and children admitted to hospital with COVID-19 are less likely to present with typical symptoms. Nausea and vomiting are common atypical presentations under 30 years. Confusion is a frequent atypical presentation of COVID-19 in adults over 60 years. Women are less likely to experience typical symptoms than men