105 research outputs found
The electronic exchange of information and respect for private life, banking secrecy and the free internal market
The purpose of this essay is to assess the automatic exchange of information as described in EU Directive 2003/48 of 3 June 2003 on taxation of savings income in the form of interest payments with regard to the fundamental right of the individual to a private life, to banking secrecy and the freedoms on which the European internal market is based. The assessment reveals the conflicts of interests and values involved in the holding by banks (particularly those offering private banking services) of increasingly extensive, detailed and intimate information about their clients and in the automatic processing of that information by ever more powerful and sophisticated systems. Banking secrecy plays an essential role in protecting clients against the dangers which the disclosure of such information without their permission might produce. Banking secrecy exists not only in Luxembourg but also in many other European countries, and in Germany and France in particular it is not very different from the system applying in Luxembourg. While the French and German tax authorities do have some investigative powers not enjoyed by their Luxembourg counterparts, those powers are strictly circumscribed and cannot rely on the electronic exchange of information set out in EU Directive 2003/48/EC. While banking secrecy is totally incompatible with the electronic exchange of information, the core question is whether the latter can be reconciled with the respect for private life. In a Europe that sets itself up as the cradle of human rights, the general and en-masse exchange of private information cannot provide adequate and sufficient guarantees that the information exchanged will not be misused. The amount of interference in private life is clearly out of proportion to the public interest involved and is contrary to sub-section 2, article 8 of the European Convention for the Protection of Human Rights and Fundamental Freedoms and to articles 7 and 8 of the Charter of Fundamental Rights of the European Union. Since the automatic exchange of information at least potentially risks restricting the free flow of capital among Member States and discouraging the use of transborder banking services, its compliance with the fundamental principles of the internal market also needs to be closely examined. The restrictions imposed by such exchange very probably go beyond the limits within which the free movement of capital and services is possible. The European Court of Justice has found that there is no proportionality if the measures supposedly undertaken in the general interest are actually based on a general presumption of tax evasion or tax fraud. However, it would be true to say that the ECJ does not always examine the tax restrictions placed on the free movement of capital particularly thoroughly to ensure that they are necessary or proportionate. The economic effectiveness of the automatic exchange of information is far from being proved and involves significant cost to the banks providing the information and to the tax authorities using it. To date the system does not appear to have produced any significant new tax revenue nor does it prevent the continuing outflow of capital from Europe. Yet withholding at source, which respects individual and economic freedoms, does generate tax revenue that is cost-free to the State. Exchange of information on request in justified cases using the OECD Tax Convention on Income and Capital model does also fight tax fraud while at the same time providing citizens with the guarantees required to ensure their private lives are respected. A combination of these two systems - withholding at source and exchange of information on request in justified cases - would create the proper balance between the public and private interest that the automatic exchange of information cannot provide
Functional expression of human cathepsin S in Saccharomyces cerevisiae. Purification and characterization of the recombinant enzyme.
A cDNA encoding the human lysosomal cysteine proteinase cathepsin S precursor has been expressed in yeast using the pVT100-U expression vector containing the alpha-factor promoter. The procathepsin S gene was expressed either as a fusion protein with the pre-region or with the prepro-region of the yeast alpha-factor precursor gene. Following in vitro processing both constructs gave an identical active mature enzyme with a molecular weight of 24,000. After prolonged cultivation of the cells the recombinant protein is also found as an active proteinase in the culture supernatant. The precursor can be activated in vitro at pH 4.5 and 40 degrees C under reducing conditions. The in vitro activated enzyme has a 6-amino acid NH2-terminal extension when compared with the native bovine enzyme. The purified enzyme displays a bell-shaped pH activity profile with a pH optimum of 6.5 and pK values of 4.5 and 7.8. The isoelectric point of the recombinant human cathepsin S is between 8.3 and 8.6 and about 1.5 pH units higher than for the bovine enzyme. The kinetic data for several synthetic substrates and inhibitors reveal a preference for smaller amino acid residues in the binding subsites S2 and S3 of cathepsin S. Like the bovine enzyme, the recombinant human cathepsin S is characterized by a broader range of pH stability (pH 5-7.5) than cathepsins B and L
Connaissance du devenir des éléments à risques dans les différentes filières de gestion des effluents porcins
Cet article résume les apports du programme "Porcherie verte" dans la connaissance et la maîtrise du devenir de l'azote, du phosphore et des éléments traces métalliques qui ont, à des titres divers, un impact sur l'environnement. La diminution de la teneur en protéines de l'aliment permet de réduire fortement les quantités d'azote excrétées par les animaux. Une part importante de l'azote est éliminée sous forme gazeuse lorsque les animaux sont placés sur litière ou lorsque le lisier est composté. L'importance de ces pertes d'azote peut cependant varier fortement selon les techniques utilisées et la nature des substrats. Le traitement biologique abat la majeure partie de l'azote et certains types de stations permettent de capter le reste dans des coproduits potentiellement exportables. La bonne valorisation agronomique des effluents nécessite de bien connaître leur valeur fertilisante azotée, ce que facilite l'approche typologique mise au point dans le cadre du programme. Un certain nombre de leviers alimentaires permettent de diminuer la fraction du phosphore alimentaire qui est excrétée dans les effluents: ajustement des apports alimentaires grâce à une meilleure connaissance des besoins des animaux, amélioration de la digestibilité du phosphore alimentaire par une meilleure connaissance de sa disponibilité dans les diverses matières premières ou via l'adjonction de phytase exogène. Les traitements biologiques avec séparation de phases permettent de capter le phosphore dans des coproduits potentiellement exportables et la valeur fertilisante phosphatée des effluents est en général très élevée et facile à prédire. Les éléments traces métalliques (cuivre et zinc) sont souvent ajoutés dans l'aliment à des concentrations dépassant largement les besoins stricts des animaux (pour éviter les carences) afin de bénéficier de leur effet protecteur vis-à-vis des pathologies digestives. La supplémentation par des éléments traces métalliques est utile pendant la phase de post-sevrage, mais pas au-delà et l'adjonction de phytase microbienne à l'aliment améliore la disponibilité du zinc pour l'animal. Les traitements biologiques avec séparation de phases permettent de capter le zinc et le cuivre dans des coproduits potentiellement exportables. Après épandage, les éléments traces métalliques sont peu mobiles dans le sol alors que les apports au sol excèdent en général largement les capacités d'exportation par les plantes, ce qui peut conduire à des situations de phytotoxicité à plus ou moins long terme. En fin de compte, la manière la plus simple et la plus économique de gérer les effluents d'élevage reste d'utiliser au mieux leur valeur fertilisante, ce qui s'obtient par un bon équilibre entre la quantité d'animaux produits et la capacité des sols à recevoir leurs effluents. En l'absence d'un tel équilibre, l'abattement de l'azote excédentaire par l'utilisation de litières ou par le compostage du lisier a un impact environnemental important alors même que ces solutions ne résolvent rien en termes de phosphore et d'éléments traces métalliques. Les traitements biologiques les plus sophistiqués permettent d'éliminer le phosphore et une partie des éléments traces métalliques dans des coproduits potentiellement exportables, mais ils ont un coût économique et écologique très élevé. (Résumé d'auteur
New evidence of a mitochondrial genetic background paradox: Impact of the J haplogroup on the A3243G mutation
International audienceBackground: The A3243G mutation in the tRNALeu gene (UUR), is one of the most common pathogenic mitochondrial DNA (mtDNA) mutations in France, and is associated with highly variable and heterogeneous disease phenotypes. To define the relationships between the A3243G mutation and mtDNA backgrounds, we determined the haplogroup affiliation of 142 unrelated French patients – diagnosed as carriers of the A3243G mutation – by control-region sequencing and RFLP survey of their mtDNAs. Results: The analysis revealed 111 different haplotypes encompassing all European haplogroups, indicating that the 3243 site might be a mutational hot spot. However, contrary to previous findings, we observed a statistically significant underepresentation of the A3243G mutation on haplogroup J in patients (p = 0.01, OR = 0.26, C.I. 95%: 0.08–0.83), suggesting that might be due to a strong negative selection at the embryo or germ line stages. Conclusion: Thus, our study supports the existence of mutational hotspot on mtDNA and a "haplogroup J paradox," a haplogroup that may increase the expression of mtDNA pathogenic mutations, but also be beneficial in certain environmental contexts
OPA1-related dominant optic atrophy is not strongly influenced by mitochondrial DNA background
<p>Abstract</p> <p>Background</p> <p>Leber's hereditary optic neuropathy (LHON) and autosomal dominant optic atrophy (ADOA) are the most frequent forms of hereditary optic neuropathies. LHON is associated with mitochondrial DNA (mtDNA) mutations whereas ADOA is mainly due to mutations in the OPA1 gene that encodes a mitochondrial protein involved in the mitochondrial inner membrane remodeling. A striking influence of mtDNA haplogroup J on LHON expression has been demonstrated and it has been recently suggested that this haplogroup could also influence ADOA expression. In this study, we have tested the influence of mtDNA backgrounds on OPA1 mutations.</p> <p>Methods</p> <p>To define the relationships between OPA1 mutations and mtDNA backgrounds, we determined the haplogroup affiliation of 41 French patients affected by OPA1-related ADOA by control-region sequencing and RFLP survey of their mtDNAs.</p> <p>Results</p> <p>The comparison between patient and reference populations did not revealed any significant difference.</p> <p>Conclusion</p> <p>Our results argue against a strong influence of mtDNA background on ADOA expression. These data allow to conclude that OPA1 could be considered as a "severe mutation", directly responsible of the optic atrophy, whereas OPA1-negative ADOA and LHON mutations need an external factor(s) to express the pathology (i.e. synergistic interaction with mitochondrial background).</p
Mutations in TUBG1, DYNC1H1, KIF5C and KIF2A cause malformations of cortical development and microcephaly.
International audienceThe genetic causes of malformations of cortical development (MCD) remain largely unknown. Here we report the discovery of multiple pathogenic missense mutations in TUBG1, DYNC1H1 and KIF2A, as well as a single germline mosaic mutation in KIF5C, in subjects with MCD. We found a frequent recurrence of mutations in DYNC1H1, implying that this gene is a major locus for unexplained MCD. We further show that the mutations in KIF5C, KIF2A and DYNC1H1 affect ATP hydrolysis, productive protein folding and microtubule binding, respectively. In addition, we show that suppression of mouse Tubg1 expression in vivo interferes with proper neuronal migration, whereas expression of altered γ-tubulin proteins in Saccharomyces cerevisiae disrupts normal microtubule behavior. Our data reinforce the importance of centrosomal and microtubule-related proteins in cortical development and strongly suggest that microtubule-dependent mitotic and postmitotic processes are major contributors to the pathogenesis of MCD
Complete exon sequencing of all known Usher syndrome genes greatly improves molecular diagnosis
<p>Abstract</p> <p>Background</p> <p>Usher syndrome (USH) combines sensorineural deafness with blindness. It is inherited in an autosomal recessive mode. Early diagnosis is critical for adapted educational and patient management choices, and for genetic counseling. To date, nine causative genes have been identified for the three clinical subtypes (USH1, USH2 and USH3). Current diagnostic strategies make use of a genotyping microarray that is based on the previously reported mutations. The purpose of this study was to design a more accurate molecular diagnosis tool.</p> <p>Methods</p> <p>We sequenced the 366 coding exons and flanking regions of the nine known USH genes, in 54 USH patients (27 USH1, 21 USH2 and 6 USH3).</p> <p>Results</p> <p>Biallelic mutations were detected in 39 patients (72%) and monoallelic mutations in an additional 10 patients (18.5%). In addition to biallelic mutations in one of the USH genes, presumably pathogenic mutations in another USH gene were detected in seven patients (13%), and another patient carried monoallelic mutations in three different USH genes. Notably, none of the USH3 patients carried detectable mutations in the only known USH3 gene, whereas they all carried mutations in USH2 genes. Most importantly, the currently used microarray would have detected only 30 of the 81 different mutations that we found, of which 39 (48%) were novel.</p> <p>Conclusions</p> <p>Based on these results, complete exon sequencing of the currently known USH genes stands as a definite improvement for molecular diagnosis of this disease, which is of utmost importance in the perspective of gene therapy.</p
Vaines et préalables poursuites contre la société et titularité de l'action contre l'associé
International audienceNote sous Cour de cassation (com.) 24 janvier 2006, SCI Azur investissement Holding c/ Société CDR Créances, RTD com. 2006, p. 435, obs. M.-H. Monsérié-Bon et L. Grosclaude, D. 2006, p. 445, obs. A. Lienhar
Rôle et place des mécanismes fondamentaux du droit civil en droit des affaires
International audienceDe l'inutilité du droit contractuel pour assurer le respect des règles de march
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