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Academic literature on the topic 'Voie alterne d'activation du complément'
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Journal articles on the topic "Voie alterne d'activation du complément"
Bouillet, L., D. Ponard, and C. Massot. "Lipodystrophie céphalothoracique et activation de la voie alterne du complément." La Revue de Médecine Interne 24 (December 2003): 470s. http://dx.doi.org/10.1016/s0248-8663(03)80569-6.
Full textLanglois, A. L., V. Frémeaux-Bacchi, L. Mouthon, Y. Delmas, J. M. Halimi, A. Pruna, M. Ficheux, Q. Raimbourg, E. Thervet, and A. Karras. "Implication de la voie alterne du complément dans la crise rénale sclérodermie." Néphrologie & Thérapeutique 12, no. 5 (September 2016): 344. http://dx.doi.org/10.1016/j.nephro.2016.07.165.
Full textBoussier, J., N. Yatim, A. Marchal, J. Hadjadj, B. Charbit, C. El Sissy, N. Carlier, et al. "Activation de la voie alterne du complément dans les formes sévères de COVID-19." La Revue de Médecine Interne 42 (December 2021): A324. http://dx.doi.org/10.1016/j.revmed.2021.10.286.
Full textBauvois, Adeline, Mélusine Larivière, Hervé Watier, and François Maillot. "Actualités des anticorps monoclonaux dans les maladies monogéniques aujourd’hui." médecine/sciences 35, no. 12 (December 2019): 1026–28. http://dx.doi.org/10.1051/medsci/2019203.
Full textCartier, J. C., A. S. Truche, L. Croze, C. Brunelle, D. Ponard, P. L. Carron, and P. Zaoui. "Activation de la voie alterne du complément au cours de la microangiopathie thrombotique associée à la maladie de Still de l’adulte." Néphrologie & Thérapeutique 8, no. 5 (September 2012): 339–40. http://dx.doi.org/10.1016/j.nephro.2012.07.104.
Full textMarchal, A., J. Zuber, S. Limou, P. Veira Martins, A. Le Clech, C. Leman, E. Rondeau, F. Fakhouri, and V. Frémeaux-Bacchi. "Impact des variants rares des gènes de la voie alterne du complément dans la susceptibilité génétique au syndrome hémolytique et urémique atypique." Néphrologie & Thérapeutique 16, no. 5 (September 2020): 314. http://dx.doi.org/10.1016/j.nephro.2020.07.175.
Full textLepeytre, F., K. Dahan, E. Plaisier, P. A. Michel, C. Morbieu, M. C. Verpont, H. Debiec, V. Frémeaux-Bacchi, I. Brocheriou, and P. Ronco. "Association atypique d’une micro-angiopathie thrombotique et d’une glomérulonéphrite familiale à dépôts de C3 : une seule cause, la voie alterne du complément ?" Néphrologie & Thérapeutique 10, no. 2 (April 2014): 134. http://dx.doi.org/10.1016/j.nephro.2014.01.005.
Full textJullien, P., P. Vieira Martins, G. Claisse, M. Dinic, I. Masson, E. Alamartine, C. Mariat, V. Fremeaux Bacchi, and N. Maillard. "Fréquence des variants rares des gènes de la voie alterne du complément d’une population de néphropathie à IgA ayant évolué vers l’insuffisance rénale chronique terminale." Néphrologie & Thérapeutique 14, no. 5 (September 2018): 348. http://dx.doi.org/10.1016/j.nephro.2018.07.221.
Full textMaillard, N., V. Fremeaux-Bacchi, E. Alamartine, and C. Mariat. "Le séquençage des gènes de la voie alterne du complément d’une cohorte de néphropathie à IgA sévère révèle une fréquence élevée de variants rares de CFH et une sur-représentation d’un variant pathogène du gène de la thrombomoduline." Néphrologie & Thérapeutique 16, no. 5 (September 2020): 256. http://dx.doi.org/10.1016/j.nephro.2020.07.023.
Full textDissertations / Theses on the topic "Voie alterne d'activation du complément"
Meuleman, Marie-Sophie. "Déterminants et conséquences intra-rénales de la dérégulation du complément au cours de la glomérulopathie à dépôts de C3." Electronic Thesis or Diss., Sorbonne université, 2024. https://accesdistant.sorbonne-universite.fr/login?url=https://theses-intra.sorbonne-universite.fr/2024SORUS260.pdf.
Full textC3 glomerulopathy (C3G) is a rare glomerulopathy resulting from dysregulation of the complement alternative pathway (AP), particularly involving the two key enzymes of the cascade: the C3 and C5 convertases. Most often, it is secondary to the acquisition of an autoantibody targeting the alternative C3 convertase. More rarely, pathogenic variants are identified in the genes encoding the components of the alternative C3 convertase (C3 and FB) or the main regulators of the AP (FH and FI).The clinical, histological, and immunological presentation (biomarker profile indicating complement activation) is heterogeneous. Half of the patients progress to end-stage renal disease within 10 years; the main prognostic factors for renal survival are clinical and histological and are not specific of C3G.In the first part, we characterized the genetic abnormalities of complement proteins within the French cohort of C3G, in relation to clinical presentation, biological profile, and renal prognosis. Among the 398 patients who underwent genetic exploration, 66 (17%) had a rare variant in CFH, CFI, or C3 (75% classified as pathogenic). Aside from the presence of biological thrombotic microangiopathy, which was more frequent in those with CFI variants, the clinical presentation was not different between groups with CFH, CFI, or C3 variants. Patients with CFH and CFI variants had an increased frequency of quantitative deficiency in FH and FI, without differences in the levels of C3, C4, and sC5b-9. The renal prognosis was poorer in the group with variants (CFH, CFI, or C3) compared to patients without variants.In the second part, we explored the link between the profile of convertase activation and intrarenal immune response. In a first C3G cohort (n=42), we demonstrated that the majority had intrarenal activation of the complement terminal pathway (TP), reflected by C5b-9 deposits. The extent of activation correlated with histological presentation and renal prognosis (poorer prognosis in patients with the most significant activation). A bulk transcriptomic analysis of renal biopsies identified 301/847 upregulated immune genes in C3G compared to controls. From these data, unsupervised clustering identified four groups. One group was characterized by enrichment in immune and fibroblastic populations (deconvoluted), more intrarenal TP activation, significant histological chronicity, and poorer renal survival.In a second C3G cohort (n=47), we demonstrated compartmentalization of the intrarenal immune infiltrate with a majority of neutrophils and macrophages in the glomeruli versus a majority of B cells, T cells, and macrophages in the interstitium. Patients with a neutrophil-rich glomerular infiltrate exhibited more biological markers of systemic AP and TP activation, greater intrarenal TP activation, and entered remission more quickly than those with low glomerular neutrophil density. Conversely, there was no association between the extent of the interstitial immune infiltrate and the complement activation profile. Neutrophil-rich glomeruli showed overexpression of genes involved in neutrophil recruitment and activation and in acquiring a mesangial profibroblastic phenotype (spatial transcriptomics). With the emergence of complement inhibitory therapies, better understanding the link between complement activation profile, intrarenal immune response, and phenotypic expression of the pathology paves the way for personalized management of these patients
Julen, Nathalie. "Etude de l'expression des protéines de la voie alterne du complément (facteur H, C3 et facteur B) lors de l'inflammation." Rouen, 1990. http://www.theses.fr/1990ROUES043.
Full textFrimat, Marie. "Lésions endothéliales liées à un défaut de contrôle du complément : de la génétique du complément au syndrome hémolytique et urémique." Thesis, Paris 5, 2013. http://www.theses.fr/2013PA05T067/document.
Full textPas de résumé en anglais
Dauchel, Hélène. "Le système du complément et la cellule endothéliale : biosynthèse in vitro des protéines de la voie alterne et activation pathologique au cours des vascularités leucocytoclasiques." Rouen, 1993. http://www.theses.fr/1993ROUES032.
Full textLe, Mignon Maxime. "Régulation de la voie alterne du complément par le facteur H : élucidation de son assemblage avec C3b par marquage oxydatif et pontage chimique couplés à la spectrométrie de masse." Thesis, Evry-Val d'Essonne, 2010. http://www.theses.fr/2010EVRY0006.
Full textThe C3b-H complex plays an essential role in regulation of the complement alternative pathway (AP) because it blocks the attack membrane complex (MAC) formation on our self-surface, thus preserving host from autoimmune disease. The C3b-H interaction leads to the proteolytic inactivation of C3b induced by factor I, thus blocking the MAC formation and consequently AP activation. Despite this importance of C3b-H complex for the innate immunity, the mechanism of interaction between these two molecules at the molecular level remains to be established. Indeed, up to date, no three-dimensional structure of this complex is available, either by X-ray crystallography or nuclear magnetic resonance, due to its large size (335 kg.mol-1) and flexibility of H. Yet, these data are required for a better understanding of AP regulation and, might moreover, be useful for the implementation of therapy against autoimmune disorders. In this study, we investigate the C3b-H complex by using oxidative footprinting and cross-linking coupled with mass spectrometry. Our experimental approaches have been successfully applied, allowing us to propose a three-dimensional model of this high molecular complex
Chauvet, Sophie. "Rôles des facteurs génétiques et acquis dans la physiopathologie des glomérulopathies à dépôts de C3." Thesis, Sorbonne Paris Cité, 2016. http://www.theses.fr/2016USPCB081/document.
Full textComplement alternative pathway is physiologically activated. It need to be tightly regulated to avaoid uncontrolled deleterious overactivation on host cell surface. In human, two renal diseaes are associated with uncontrolled AP activation, hemolytic uremic syndrom atypical (aHUS) and C3 glomerulopathy (C3G). C3G occures mainly in children and young adults and regoups two distinct histopathological entities, dense deposit disease (DDD) and C3 glomerulonephritis (C3GN). Renal outcomes in C3G is poor since up to 50% of patients reach end stage renal disease 8 to 10 years after diagnosis. Complement abnormalities in C3G are mainly acquired induced by the presence of C3 Nephritic Factor (C3NeF): an autoantibdy targeting the AP C3 convertase. Less frequently C3G patients have anti-FH autoantibodies (Ref) or genetic abnormalities (variants in the FH, FI or CFHR5 genes. In silico analysis of mutated proteins give information about the role of mutation on AP overactivation. However, characterization of functional consequences of these mutations is required to proved the direct link between the abnormality and the occurrence of C3G. I first studied the functional consequences of the first C3 mutation, C3I734T, identified in a familial C3GN. In silico analysis revealed that the mutated residue, T734, is located on the C3 and C3b protein surface and that the substitution I734T may not be associated with major structural changes. The mutated amino acid is located on interaction site between C3b and complement regulatory proteins, FH and CR1. In vitro, the major defect of C3I734T was a decrease in binding to CR1, resulting in lower CR1 dependent cleavage of C3b by FI. These results provide evidence for a CR1 functional deficiency being responsible for deficient complement regulation. Binding of C3I734T to Factor H (FH) was normal, but C3I734T was less efficiently cleaved by Factor I, leading to enhanced C3 fragments binding on glomerular cells. In the second part of my work, I studied acquired C3G in patients with concomitant monoclonal gammopathy. In the clinical part of this study, we demonstrated The high prevalence of monoclonal gammopathy in C3G patients aged over 50, reaching 65% in the French C3G national cohort, strongly suggests a pathogenic link between the two conditions. Next, we demonstrated that renal outcomes is significantly worser in patients with monoclonal gammopathy compared to patients without monoclonal gammopathy but that efficient chemotherapy resulted in higher renal response rate and longer renal survival than conservative or immunosuppressive therapy. Results of the clinical part of the study strongly suggest a link betwwen the monoclonal gammopathy and the occurrence of C3G. In the experimental part of this work, I studied the mechanisms of complement AP activation in these population. Biomarkers of C3 and C5 convertase activation were present 40% and 81% of patients respectively. Anti-complement protein antibodies were found in 23/41 (56%) patients, including in most of patients, anti-FH and anti-CR1 antibodies. I found new antigenic target, C5 and properdin in few cases. The anti-FH and anti-CR1 antibodies were associated with clear functional consequences. Nevertheless, the anti-complement proteins reactivity was not carried out by the MIg in 75% of the cases. I discovered that the MIg induced a direct AP C3 convertase overactivation in 23/34 (67%) patients responsaible for a C5 convertase overactivation in presence of patients’ Ig, in a properdin dependant manner. Our results suggest that MIg and polyclonal autoantibodies could act in synergy: AP overactivation induced by the MIg could be amplified by the inefficient complement regulation, caused by the polyclonal anti-complement autoantibodies. All my results allow to better understand the pathophysiological mechanisms involved in C3G and open up reflection on therapeutic approaches for C3G associated with monoclonal gammopathy
Gasque, Philippe. "Expression du complément par des cellules du système nerveux central : analyse in vitro de la biosynthèse des protéines du complément par un modèle de lignées d'astrocytes humains." Rouen, 1993. http://www.theses.fr/1993ROUES038.
Full textAude, Catherine. "Etude de la structure et du mécanisme d'activation de C1s-C1r-C1r-C1s, sous-unité catalytique de C1, premier composant de la voie classique du complément." Grenoble 1, 1988. http://www.theses.fr/1988GRE10143.
Full textDémares, Marie-José. "Etude de l'expression du facteur H du complément par les leucocytes humains." Rouen, 1987. http://www.theses.fr/1987ROUES053.
Full textGarnier, Gérard. "Le facteur B du complément humain : bases structurales et biosynthétiques de sa microhétérogénéité, mises en évidence par les techniques électrophorétiques." Rouen, 1988. http://www.theses.fr/1988ROUES026.
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