611 research outputs found
L'IMITATION, MOTEUR DE LA RÉGLEMENTATION FINANCIÈRE ? L'EXEMPLE DE L'ADOPTION DU CO-COMMISSARIAT AUX COMPTES EN TUNISIE
Comment peut-on expliquer la diffusion internationale des règles juridiques destinée à renforcer la sécurité financière ? Dans un contexte économique caractérisé par la globalisation des échanges, l'imitation nous permet-elle d'expliquer et de comprendre l'adoption des règles juridiques dans le cadre particulier du droit financier ? Afin de répondre à ces questions et de tester la valeur heuristique du principe de l'imitation comme loi de diffusion du droit, nous étudions l'exemple de l'adoption du co-commissariat aux comptes en Tunisie.Règle juridique, Sécurité financière
An observational, non-interventional, multicenter, multinational registry of patients with atypical hemolytic uremic syndrome: initial patient characteristics
An observational, non-interventional, multicenter, multinational registry of patients with atypical hemolytic uremic syndrome: methodology
Complement System Part I – Molecular Mechanisms of Activation and Regulation
Complement is a complex innate immune surveillance system, playing a key role in defense against pathogens and in host homeostasis. The complement system is initiated by conformational changes in recognition molecular complexes upon sensing danger signals. The subsequent cascade of enzymatic reactions is tightly regulated to assure that complement is activated only at specific locations requiring defense against pathogens, thus avoiding host tissue damage. Here we discuss the recent advances describing the molecular and structural basis of activation and regulation of the complement pathways and their implication on physiology and pathology. This article will review the mechanisms of activation of alternative, classical and lectin pathways, the formation of C3 and C5 convertases, the action of anaphylatoxins and the membrane attack complex. We will also discuss the importance of structure-function relationships using the example of atypical hemolytic uremic syndrome. Lastly we will discuss the development and benefits of therapies using complement inhibitors
Rescue at sea and the establishment of jurisdiction : new direction from the human rights committee? Part I
On 27 January 2021, the United Nations Human Rights Committee (HRC or Committee) found that Italy had failed to protect the right to life of more than 200 migrants who had perished in a shipwreck in 2013. The decision in A.S., D.I., O.I. and G.D. v. Italy marks a significant development in the interpretation and application of extraterritorial jurisdiction in rescue operations on the high seas. For the first time, a human rights body has affirmed that the relationship of dependency between persons in distress and States receiving calls for assistance is sufficient to trigger extraterritorial jurisdiction and consequent application of the International Covenant on Civil and Political Rights (ICCPR). In this note, we will examine the claims of the complainant, the findings of the HRC, and will close by addressing the ramifications of the decision on human rights protection at sea.peer-reviewe
Complement System Part II: Role in Immunity
International audienceThe complement system has been considered for a long time as a simple lytic cascade, aimed to kill bacteria infecting the host organism. Nowadays, this vision has changed and it is well accepted that complement is a complex innate immune surveillance system, playing a key role in host homeostasis, inflammation, and in the defense against pathogens. This review discusses recent advances in the understanding of the role of complement in physiology and pathology. It starts with a description of complement contribution to the normal physiology (homeostasis) of a healthy organism, including the silent clearance of apoptotic cells and maintenance of cell survival. In pathology, complement can be a friend or a foe. It acts as a friend in the defense against pathogens, by inducing opsonization and a direct killing by C5b–9 membrane attack complex and by triggering inflammatory responses with the anaphylatoxins C3a and C5a. Opsonization plays also a major role in the mounting of an adaptive immune response, involving antigen presenting cells, T-, and B-lymphocytes. Nevertheless, it can be also an enemy, when pathogens hijack complement regulators to protect themselves from the immune system. Inadequate complement activation becomes a disease cause, as in atypical hemolytic uremic syndrome, C3 glomerulopathies, and systemic lupus erythematosus. Age-related macular degeneration and cancer will be described as examples showing that complement contributes to a large variety of conditions, far exceeding the classical examples of diseases associated with complement deficiencies. Finally, we discuss complement as a therapeutic target
Irregular maritime migration in context
Irregular maritime migration presents a conceptual challenge
to States: security interests and the sovereign right of a State to
control access to its territory come face to face with fundamental
principles of protection of persons. Individuals will always seek to
leave their own countries and enter States in an irregular manner, be
they persons who are attempting to flee conflict, persecution or
natural disasters, as well as those seeking to circumvent migration
and border controls, often in order to improve their economic
circumstances. In this area, therefore, the rights of States and duties
of those same States towards individuals meet and often collide.
Apart from conflicting rights and duties, one is also aware of a variety
of legal regimes applying to the same factual phenomenon which is
notoriously difficult to control. [Excerpt]peer-reviewe
Autoantibodies Against C3b—Functional Consequences and Disease Relevance
The complement component C3 is at the heart of the complement cascade. It is a complex protein, which generates different functional activated fragments (C3a, C3b, iC3b, C3c, C3d). C3b is a constituent of the alternative pathway C3 convertase (C3bBb), binds multiple regulators, and receptors, affecting thus the functioning of the immune system. The activated forms of C3 are a target for autoantibodies. This review focuses on the discovery, disease relevance, and functional consequences of the anti-C3b autoantibodies. They were discovered about 70 years ago and named immunoconglutinins. They were found after infections and considered convalescent factors. At the end of the twentieth century IgG against C3b were found in systemic lupus erythematosus and recently in lupus nephritis, correlating with the disease severity and flare. Cases of C3 glomerulopathy and immune complex glomerulonephritis were also reported. These antibodies recognize epitopes, shared between C3(H2O)/C3b/iC3b/C3c and have overt functional activity. They correlate with low plasmatic C3 levels in patients. In vitro, they increase the activity of the alternative pathway C3 convertase, without being C3 nephritic factors. They perturb the binding of the negative regulators Complement Receptor 1 and Factor H. The clear functional consequences and association with disease severity warrant further studies to establish the link between the anti-C3b autoantibodies and tissue injury. Comparative studies with such antibodies, found in patients with infections, may help to uncover their origin and epitopes specificity. Patients with complement overactivation due to presence of anti-C3b antibodies may benefit from therapeutic targeting of C3
Complement Factor H Gene Abnormalities in Haemolytic Uraemic Syndrome: From Point Mutations to Hybrid Gene
Noris and Remuzzi discuss a new study showing an association between atypical haemolytic uremic syndrome and a hybrid complement gene,CFH/CFHL1
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