Culture Matters: French-German Conflicts on European Central Bank Independence

With the Maastricht treaty, the members of the Eurozone agreed on the establishment of a very independent European Central Bank, as well as making their National Central Banks far more independent. However, over the years French political leaders systematically brought forward proposals undermining the ECB's independence, to the dismay of Germany. A pattern that surfaced again during the current sovereign debt crisis and has complicated finding a timely and unified answer to the problems. The article conducts tests of various factors expected to influence the preference for central bank independence. It shows that economic explanations are unable to account for the persistent differences amongst European member-states on this issue. Instead, cultural differences in attitudes, especially a nation's score on the dimension of Power Distance - its acceptance of centralisation of power in a small set of political leaders or institutions - does show a correlation with the different levels of internalisation of the Central Bank independence norm.Central Bank Independence; Culture; European Central Bank; Franco- German relations

A novel fragile X syndrome mutation reveals a conserved role for the carboxy-terminus in FMRP localization and function

Loss of function of the FMR1 gene leads to fragile X syndrome (FXS), the most common form of intellectual disability. The loss of FMR1 function is usually caused by epigenetic silencing of the FMR1 promoter leading to expansion and subsequent methylation of a CGG repeat in the 5′ untranslated region. Very few coding sequence variations have been experimentally characterized and shown to be causal to the disease. Here, we describe a novel FMR1 mutation and reveal an unexpected nuclear export function for the C-terminus of FMRP. We screened a cohort of patients with typical FXS symptoms who tested negative for CGG repeat expansion in the FMR1 locus. In one patient, we identified a guanine insertion in FMR1 exon 15. This mutation alters the open reading frame creating a short novel C-terminal sequence, followed by a stop codon. We find that this novel peptide encodes a functional nuclear localization signal (NLS) targeting the patient FMRP to the nucleolus in human cells. We also reveal an evolutionarily conserved nuclear export function associated with the endogenous C-terminus of FMRP. In vivo analyses in Drosophila demonstrate that a patient-mimetic mutation alters the localization and function of Dfmrp in neurons, leading to neomorphic neuronal phenotypes

Epigenetic characterization of the FMR1 promoter in induced pluripotent stem cells from human fibroblasts carrying an unmethylated full mutation

Silencing of the FMR1 gene leads to fragile X syndrome, the most common cause of inherited intellectual disability. To study the epigenetic modifications of the FMR1 gene during silencing in time, we used fibroblasts and induced pluripotent stem cells (iPSCs) of an unmethylated full mutation (uFM) individual with normal intelligence. The uFM fibroblast line carried an unmethylated FMR1 promoter region and expressed normal to slightly increased FMR1 mRNA levels. The FMR1 expression in the uFM line corresponds with the increased H3 acetylation and H3K4 methylation in combination with a reduced H3K9 methylation. After reprogramming, the FMR1 promoter region was methylated in all uFM iPSC clones. Two clones were analyzed further and showed a lack of FMR1 expression, whereas the presence of specific histone modifications also indicated a repressed FMR1 promoter. In conclusion, these findings demonstrate that the standard reprogramming procedure leads to epigenetic silencing of the fully mutated FMR1 gene

The nature of the european leadership crisis and how to solve it

Observers of the European Union (EU) agree that it suffers from a leadership crisis. However, diagnoses of the precise nature of this crisis vary: some lament the lack of strong, visionary leaders, while others argue that the EU suffers from too much elite leadership. This article takes issue with both diagnoses and argues that the root of Europe's leadership crisis lies in the misfit between the nature of EU leadership and the legitimating logic it is rooted in. All leadership implies inequality and therefore requires solid justification especially in the democratic European context. However, at the European level, the vectors of legitimacy that provide such justification are weak and contradictory, thereby tempting leaders to overstep the level of justification bestowed on them. Making use of ideological and identity leadership may help European leaders overcome the misfit between leadership and legitimacy that lies at the root of the leadership crisis

The nature of the european leadership crisis and how to solve it

Observers of the European Union (EU) agree that it suffers from a leadership crisis. However, diagnoses of the precise nature of this crisis vary: some lament the lack of strong, visionary leaders, while others argue that the EU suffers from too much elite leadership. This article takes issue with both diagnoses and argues that the root of Europe's leadership crisis lies in the misfit between the nature of EU leadership and the legitimating logic it is rooted in. All leadership implies inequality and therefore requires solid justification especially in the democratic European context. However, at the European level, the vectors of legitimacy that provide such justification are weak and contradictory, thereby tempting leaders to overstep the level of justification bestowed on them. Making use of ideological and identity leadership may help European leaders overcome the misfit between leadership and legitimacy that lies at the root of the leadership crisis