L'évolution du partenariat UE-ACP de Lomé à Cotonou : de l'exception à la normalisation

L'accord de Cotonou marque un tournant dans les relations entre l'Union européenne et les Etats ACP. Ces relations étaient caractérisées par la volonté des Etats européens d'aider les Etats ACP aussi bien par le moyen classique de l'aide au développement qu'en utilisant l'outil de la politique commerciale. Les vives contestations des exportateurs de bananes sud-américaines devaient conduire, après la condamnation de l'Union européenne par l'Organe de Règlement des Différends de l'OMC en 1997, à une remise en cause de la discrimination commerciale positive des pays ACP. Pour sa part, le débat sur l'aide au développement de l'UE s'inscrit dans la perspective d'une réforme de la conditionnalité de l'aide internationale aussi bien dans son contenu que dans sa mise en oeuvre. Sous cet aspect, l'UE pourrait également perdre en originalité et simplement devenir un des acteurs du nouveau consensus orchestré par les institutions de Bretton Woods (stratégies de lutte contre la pauvreté) et le système onusien (objectifs du millénaire).Organisation Mondiale du Commerce ; politique commerciale ; régionalisation ; multilatéralisme ; accord de Cotonou ; aide au développement ; conditionnalité

The Fragmentation of French Collective Bargaining

Since the 1980s, collective bargaining in France has transitioned from a centralised model based on national labour laws and sectoral agreements to a decentralised system emphasising company-level agreements. Historically, law, and sectoral agreements provided strong, uniform worker protections, but reforms, including President Emmanuel Macron’s 2017 executive orders, have allowed company agreements to override sectoral agreements and legislation, even when less favourable to employees. This shift aims to enhance flexibility and competitiveness but has fragmented protections. Small companies increasingly use direct referendums to approve agreements without formal negotiation, leading to reduced protections such as longer hours and lower pay. Many companies use a new category of collective agreements, the “collective performance agreements,” to adapt to economic challenges and alter the protected contents of employments contracts. However, many companies maintain existing balances. Simultaneously, some trends towards recentralisation are evident. Group-level agreements now allow corporate groups to standardise employment policies across subsidiaries. Many agreements rely on generic templates to comply with state-mandated collective bargaining on issues like gender equality. This evolving system underscores the tension between economic flexibility and maintaining workers’ rights, with long-term impacts still unclear

The 2018 French Asylum and Immigration Act

As many others in the European Union, the French government attempts to tackle the so-called “migrants crisis”. A new bill aims at reducing the length of asylum proceedings and fighting against irregular migration. Despite disagreements between the two chambers, the National Assembly has adopted the last version of the bill on August 1st. Left-wing Members of Parliament have brought an action before the Constitutional Council. The bill may not be enacted before the Council has given its decision, at the beginning of September. The text will no longer change, unless some provisions are deemed unconstitutional

The French Antiterrorist Bill: A Permanent State of Emergency

In July, a government bill against terrorism was adopted to replace the French state of emergency, which is in force since the terrorist attacks of November 2015. Critics have long complained about the lasting of the etat d'urgence. An analysis of the new bill however reveals that it is still a threat for human rights and in that matter rather a softened version of the state of emergency

X-exome sequencing of 405 unresolved families identifies seven novel intellectual disability genes

X-linked intellectual disability (XLID) is a clinically and genetically heterogeneous disorder. During the past two decades in excess of 100 X-chromosome ID genes have been identified. Yet, a large number of families mapping to the X-chromosome remained unresolved suggesting that more XLID genes or loci are yet to be identified. Here, we have investigated 405 unresolved families with XLID. We employed massively parallel sequencing of all X-chromosome exons in the index males. The majority of these males were previously tested negative for copy number variations and for mutations in a subset of known XLID genes by Sanger sequencing. In total, 745 X-chromosomal genes were screened. After stringent filtering, a total of 1297 non-recurrent exonic variants remained for prioritization. Co-segregation analysis of potential clinically relevant changes revealed that 80 families (20%) carried pathogenic variants in established XLID genes. In 19 families, we detected likely causative protein truncating and missense variants in 7 novel and validated XLID genes (CLCN4, CNKSR2, FRMPD4, KLHL15, LAS1L, RLIM and USP27X) and potentially deleterious variants in 2 novel candidate XLID genes (CDK16 and TAF1). We show that the CLCN4 and CNKSR2 variants impair protein functions as indicated by electrophysiological studies and altered differentiation of cultured primary neurons from Clcn4−/− mice or after mRNA knock-down. The newly identified and candidate XLID proteins belong to pathways and networks with established roles in cognitive function and intellectual disability in particular. We suggest that systematic sequencing of all X-chromosomal genes in a cohort of patients with genetic evidence for X-chromosome locus involvement may resolve up to 58% of Fragile X-negative cases

Mutation update for the GPC3 gene involved in Simpson-Golabi-Behmel syndrome and review of the literature

Simpson-Golabi-Behmel syndrome (SGBS) is an X-linked multiple congenital anomalies and overgrowth syndrome caused by a defect in the glypican-3 gene (GPC3). Until now, GPC3 mutations have been reported in isolated cases or small series and the global genotypic spectrum of these mutations has never been delineated. In this study, we review the 57 previously described GPC3 mutations and significantly expand this mutational spectrum with the description of 29 novel mutations. Compiling our data and those of the literature, we provide an overview of 86 distinct GPC3 mutations identified in 120 unrelated families, ranging from single nucleotide variations to complex genomic rearrangements and dispersed throughout the entire coding region of GPC3. The vast majority of them are deletions or truncating mutations (frameshift, nonsense mutations) predicted to result in a loss-of-function. Missense mutations are rare and the two which were functionally characterized, impaired GPC3 function by preventing GPC3 cleavage and cell surface addressing respectively. This report by describing for the first time the wide mutational spectrum of GPC3 could help clinicians and geneticists in interpreting GPC3 variants identified incidentally by high-throughput sequencing technologies and also reinforces the need for functional validation of non-truncating mutations (missense, in frame mutations, duplications)

The Shortest Isoform of Dystrophin (Dp40) Interacts with a Group of Presynaptic Proteins to Form a Presumptive Novel Complex in the Mouse Brain

Duchenne muscular dystrophy (DMD) causes cognitive impairment in one third of the patients, although the underlying mechanisms remain to be elucidated. Recent studies showed that mutations in the distal part of the dystrophin gene correlate well with the cognitive impairment in DMD patients, which is attributed to Dp71. The study on the expression of the shortest isoform, Dp40, has not been possible due to the lack of an isoform specific antibody. Dp40 has the same promoter as that found in Dp71 and lacks the normal C-terminal end of Dp427. In the present study, we have raised polyclonal antibody against the N-terminal sequence common to short isoforms of dystrophin, including Dp40, and investigated the expression pattern of Dp40 in the mouse brain. Affinity chromatography with this antibody and the consecutive LC-MS/MS analysis on the interacting proteins revealed that Dp40 was abundantly expressed in synaptic vesicles and interacted with a group of presynaptic proteins, including syntaxin1A and SNAP25, which are involved in exocytosis of synaptic vesicles in neurons. We thus suggest that Dp40 may form a novel protein complex and play a crucial role in presynaptic function. Further studies on these aspects of Dp40 function might provide more insight into the molecular mechanisms of cognitive impairment found in patients with DMD