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2. FDX2 and ISCU Gene Variations Lead to Rhabdomyolysis With Distinct Severity and Iron Regulation

Author

Montealegre, Sebastian, Lebigot, Elise, Debruge, Hugo, Romero, Norma, Héron, Bénédicte, Gaignard, Pauline, Legendre, Antoine, Imbard, Apolline, Gobin, Stéphanie, Lacène, Emmanuelle, Nusbaum, Patrick, Hubas, Arnaud, Desguerre, Isabelle, Servais, Aude, Laforêt, Pascal, van Endert, Peter, Authier, François Jérome, Gitiaux, Cyril, and de Lonlay, Pascale

Published
2022
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3. TANGO2-related rhabdomyolysis symptoms are associated with abnormal autophagy functioning

Author

de Calbiac, Hortense, primary, Montealegre, Sebastian, additional, Straube, Marjolène, additional, Renault, Solène, additional, Debruge, Hugo, additional, Chentout, Loïc, additional, Ciura, Sorana, additional, Imbard, Apolline, additional, Le Guillou, Edouard, additional, Marian, Anca, additional, Goudin, Nicolas, additional, Caccavelli, Laure, additional, Fabrega, Sylvie, additional, Hubas, Arnaud, additional, van Endert, Peter, additional, Dupont, Nicolas, additional, Diana, Julien, additional, Kabashi, Edor, additional, and de Lonlay, Pascale, additional

Published
2024
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4. Innate Immune Signals Induce Anterograde Endosome Transport Promoting MHC Class I Cross-Presentation

Author

Weimershaus, Mirjana, Mauvais, François-Xavier, Saveanu, Loredana, Adiko, Cézaire, Babdor, Joël, Abramova, Anastasia, Montealegre, Sebastian, Lawand, Myriam, Evnouchidou, Irini, Huber, Katharina Julia, Chadt, Alexandra, Zwick, Markus, Vargas, Pablo, Dussiot, Michael, Lennon-Dumenil, Ana Maria, Brocker, Thomas, Al-Hasani, Hadi, and van Endert, Peter

Published
2018
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6. Estudios topográficos y de afectación de perfil vial, que contribuyen a la legalización del barrio Villa Suarez, en la comuna 4 del Municipio de Villavicencio – Meta

Author

Ruiz Echeverry, Oscar Ivan, Torres Guarin, Paula Alejandra, Martinez Montealegre, Sebastian Jefred, Salgado Diaz, Juan Manuel, and Universidad Santo Tomás

Subjects
Topography, Viabilidad, Afectación vial, Ingeniería civil - Investigación, Legalización, POT, Topografía, Tesis y disertaciones académicas, Vías, Viability, Affectation by flood, Ordenamiento territorial - POT, Human Settlement, Asentamientos urbanos - Vías, Legalization, Road Impact, Afectación por inundación, Asentamiento Humano
Abstract

A través de los años la guerra en Colombia ha generado miles de desplazados y el departamento del Meta y su capital Villavicencio no han sido ajenos a esta situación, debido a que por este motivo y otros más, los colombianos de diferentes partes del país han ansiado escapar de su realidad en busca de nuevas oportunidades, repercutiendo en el Plan de Ordenamiento Territorial (POT); al producirse un crecimiento poblacional no controlado, donde la ciudad crece hacia su periferia, conllevando a que Villavicencio tenga más del 40% de sus barrios sin legalizar y como consecuencia, estos asentamientos humanos no pueden ser beneficiados con la intervención de la inversión pública y se vea afectada su calidad de vida. De esta forma, la Universidad Santo Tomas a través de Proyección Social de la Facultad de Ingeniería Civil pretende junto con la Alcaldía Municipal, realizar proyectos que contribuyan a la legalización de algunos asentamientos como Villa Suarez, el cual es el centro de estudio de este proyecto de investigación. Villa Suarez cuenta con dos problemáticas para poder ser legalizado, los cuales son por afectación vial y por inundación, ya que algunos de sus habitantes se expandieron hacia la ronda hídrica del rio, poniendo sus vidas en riesgo; por ende, el presente proyecto llevará a cabo estudios y análisis que contribuyan con la viabilidad de legalizar dicho asentamiento. Over the years the war in Colombia has generated thousands of displaced people and the department of Meta and its capital Villavicencio have not been alien to this situation, because for this reason and others, Colombians from different parts of the country have longed to escape from their reality in search of new opportunities, affecting the Land Management Plan (POT); As a result, Villavicencio has more than 40% of its neighborhoods without legalization and as a consequence, these human settlements cannot benefit from the intervention of public investment and their quality of life is affected. In this way, the Santo Tomas University through the Social Projection of the Faculty of Civil Engineering intends, together with the Municipal Mayor's Office, to carry out projects that contribute to the legalization of some settlements such as Villa Suarez, which is the focus of study of this research project. Villa Suarez has two problems to be legalized, which are due to road affectation and flooding, since some of its inhabitants expanded towards the river's hydrological round, putting their lives at risk; therefore, this project will carry out studies and analysis that contribute to the feasibility of legalizing this settlement. Ingeniero Civil http://www.ustavillavicencio.edu.co/home/index.php/unidades/extension-y-proyeccion/investigacion Pregrado

Published
2023

7. Abnormal autophagy is a critical mechanism in TANGO2-related rhabdomyolysis

Author

Montealegre, Sebastian, primary, de Calbiac, Hortense, additional, Straube, Marjolène, additional, Debruge, Hugo, additional, Chentout, Loïc, additional, Ciura, Sorana, additional, Imbard, Apolline, additional, Le Guillou, Edouard, additional, Marian, Anca, additional, Goudin, Nicolas, additional, Caccavelli, Laure, additional, Fabrega, Sylvie, additional, Hubas, Arnaud, additional, van Endert, Peter, additional, Dupont, Nicolas, additional, Diana, Julien, additional, Kabashi, Edor, additional, and de Lonlay, Pascale, additional

Published
2023
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10. Clinical and biological characterization of 20 patients with TANGO2 deficiency indicates novel triggers of metabolic crises and no primary energetic defect

Author

Bérat, Claire‐Marine, primary, Montealegre, Sebastian, additional, Wiedemann, Arnaud, additional, Nuzum, Malou Le Corronc, additional, Blondel, Amélie, additional, Debruge, Hugo, additional, Cano, Aline, additional, Chabrol, Brigitte, additional, Hoebeke, Célia, additional, Polak, Michel, additional, Stoupa, Athanasia, additional, Feillet, François, additional, Torre, Stéphanie, additional, Boddaert, Nathalie, additional, Bruel, Henri, additional, Barth, Magalie, additional, Damaj, Lena, additional, Abi‐Wardé, Marie‐Thérèse, additional, Afenjar, Alexandra, additional, Benoist, Jean‐François, additional, Madrange, Marine, additional, Caccavelli, Laure, additional, Renard, Perrine, additional, Hubas, Arnaud, additional, Nusbaum, Patrick, additional, Pontoizeau, Clément, additional, Gobin, Stéphanie, additional, Endert, Peter, additional, Ottolenghi, Chris, additional, Maltret, Alice, additional, and Lonlay, Pascale, additional

Published
2020
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11. The role of MHC class I recycling and Arf6 in cross-presentation by murine dendritic cells

Author

Montealegre, Sebastian, Abramova, Anastasia, Manceau, Valerie, de Kanter, Anne-Floor, and van Endert, Peter

Subjects
Male, Genes, MHC Class I, chemical and pharmacologic phenomena, Mice, Transgenic, Endoplasmic Reticulum, Mice, Cross-Priming, Phagocytosis, Animals, Antigens, Research Articles, Antigen Presentation, Mice, Inbred BALB C, ADP-Ribosylation Factors, Histocompatibility Antigens Class I, Dendritic Cells, Endocytosis, Mice, Inbred C57BL, Protein Transport, ADP-Ribosylation Factor 6, rab GTP-Binding Proteins, Female, Lysosomes, Research Article, T-Lymphocytes, Cytotoxic
Abstract

MHC-I internalization and recycling in DCs neither requires Arf6 nor provides MHC-I to the Rab11+ endocytic recycling compartment; however, cross-presentation of immune complexes depends on Arf6., Cross-presentation by MHC class I molecules (MHC-I) is critical for priming of cytotoxic T cells. Peptides derived from cross-presented antigens can be loaded on MHC-I in the endoplasmic reticulum and in endocytic or phagocytic compartments of murine DCs. However, the origin of MHC-I in the latter compartments is poorly understood. Recently, Rab22-dependent MHC-I recycling through a Rab11+ compartment has been suggested to be implicated in cross-presentation. We have examined the existence of MHC-I recycling and the role of Arf6, described to regulate recycling in nonprofessional antigen presenting cells, in murine DCs. We confirm folded MHC-I accumulation in a juxtanuclear Rab11+ compartment and partially localize Arf6 to this compartment. MHC-I undergo fast recycling, however, both folded and unfolded internalized MHC-I fail to recycle to the Rab11+Arf6+ compartment. Therefore, the source of MHC-I molecules in DC endocytic compartments remains to be identified. Functionally, depletion of Arf6 compromises cross-presentation of immune complexes but not of soluble, phagocytosed or mannose receptor–targeted antigen, suggesting a role of Fc receptor–regulated Arf6 trafficking in cross-presentation of immune complexes.

Published
2019

12. Innate Immune Signals Induce Anterograde Endosome Transport Promoting MHC Class I Cross-Presentation

Author

Beaune, Gregory, Blanch-Mercader, Carles, Douezan, Stephane, Dumond, Julien, Gonzalez-Rodriguez, David, Cuvelier, Damien, Ondarçuhu, Thierry, Sens, Pierre, Dufour, Sylvie, Murrell, Michael, Charles-Orszag, Arthur, Tsai, Feng-Ching, Bonazzi, Daria, Manriquez, Valeria, Sachse, Martin, Mallet, Adeline, Salles, Audrey, Melican, Keira, Staneva, Ralitza, Bertin, Aurélie, Millien, Corinne, Goussard, Sylvie, Lafaye, Pierre, Shorte, Spencer, Piel, Matthieu, Krijnse-Locker, Jacomine, Brochard-Wyart, Françoise, Bassereau, Patricia, Duménil, Guillaume, Weimershaus, Mirjana, Mauvais, Francois-Xavier, Saveanu, Loredana, Adiko, Cézaire, Babdor, Joel, Abramova, Anastasia, Montealegre, Sebastian, Lawand, Myriam, Evnouchidou, Irini, Huber, Katharina Julia, Chadt, Alexandra, Zwick, Markus, Vargas, Pablo, Dussiot, Michaël, Lennon-Dumenil, Ana Maria, Brocker, Thomas, Al-Hasani, Hadi, Van Endert, Peter, National Institute for Materials Science (NIMS), Physico-Chimie-Curie (PCC), Institut Curie-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Centre National de la Recherche Scientifique (CNRS)-Institut Curie-Université Pierre et Marie Curie - Paris 6 (UPMC), Centre d'élaboration de matériaux et d'études structurales (CEMES), Institut National des Sciences Appliquées - Toulouse (INSA Toulouse), Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Institut de Chimie de Toulouse (ICT-FR 2599), Institut National Polytechnique (Toulouse) (Toulouse INP), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut de Recherche pour le Développement (IRD)-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Centre National de la Recherche Scientifique (CNRS)-Institut National Polytechnique (Toulouse) (Toulouse INP), Université Fédérale Toulouse Midi-Pyrénées-Centre National de la Recherche Scientifique (CNRS)-Centre National de la Recherche Scientifique (CNRS), Biologie des Organismes et Ecosystèmes Aquatiques (BOREA), Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-Muséum national d'Histoire naturelle (MNHN)-Institut de Recherche pour le Développement (IRD)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS)-Université des Antilles (UA), Paris-Centre de Recherche Cardiovasculaire (PARCC - UMR-S U970), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Institut Jacques Monod (IJM (UMR_7592)), Centre National de la Recherche Scientifique (CNRS)-Université Paris Diderot - Paris 7 (UPD7), Pathogénèse des Infections vasculaires, Institut Pasteur [Paris]-Institut National de la Santé et de la Recherche Médicale (INSERM), Microscopie ultrastructurale (plate-forme), Institut Pasteur [Paris], Imagopole (CITECH), Centre d'Immunologie de Marseille - Luminy (CIML), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Department of Neuroscience, Karolinska Institutet [Stockholm], Institut Curie, SAFT [Bordeaux], Société des accumulateurs fixes et de traction (SAFT), Ingénierie des Anticorps (plate-forme) - Antibody Engineering (Platform), Institut Pasteur [Paris]-Centre National de la Recherche Scientifique (CNRS), Compartimentation et dynamique cellulaires (CDC), Microscopie ultrastructurale - Ultrapole (CITECH), Diabète de Type 1 : mécanismes et traitements immunologiques, Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut Necker Enfants-Malades (INEM - UM 111 (UMR 8253 / U1151)), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre de recherche sur l'Inflammation (CRI (UMR_S_1149 / ERL_8252 / U1149)), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre de Recherche des Cordeliers (CRC), Université Paris Diderot - Paris 7 (UPD7)-École pratique des hautes études (EPHE)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Immunité et cancer, Institut Curie-Institut National de la Santé et de la Recherche Médicale (INSERM), Hémostase, bio-ingénierie et remodelage cardiovasculaires (LBPC), Université Paris 13 (UP13)-Université Paris Diderot - Paris 7 (UPD7)-Institut Galilée-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Diderot - Paris 7 (UPD7), Université Paris Diderot - Paris 7 (UPD7)-École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Curie [Paris], Université Paris Diderot - Paris 7 (UPD7)-Université Paris 13 (UP13)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Galilée, and Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Diderot - Paris 7 (UPD7)-Centre National de la Recherche Scientifique (CNRS)

Subjects
0301 basic medicine, Male, Endosome, Antigen presentation, Fc receptor, Kinesins, Endosomes, Receptors, Fc, [SDV.BC.BC]Life Sciences [q-bio]/Cellular Biology/Subcellular Processes [q-bio.SC], Microtubules, General Biochemistry, Genetics and Molecular Biology, 03 medical and health sciences, Mice, Cross-Priming, Phagosomes, Animals, lcsh:QH301-705.5, Cells, Cultured, ComputingMilieux_MISCELLANEOUS, Phagosome, Innate immune system, biology, Chemistry, Histocompatibility Antigens Class I, Cross-presentation, Dendritic cell, Immunity, Innate, Cell biology, Toll-Like Receptor 4, Protein Transport, 030104 developmental biology, lcsh:Biology (General), rab GTP-Binding Proteins, biology.protein, Kinesin, Female
Abstract

Summary: Both cross-presentation of antigens by dendritic cells, a key pathway triggering T cell immunity and immune tolerance, and survival of several pathogens residing in intracellular vacuoles are intimately linked to delayed maturation of vesicles containing internalized antigens and microbes. However, how early endosome or phagosome identity is maintained is incompletely understood. We show that Toll-like receptor 4 (TLR4) and Fc receptor ligation induces interaction of the GTPase Rab14 with the kinesin KIF16b mediating plus-end-directed microtubule transport of endosomes. As a result, Rab14 recruitment to phagosomes delays their maturation and killing of an internalized pathogen. Enhancing anterograde transport by overexpressing Rab14, promoting the GTP-bound Rab14 state, or inhibiting retrograde transport upregulates cross-presentation. Conversely, reducing Rab14 expression, destabilizing Rab14 endosomes, and inhibiting anterograde microtubule transport by Kif16b knockdown compromise cross-presentation. Therefore, regulation of early endosome trafficking by innate immune signals is a critical parameter in cross-presentation by dendritic cells. : Weimershaus et al. identify a molecular complex that controls the intracellular trafficking along microtubules of antigens internalized by dendritic cells. They show that this trafficking is regulated by innate immune signals and regulates presentation of internalized antigens to T lymphocytes. Keywords: antigen presentation, cross-presentation, dendritic cell, MHC class I, endosome, small GTPase, kinesin, Rab14

Published
2018
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14. Clinical and biological characterization of 20 patients with TANGO2 deficiency indicates novel triggers of metabolic crises and no primary energetic defect.

Author

Bérat, Claire‐Marine, Montealegre, Sebastian, Wiedemann, Arnaud, Nuzum, Malou Le Corronc, Blondel, Amélie, Debruge, Hugo, Cano, Aline, Chabrol, Brigitte, Hoebeke, Célia, Polak, Michel, Stoupa, Athanasia, Feillet, François, Torre, Stéphanie, Boddaert, Nathalie, Bruel, Henri, Barth, Magalie, Damaj, Lena, Abi‐Wardé, Marie‐Thérèse, Afenjar, Alexandra, and Benoist, Jean‐François

Abstract

TANGO2 disease is a severe inherited disorder associating multiple symptoms such as metabolic crises, encephalopathy, cardiac arrhythmias, and hypothyroidism. The mechanism of action of TANGO2 is currently unknown. Here, we describe a cohort of 20 French patients bearing mutations in the TANGO2 gene. We found that the main clinical presentation was the association of neurodevelopmental delay (n = 17), acute metabolic crises (n = 17) and hypothyroidism (n = 12), with a large intrafamilial clinical variability. Metabolic crises included rhabdomyolysis (15/17), neurological symptoms (14/17), and cardiac features (12/17; long QT (n = 10), Brugada pattern (n = 2), cardiac arrhythmia (n = 6)) that required intensive care. We show previously uncharacterized triggers of metabolic crises in TANGO2 patients, such as some anesthetics and possibly l‐carnitine. Unexpectedly, plasma acylcarnitines, plasma FGF‐21, muscle histology, and mitochondrial spectrometry were mostly normal. Moreover, in patients' primary myoblasts, palmitate and glutamine oxidation rates, and the mitochondrial network were also normal. Finally, we found variable mitochondrial respiration and defective clearance of oxidized DNA upon cycles of starvation and refeeding. We conclude that TANGO2 disease is a life‐threatening disease that needs specific cardiac management and anesthesia protocol. Mechanistically, TANGO2 disease is unlikely to originate from a primary mitochondrial defect. Rather, we suggest that mitochondrial defects are secondary to strong extrinsic triggers in TANGO2 deficient patients. [ABSTRACT FROM AUTHOR]

Published
2021
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