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2. Role of IL-27 in Epstein–Barr virus infection revealed by IL-27RA deficiency

Author

Martin, Emmanuel, Winter, Sarah, Garcin, Cécile, Tanita, Kay, Hoshino, Akihiro, Lenoir, Christelle, Fournier, Benjamin, Migaud, Mélanie, Boutboul, David, Simonin, Mathieu, Fernandes, Alicia, Bastard, Paul, Le Voyer, Tom, Roupie, Anne-Laure, Ben Ahmed, Yassine, Leruez-Ville, Marianne, Burgard, Marianne, Rao, Geetha, Ma, Cindy S., Masson, Cécile, Soudais, Claire, Picard, Capucine, Bustamante, Jacinta, Tangye, Stuart G., Cheikh, Nathalie, Seppänen, Mikko, Puel, Anne, Daly, Mark, Casanova, Jean-Laurent, Neven, Bénédicte, Fischer, Alain, and Latour, Sylvain

Published
2024
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3. Somatic RAP1B gain-of-function variant underlies isolated thrombocytopenia and immunodeficiency

Author

Benavides-Nieto, Marta, Adam, Frederic, Martin, Emmanuel, Boussard, Charlotte, Lagresle-Peyrou, Chantal, Callebaut, Isabelle, Kauskot, Alexandre, Reperant, Christelle, Feng, Miao, Bordet, Jean-Claude, Castelle, Martin, Morelle, Guillaume, Brouzes, Chantal, Zarhrate, Mohammed, Panikulam, Patricia, Lambert, Nathalie, Picard, Capucine, Bodet, Damien, Rouger-Gaudichon, Jeremie, Revy, Patrick, de Villartay, Jean- Pierre, and Moshous, Despina

Subjects
Physiological aspects, Complications and side effects, Genetic aspects, Risk factors, Health aspects, Thrombocytopenia -- Physiological aspects -- Genetic aspects -- Risk factors, Immunodeficiency -- Genetic aspects -- Physiological aspects -- Complications and side effects, Medical research, Genetic variation -- Health aspects, GTPases -- Health aspects -- Genetic aspects, Medicine, Experimental, Guanosine triphosphatase -- Health aspects -- Genetic aspects
Abstract

Introduction Thanks to next-generation sequencing (NGS) approaches, the list of genetic syndromes that cause thrombocytopenia has grown in recent years and now includes over 40 genes (1). However, there are [...], The ubiquitously expressed small GTPase Ras-related protein 1B (RAP1B) acts as a molecular switch that regulates cell signaling, cytoskeletal remodeling, and cell trafficking and activates integrins in platelets and lymphocytes. The residue G12 in the P-loop is required for the RAP1B-GTPase conformational switch. Heterozygous germline RAP1B variants have been described in patients with syndromic thrombocytopenia. However, the causality and pathophysiological impact remained unexplored. We report a boy with neonatal thrombocytopenia, combined immunodeficiency, neutropenia, and monocytopenia caused by a heterozygous de novo single nucleotide substitution, c.35G>A (p.G12E) in RAP1B. We demonstrate that G12E and the previously described G12V and G60R were gain-of-function variants that increased RAP1B activation, talin recruitment, and integrin activation, thereby modifying late responses such as platelet activation, T cell proliferation, and migration. We show that in our patient, G12E was a somatic variant whose allele frequency decreased over time in the peripheral immune compartment, but remained stable in bone marrow cells, suggesting a differential effect in distinct cell populations. Allogeneic hematopoietic stem cell transplantation fully restored the patient's hemato-immunological phenotype. Our findings define monoallelic RAP1B gain-of-function variants as a cause for constitutive immunodeficiency and thrombocytopenia. The phenotypic spectrum ranged from isolated hematological manifestations in our patient with somatic mosaicism to complex syndromic features in patients with reported germline RAP1B variants.

Published
2024
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7. Microstrip patch antenna for energy harvesting in smart buildings.

Author

Brucal, Stanley Glenn E., Africa, Aaron Don M., Chavez, Julian Carlos P., Devera, Nathan H., Escamilla, Philip Martin Emmanuel A., and Payuyo, John Louie L.

Subjects
WIRELESS power transmission, ANTENNA radiation patterns, WIRELESS sensor networks, ELECTROMAGNETIC radiation, ANTENNA design, INTELLIGENT sensors
Abstract

The present study analyzes the microstrip antenna design for wireless power transfer in smart buildings, harnessing the ambient electromagnetic radiation due to common electronic gadgets that energize wireless sensor networks, computing devices, and connected appliances. With the increased number of these devices, so does the potential for health problems caused by electromagnetic radiation. However, these devices also provide a renewable energy source through their emissions. This study suggests the creation of a 5G Microstrip antenna that enhances the absorption of this radiation for the purpose of recharging batteries in smart buildings. The design capitalizes on the inherent low-profile and cost-effective features of microstrip antennas, making them well-suited for incorporation into building infrastructure and 5G wireless technologies. Although each individual device emits a little amount of energy, the combined effect achieved by advanced antenna design and power converters is anticipated to result in a substantial energy production. The antenna designer tool from MATLAB was used to carry out a conceptual simulation of the microstrip antenna. This has set up the framework of a feasible way of predicting the performance with high efficiency and sustainability for a wireless power transfer (WPT) system. [ABSTRACT FROM AUTHOR]

Published
2024
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8. Long‐term assessment of haematological recovery following somatic genetic rescue in a MYSM1‐deficient patient: Implications for in vivo gene therapy.

Author

Tocqueville, Sophie, Martin, Emmanuel, Riller, Quentin, Kermasson, Laëtitia, France, Benoit, Magérus, Aude, Rieux‐Laucat, Frédéric, Delhommeau, François, Hirsch, Pierre, Touzart, Aurore, Echalier, Aude, Fischer, Alain, Moshous, Despina, and Revy, Patrick

Subjects
*HEMATOPOIETIC system, *HEMATOPOIETIC stem cells, *BONE marrow, *GENE therapy, *NATUROPATHY
Abstract

Summary MYSM1 deficiency causes inherited bone marrow failure syndrome (IBMFS). We have previously identified an IBMFS patient with a homozygous pathogenic variant in MYSM1 who recovered from cytopenia due to spontaneous correction of one MYSM1 variant in the haematopoietic compartment, an event called somatic genetic rescue (SGR). The study of the genetic and biological aspects of the patient's haematopoietic/lymphopoietic system over a decade after SGR shows that one genetically corrected haematopoietic stem cell (HSC) can restore a healthy and stable haematopoietic system. This supports in vivo gene correction of HSCs as a promising treatment for IBMFS, including MYSM1 deficiency. [ABSTRACT FROM AUTHOR]

Published
2024
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9. Super-resolved live-cell imaging using random illumination microscopy

Author

Mangeat, Thomas, Labouesse, Simon, Allain, Marc, Negash, Awoke, Martin, Emmanuel, Guénolé, Aude, Poincloux, Renaud, Estibal, Claire, Bouissou, Anaïs, Cantaloube, Sylvain, Vega, Elodie, Li, Tong, Rouvière, Christian, Allart, Sophie, Keller, Debora, Debarnot, Valentin, Wang, Xia Bo, Michaux, Grégoire, Pinot, Mathieu, Le Borgne, Roland, Tournier, Sylvie, Suzanne, Magali, Idier, Jérome, and Sentenac, Anne

Published
2021
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11. Transient mTOR inhibition rescues 4-1BB CAR-Tregs from tonic signal-induced dysfunction

Author

Lamarthée, Baptiste, Marchal, Armance, Charbonnier, Soëli, Blein, Tifanie, Leon, Juliette, Martin, Emmanuel, Rabaux, Lucas, Vogt, Katrin, Titeux, Matthias, Delville, Marianne, Vinçon, Hélène, Six, Emmanuelle, Pallet, Nicolas, Michonneau, David, Anglicheau, Dany, Legendre, Christophe, Taupin, Jean-Luc, Nemazanyy, Ivan, Sawitzki, Birgit, Latour, Sylvain, Cavazzana, Marina, André, Isabelle, and Zuber, Julien

Published
2021
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13. MAGT1 deficiency in XMEN disease is associated with severe platelet dysfunction and impaired platelet glycoprotein N-glycosylation

Author

Kauskot, Alexandre, primary, Mallebranche, Coralie, additional, Bruneel, Arnaud, additional, Fenaille, François, additional, Solarz, Jean, additional, Viellard, Toscane, additional, Feng, Miao, additional, Repérant, Christelle, additional, Bordet, Jean-Claude, additional, Cholet, Sophie, additional, Denis, Cécile V., additional, McCluskey, Geneviève, additional, Latour, Sylvain, additional, Martin, Emmanuel, additional, Pellier, Isabelle, additional, Lasne, Dominique, additional, Borgel, Delphine, additional, Kracker, Sven, additional, Ziegler, Alban, additional, Tuffigo, Marie, additional, Fournier, Benjamin, additional, Miot, Charline, additional, and Adam, Frédéric, additional

Published
2023
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14. Pan-tropical prediction of forest structure from the largest trees

Author

Bastin, Jean-François, Rutishauser, Ervan, Kellner, James R., Saatchi, Sassan, Pélissier, Raphael, Hérault, Bruno, Slik, Ferry, Bogaert, Jan, De Cannière, Charles, Marshall, Andrew R., Poulsen, John, Alvarez-Loyayza, Patricia, Andrade, Ana, Angbonga-Basia, Albert, Araujo-Murakami, Alejandro, Arroyo, Luzmila, Ayyappan, Narayanan, de Azevedo, Celso Paulo, Banki, Olaf, Barbier, Nicolas, Barroso, Jorcely G., Beeckman, Hans, Bitariho, Robert, Boeckx, Pascal, Boehning-Gaese, Katrin, Brandão, Hilandia, Brearley, Francis Q., Hockemba, Mireille Breuer Ndoundou, Brienen, Roel, Camargo, Jose Luis C., Campos-Arceiz, Ahimsa, Cassart, Benoit, Chave, Jérôme, Chazdon, Robin, Chuyong, Georges, Clark, David B., Clark, Connie J., Condit, Richard, Coronado, Euridice N. Honorio, Davidar, Priya, de Haulleville, Thalès, Descroix, Laurent, Doucet, Jean-Louis, Dourdain, Aurelie, Droissart, Vincent, Duncan, Thomas, Espejo, Javier Silva, Espinosa, Santiago, Farwig, Nina, Fayolle, Adeline, Feldpausch, Ted R., Ferraz, Antonio, Fletcher, Christine, Gajapersad, Krisna, Gillet, Jean-François, do Amaral, Iêda Leão, Gonmadje, Christelle, Grogan, James, Harris, David, Herzog, Sebastian K., Homeier, Jürgen, Hubau, Wannes, Hubbell, Stephen P., Hufkens, Koen, Hurtado, Johanna, Kamdem, Narcisse G., Kearsley, Elizabeth, Kenfack, David, Kessler, Michael, Labrière, Nicolas, Laumonier, Yves, Laurance, Susan, Laurance, William F., Lewis, Simon L., Libalah, Moses B., Ligot, Gauthier, Lloyd, Jon, Lovejoy, Thomas E., Malhi, Yadvinder, Marimon, Beatriz S., Junior, Ben Hur Marimon, Martin, Emmanuel H., Matius, Paulus, Meyer, Victoria, Bautista, Casimero Mendoza, Monteagudo-Mendoza, Abel, Mtui, Arafat, Neill, David, Gutierrez, Germaine Alexander Parada, Pardo, Guido, Parren, Marc, Parthasarathy, N., Phillips, Oliver L., Pitman, Nigel C. A., Ploton, Pierre, Ponette, Quentin, Ramesh, B. R., Razafimahaimodison, Jean-Claude, Réjou-Méchain, Maxime, Rolim, Samir Gonçalves, Saltos, Hugo Romero, Rossi, Luiz Marcelo Brum, Spironello, Wilson Roberto, Rovero, Francesco, Saner, Philippe, Sasaki, Denise, Schulze, Mark, Silveira, Marcos, Singh, James, Sist, Plinio, Sonke, Bonaventure, Soto, J. Daniel, de Souza, Cintia Rodrigues, Stropp, Juliana, Sullivan, Martin J. P., Swanepoel, Ben, ter Steege, Hans, Terborgh, John, Texier, Nicolas, Toma, Takeshi, Valencia, Renato, Valenzuela, Luis, Ferreira, Leandro Valle, Valverde, Fernando Cornejo, Van Andel, Tinde R., Vasque, Rodolfo, Verbeeck, Hans, Vivek, Pandi, Vleminckx, Jason, Vos, Vincent A., Wagner, Fabien H., Papi Puspa, Warsudi, Wortel, Verginia, Zagt, Roderick J., and Zebaze, Donatien

Published
2018

18. Perjamuan Tuhan di Korintus

Author

Richardo Martin Emmanuel De Vries

Abstract

Paul in his first letter to the church at Corinthians condemned the church at Corinth. The criticism was made because the assembly there considered the Lord's banquet which they often held was a banquet in general, it was not separated from the banquets in public places. That supper is no longer remembered as the Lord's act on the last night with His Disciples. Paul concerning this phenomenon. In addition, the banquet at Corinth did not bring fellowship to the church, instead it brought division to the church. In this article, the author will discuss some of the main problems above contained in the text of 1 Corinthians, and draw on the theological meaning contained in the text.

Published
2022

20. Abstract 3363: Spatial and longitudinal tumor heterogeneity in head and neck squamous cell carcinoma patients treated with primary surgery

Author

Marret, Grégoire, primary, Lamy, Constance, additional, Vacher, Sophie, additional, Séné, Mathieu, additional, Ahmanache, Ladidi, additional, Courtois, Laura, additional, Beiano, Zakhia El, additional, Klijanienko, Jerzy, additional, Martinat, Charlotte, additional, Servant, Nicolas, additional, Kamoun, Choumouss, additional, Chérif, Linda Larbi, additional, Bronzini, Thierry, additional, Balsat, Cédric, additional, Laes, Jean-François, additional, Prévot, Aubray, additional, Sauvage, Sébastien, additional, Lienard, Maxime, additional, Martin, Emmanuel, additional, Genin, Bérengère, additional, Badois, Nathalie, additional, Lesnik, Maria, additional, Dubray-Vautrin, Antoine, additional, Choussy, Olivier, additional, Ghanem, Wahib, additional, Taouachi, Rabah, additional, Planchon, Julien Masliah, additional, Bièche, Ivan, additional, Kamal, Maud, additional, and Tourneau, Christophe Le, additional

Published
2023
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26. The Fezouata Biota (Central Anti-Atlas, Morocco): Biostratigraphy and Associated Environmental Conditions of an Ordovician Burgess Shale

Author

Martin, Emmanuel, Lefebvre, Bertrand, Pittet, Bernard, Vannier, Jean, Bachnou, Ali, Hariri, Khadija El, Hafid, Ahmid, Masrour, Moussa, Noailles, Fleur, Nowak, Hendrik, Servais, Thomas, Vandenbroucke, Thijs R. A., Van Roy, Peter, Vidal, Muriel, Vizcaïno, Daniel, Rocha, Rogério, editor, Pais, João, editor, Kullberg, José Carlos, editor, and Finney, Stanley, editor

Published
2014
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30. Human CARMIL2 deficiency underlies a broader immunological and clinical phenotype than CD28 deficiency

Author

Lévy, Romain, Gothe, Florian, Momenilandi, Mana, Magg, Thomas, Materna, Marie, Peters, Philipp, Raedler, Johannes, Philippot, Quentin, Rack-Hoch, Anita, Langlais, David, Bourgey, Mathieu, Lanz, Anna-Lisa, Ogishi, Masato, Rosain, Jérémie, Martin, Emmanuel, Latour, Sylvain, Vladikine, Natasha, Distefano, Marco, Khan, Taushif, Rapaport, Franck, Schulz, Marian, Holzer, Ursula, Fasth, Anders, Sogkas, Georgios, Speckmann, Carsten, Troilo, Arianna, Bigley, Venetia, Roppelt, Anna, Dinur-Schejter, Yael, Toker, Ori, Bronken Martinsen, Karen, Sherkat, Roya, Somekh, Ido, Somech, Raz, Shouval, Dror, Kühl, Jörn-Sven, Ip, Winnie, Mcdermott, Elizabeth, Cliffe, Lucy, Ozen, Ahmet, Baris, Safa, Rangarajan, Hemalatha, Jouanguy, Emmanuelle, Puel, Anne, Bustamante, Jacinta, Alyanakian, Marie-Alexandra, Fusaro, Mathieu, Wang, Yi, Kong, Xiao-Fei, Cobat, Aurélie, Boutboul, David, Castelle, Martin, Aguilar, Claire, Hermine, Olivier, Cheminant, Morgane, Suarez, Felipe, Yildiran, Alisan, Bousfiha, Aziz, Al-Mousa, Hamoud, Alsohime, Fahad, Cagdas, Deniz, Abraham, Roshini, Knutsen, Alan, Fevang, Borre, Bhattad, Sagar, Kiykim, Ayca, Erman, Baran, Arikoglu, Tugba, Unal, Ekrem, Kumar, Ashish, Geier, Christoph, Baumann, Ulrich, Neven, Bénédicte, Calas, Julie, Feuille, Elizabeth, Chan, Angela, Yesil, Gozde, Nammour, Justine, Bandet, Élise, Picard, Capucine, Benhsaien, Ibtihal, Lang, Peter, Atschekzei, Faranaz, Warnatz, Klaus, Hambleton, Sophie, Desai, Mukesh, Karakoc-Aydiner, Elif, Kolukisa, Burcu, Al-Muhsen, Saleh, Alosaimi, Mohammed, Cipe, Funda, Alazami, Anas, Hancioglu, Gonca, Can Meydan, Bilge, Sorte, Hanne, Stray-Pedersen, Asbjørg, Mammayil, Geetha, Tökmeci, Nazan, Shcherbina, Anna, Stepensky, Polina, Nasereddin, Adeeb, Rouzaud, Claire, Hoshino, Akihiro, Shamriz, Oded, Ledder, Oren, Maccari, Maria, Castro, Carla, Grimbacher, Bodo, Schmidt, Reinhold, Collin, Matthew, Zakharova, Victorya, Rohlfs, Meino, Walz, Christoph, Abel, Laurent, Malissen, Bernard, Marr, Nico, Klein, Christoph, Casanova, Jean-Laurent, Hauck, Fabian, Béziat, Vivien, Lévy R., Gothe F., Momenilandi M., Magg T., Materna M., Peters P., Raedler J., Philippot Q., Rack-Hoch A. L., Langlais D., et al., 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), Centre d'Immunophénomique (CIPHE), ANR-10-LABX-0062,IBEID,Integrative Biology of Emerging Infectious Diseases(2010), ANR-10-IAHU-0003,Méditerranée Infection,I.H.U. Méditerranée Infection(2010), and ANR-21-CE15-0034,CARMIL2,Bases moléculaires, cellulaires et immunologiques de la déficience combinée résultant de mutations dans CARMIL2(2021)

Subjects
CD4-Positive T-Lymphocytes, Phenotype, CD28 Antigens, [SDV]Life Sciences [q-bio], Microfilament Proteins, Mutation, Immunology, Humans, Immunology and Allergy
Abstract

International audience; Patients with inherited CARMIL2 or CD28 deficiency have defective T cell CD28 signaling, but their immunological and clinical phenotypes remain largely unknown. We show that only one of three CARMIL2 isoforms is produced and functional across leukocyte subsets. Tested mutant CARMIL2 alleles from 89 patients and 52 families impair canonical NF-κB but not AP-1 and NFAT activation in T cells stimulated via CD28. Like CD28-deficient patients, CARMIL2-deficient patients display recalcitrant warts and low blood counts of CD4 + and CD8 + memory T cells and CD4 + T REG s. Unlike CD28-deficient patients, they have low counts of NK cells and memory B cells, and their antibody responses are weak. CARMIL2 deficiency is fully penetrant by the age of 10 yr and is characterized by numerous infections, EBV + smooth muscle tumors, and mucocutaneous inflammation, including inflammatory bowel disease. Patients with somatic reversions of a mutant allele in CD4 + T cells have milder phenotypes. Our study suggests that CARMIL2 governs immunological pathways beyond CD28.

Published
2022

31. Inherited human ITK deficiency impairs IFN-γ immunity and underlies tuberculosis

Author

Ogishi, Masato, primary, Yang, Rui, additional, Rodriguez, Rémy, additional, Golec, Dominic P., additional, Martin, Emmanuel, additional, Philippot, Quentin, additional, Bohlen, Jonathan, additional, Pelham, Simon J., additional, Arias, Andrés Augusto, additional, Khan, Taushif, additional, Ata, Manar, additional, Al Ali, Fatima, additional, Rozenberg, Flore, additional, Kong, Xiao-Fei, additional, Chrabieh, Maya, additional, Laine, Candice, additional, Lei, Wei-Te, additional, Han, Ji Eun, additional, Seeleuthner, Yoann, additional, Kaul, Zenia, additional, Jouanguy, Emmanuelle, additional, Béziat, Vivien, additional, Youssefian, Leila, additional, Vahidnezhad, Hassan, additional, Rao, V. Koneti, additional, Neven, Bénédicte, additional, Fieschi, Claire, additional, Mansouri, Davood, additional, Shahrooei, Mohammad, additional, Pekcan, Sevgi, additional, Alkan, Gulsum, additional, Emiroğlu, Melike, additional, Tokgöz, Hüseyin, additional, Uitto, Jouni, additional, Hauck, Fabian, additional, Bustamante, Jacinta, additional, Abel, Laurent, additional, Keles, Sevgi, additional, Parvaneh, Nima, additional, Marr, Nico, additional, Schwartzberg, Pamela L., additional, Latour, Sylvain, additional, Casanova, Jean-Laurent, additional, and Boisson-Dupuis, Stéphanie, additional

Published
2022
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33. New Middle and Late Ordovician cornute stylophorans (Echinodermata) from Morocco and other peri-Gondwanan areas

Author

Lefebvre, Bertrand, Nohejlová, Martina, Martin, Emmanuel L.O., Kasicka, Libor, Zicha, Ondřej, Gutiérrez-Marco, Juan Carlos, Laboratoire de Géologie de Lyon - Terre, Planètes, Environnement (LGL-TPE), École normale supérieure de Lyon (ENS de Lyon)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut national des sciences de l'Univers (INSU - CNRS)-Université Jean Monnet - Saint-Étienne (UJM)-Centre National de la Recherche Scientifique (CNRS), and Czech Geological Survey [Praha]

Subjects
[SDU.STU.ST]Sciences of the Universe [physics]/Earth Sciences/Stratigraphy, [SDU.STU.PG]Sciences of the Universe [physics]/Earth Sciences/Paleontology
Abstract

International audience

Published
2022

38. Mucosal-associated invariant T cell-rich congenie mouse strain allows functional evaluation

Author

Cui, Yue, Franciszkiewicz, Katarzyna, Mburu, Yvonne K., Mondot, Stanislas, Bourhis, Lionel Le, Premel, Virginie, Martin, Emmanuel, Kachaner, Alexandra, Duban, Livine, Ingersoll, Molly A., Rabot, Sylvie, Jaubert, Jean, De Villartay, Jean- Pierre, Soudais, Claire, and Lantz, Olivier

Subjects
Medical research, Medicine, Experimental, T cells -- Properties, Immune response -- Observations, Disease susceptibility -- Research, Health care industry
Abstract

Mucosal-associated invariant T cells (MAITs) have potent antimicrobial activity and are abundant in humans (5%-10% in blood). Despite strong evolutionary conservation of the invariant TCR-α chain and restricting molecule MR1, this population is rare in laboratory mouse strains (≅ 0.1% in lymphoid organs), and lack of an appropriate mouse model has hampered the study of MAIT biology. Herein, we show that MAITs are 20 times more frequent in clean wild-derived inbred CAST/EiJ mice than in C57BL/6J mice. Increased MAIT frequency was linked to one CAST genetic trait that mapped to the TCR-α locus and led to higher usage of the distal Vα segments, including Vα19. We generated a [MAIT.sup.hi] congenic strain that was then crossed to a transgenic Rorcgt-GFP reporter strain. Using this tool, we characterized polyclonal mouse MAITs as memory ([CD44.sup.+]) [CD4.sup.-][CD8.sup.lo/neg] T cells with tissue-homing properties ([CCR6.sup.+][CCR7.sup.-]). Similar to human MAITs, mouse MAITs expressed the cytokine receptors IL-7R, IL-18Rα, and IL-12Rβ and the transcription factors promyelocytic leukemia zinc finger (PLZF) and RAR-related orphan receptor γ (RORγt). Mouse MAITs produced Th1/2/17 cytokines upon TCR stimulation and recognized a bacterial compound in an MR1-dependent manner. During experimental urinary tract infection, MAITs migrated to the bladder and decreased bacterial load. Our study demonstrates that the [MAIT.sup.hi] congenic strain allows phenotypic and functional characterization of naturally occurring mouse MAITs in health and disease., Introduction Mucosal-associated invariant T cells (MAITs) express an invariant TCR-α (iTCR-α) chain (iVα7.2-Jα33 in human and iVα19-Jα33 in mouse) restricted by the major histocompatibility complex (MHC) class I-related protein 1 [...]

Published
2015
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46. CTP synthase 1 deficiency in humans reveals its central role in lymphocyte proliferation

Author

Martin, Emmanuel, Palmic, Noe, Sanquer, Sylvia, Lenoir, Christelle, Hauck, Fabian, Mongellaz, Cedric, Fabrega, Sylvie, Nitschke, Patrick, Esposti, Mauro Degli, Schwartzentruber, Jeremy, Taylor, Naomi, Majewski, Jacek, Jabado, Nada, Wynn, Robert F., Picard, Capucine, Fischer, Alain, Arkwright, Peter D., and Latour, Sylvain

Subjects
Pyrimidine nucleotides -- Physiological aspects, Cell research, Lymphocytes -- Physiological aspects, Immunodeficiency -- Research, Environmental issues, Science and technology, Zoology and wildlife conservation
Abstract

Lymphocyte functions triggered by antigen recognition and costimulation signals are associated with a rapid and intense cell division, and hence with metabolism adaptation (1). The nucleotide cytidine 5' triphosphate (CTP) is a precursor required for the metabolism of DNA, RNA and phospholipids (2-4). CTP originates from two sources: a salvage pathway and a denovosynthesis pathway that depends on two enzymes, the CTP synthases (or synthetases) 1 and 2 (CTPS1 with CTPS2); the respective roles of these two enzymes are not known (5-7). CTP synthase activity is a potentially important step for DNA synthesis in lymphocytes (8,9). Here we report the identification of a loss-of-functionhomozygous mutation (rs145092287) in CTPS1 inhumans that causes a novel and life-threatening immunodeficiency, characterized by an impaired capacity of activated T and B cells to proliferate in response to antigen receptor-mediated activation. In contrast, proximal and distal T-cell receptor (TCR) signalling events and responses were only weakly affected by the absence of CTPS1. Activated CTPS1-deficient cells had decreased levels of CTP. Normal T-cell proliferation was restored in CTPS1 -deficient cells by expressing wild-type CTPS1 or by addition of exogenous CTP or its nucleoside precursor, cytidine. CTPS1 expression was found to be low in resting T cells, but rapidly upregulated following TCR activation. These results highlight a key and specific role of CTPS1 in the immune system by its capacity to sustain the proliferation of activated lymphocytes during the immune response. CTPS1 may therefore represent a therapeutic target of immunosuppressive drugs that could specifically dampen lymphocyte activation., We initially studied two unrelated families (family 1 and 2) originating from the northwest region of England, whose four children suffered from severe and recurrent Epstein-Barr virus (EBV) infection, in [...]

Published
2014

47. Saccharide isomerate ameliorates cosmetic scalp conditions in a Chinese study population.

Author

Martin, Emmanuel, Zhang, Anson, and Campiche, Remo

Subjects
*CHINESE people, *SACCHARIDES, *SCALP, *PSYCHOLOGICAL distress
Abstract

Background: Scalp conditions such as flaky or oily scalp affect people across ethnicities and age groups. In addition to flaking, increased sebum secretion, itching, and compromised scalp barrier function were described. Scalp conditions are aesthetically disturbing and may cause psychological distress in affected individuals who are looking for mild and effective treatment at the same time. Saccharide isomerate has a long history as a skin moisturizer, and it was found to improve skin barrier function, also suggesting possible beneficial effects on scalp. Aims: To provide relevant claim substantiation to introduce saccharide isomerate as a new scalp care active against scalp flaking condition. Material and Methods: We conducted a placebo‐controlled clinical study in an adult Chinese population affected by dandruff scalp as assessed by an adherent scalp flaking score. We monitored transepidermal water loss (TEWL), sebum secretion, and scalp flaking during 28 days. Results: Formulations containing Saccharide isomerate significantly improved all parameters both over time as well as compared to the placebo formulation. Conclusion: We propose Saccharide isomerate for cosmetic formulations directed toward improving scalp conditions such as dandruff or oily scalp. [ABSTRACT FROM AUTHOR]

Published
2023
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