Review
Immunology
Hyoungjun Ham, Michael Medlyn, Daniel D. Billadeau
Summary: NK cell-mediated cytotoxicity is essential for protection from infections and cancer, involving lytic granule development and degranulation processes. Studies on molecular regulations of NK cell cytotoxicity are providing insights into improving anti-cancer therapies and discovering new therapeutic opportunities.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Immunology
Michael B. Jordan
Summary: HLH is an immune system disorder that has seen advancements in diagnosis and treatment in recent decades. It is characterized by excessive T cell activation and myeloid cell activation, largely driven by interferon-gamma. Diagnosis and treatment now rely on measuring T cell activation, IFN-gamma activity, and inflammation.
IMMUNOLOGICAL REVIEWS
(2023)
Article
Immunology
Matias Ruggieri, Nicolas Ducasa, Claudia Juraske, Virginia Gonzalez Polo, Carolina Berini, Maria Florencia Quiroga, Petros Christopoulos, Susana Minguet, Mirna Biglione, Wolfgang W. W. Schamel
Summary: The study demonstrates a potential protective role of Vγ9Vδ2T cells against HTLV-1 infection, showing a decrease in these cells in patients with HTLV-1 and their ability to kill cells expressing viral proteins. This insight could lead to novel tools for preventing and treating HTLV-1-associated malignancies and neurologic complications.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Immunology
Vera Goda, Gergely Krivan, Andrea Kulcsar, Marton Gonczi, Szabolcs Tasnady, Zsolt Matula, Ginette Nagy, Gabriella Beko, Mate Horvath, Ferenc Uher, Zoltan Szekanecz, Istvan Valyi-Nagy
Summary: This study evaluated the immune response of CVID patients receiving booster vaccination with BNT162b2 against SARS-CoV-2. The results showed that the vaccine can increase antibody levels and T cell response in CVID patients, but the response levels are still lower compared to healthy individuals.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Immunology
Laura Vinas-Gimenez, Rafael Rincon, Roger Colobran, Xavier de la Cruz, Veronica Paola Celis, Jose Luis Dapena, Laia Alsina, Joan Sayos, Monica Martinez-Gallo
Summary: This study analyzed a novel monoallelic mutation found in a patient with two episodes of HLH, showing that the mutation diminishes degranulation capacity.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Immunology
Han-qi Zhang, Si-Wei Yang, Yi-Cheng Fu, Ming-Cong Chen, Cheng-Hao Yang, Ming-Hua Yang, Xiao-Dan Liu, Qing-Nan He, Hua Jiang, Ming-Yi Zhao
Summary: This article summarizes the main pathogenesis and treatment methods of hemophagocytic lymphohistiocytosis (HLH).
IMMUNOLOGIC RESEARCH
(2022)
Article
Immunology
Joseph Jelinski, Monika M. Kowatsch, Marc-Alexandre Lafrance, Alice Berger, Jannie Pedersen, Hiva Azizi, Yue Li, Florine Scholte, Alejandro Gomez, Natasha Hollett, Toby Le, Matthew Wade, Hugues Fausther-Bovendo, Marc-Antoine de la Vega, George Babuadze, Ara Xiii, Claude Lamarre, Trina Racine, Chil-Yong Kang, Xiao-Jian Yao, Galit Alter, Eric Arts, Keith R. Fowke, Gary P. Kobinger
Summary: Despite the 40-year global HIV pandemic, no licensed vaccine has been developed for high-risk populations. However, a novel recombinant vesicular stomatitis virus (rVSV) vector vaccine expressing modified HIV envelope glycoproteins and Ebola virus glycoprotein successfully prevented SHIV infection in 60% of non-human primates (NHPs). Resistance to infection was associated with specific immune responses, including CD69 and CD154 upregulation, increased IL-2 production, and reduced IFN-γ response, providing insights into potential correlates of protection.
EMERGING MICROBES & INFECTIONS
(2023)
Review
Immunology
Ye Qiu, Gaoneng Fang, Feng Ye, Wen Zeng, Mengxin Tang, Xuan Wei, Jinglu Yang, Zhengtu Li, Jianquan Zhang
Summary: This study describes the pathogen spectrum and treatment strategies among patients with AIGA positivity. Non-tuberculous mycobacteria (NTM) is the most common pathogen, with 41.2% of patients experiencing recurrence and/or persistent infection. Rituximab and cyclophosphamide are common treatment options, and most patients achieve remission.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Cell Biology
Minghang Yu, Ziyang Su, Xuefeng Huang, Yujie Zhou, Xulong Zhang, Bingbing Wang, Zihan Wang, Yi Liu, Nianzeng Xing, Miaoran Xia, Xi Wang
Summary: In this study, it was found that inhibiting the Ezh2 gene can promote the development and cytotoxicity of NK cells, enhancing the immune response of NK cells to tumor cells. Further experiments showed that Ezh2 may affect NK cell development by directly modulating the expression of the transcription factor Pbx1.
JOURNAL OF LEUKOCYTE BIOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Li Du, Yaru Nai, Meiying Shen, Tingting Li, Jingjing Huang, Xiaojian Han, Wang Wang, Da Pang, Aishun Jin
Summary: The addition of IL-21 in CAR-T cell preparation was found to improve T cell transfection efficiency, enhance the enrichment and expansion of less differentiated CAR-T cells, and improve CAR-T cell cytotoxicity by promoting the secretion of effector cytokines.
FRONTIERS IN MOLECULAR BIOSCIENCES
(2021)
Article
Cell Biology
Yolla German, Loan Vulliard, Anton Kamnev, Laurene Pfajfer, Jakob Huemer, Anna-Katharina Mautner, Aude Rubio, Artem Kalinichenko, Kaan Boztug, Audrey Ferrand, Joerg Menche, Loic Dupre
Summary: The immunological synapse decodes stimulatory signals into adapted lymphocyte responses, and high-content imaging is used to analyze T and NK cells. The study demonstrates how actin cytoskeleton remodeling shapes synapse architecture and affects lytic granule positioning. Analysis of immunodeficient individuals' CD8(+) T cells reveals the roles of ARPC1B and WASP in synapse assembly.
Article
Multidisciplinary Sciences
Marius Schwabenland, Omar Mossad, Annika Sievert, Adam G. Peres, Elena Ringel, Sebastian Baasch, Julia Kolter, Giulia Cascone, Nikolaos Dokalis, Andreas Vlachos, Zsolt Ruzsics, Philipp Henneke, Marco Prinz, Thomas Blank
Summary: The study found that mimicking early systemic viral infection in neonatal mice resulted in behavioral deficits and immune response changes only in male adolescent mice, while female mice were unaffected. This suggests that there may be sex-dependent differences in the susceptibility and long-term effects of CNS disorders following early life immune activation.
NATURE COMMUNICATIONS
(2023)
Article
Oncology
Nishant Chovatiya, Kawaljit Kaur, Sara Huerta-Yepez, Po-Chun Chen, Adam Neal, Gabriella DiBernardo, Serhat Gumrukcu, Sanaz Memarzadeh, Anahid Jewett
Summary: We studied the sensitivity of different ovarian tumor cell lines to NK cell-mediated cytotoxicity and found that the levels of tumor differentiation and aggressiveness were correlated with the sensitivity to NK cell killing. Poorly differentiated ovarian tumors were unable to upregulate MHC-class I under activating conditions, which may be a specific feature of ovarian tumors. NK cells showed targeted killing of these tumors and could be utilized in immunotherapeutic strategies.
CANCER IMMUNOLOGY IMMUNOTHERAPY
(2022)
Article
Immunology
Wen-I. Lee, Yao-Fan Fang, Jing-Long Huang, Huey-Ling You, Meng-Ying Hsieh, Wan-Ting Huang, Chi-Jou Liang, Chen-Chen Kang, Ting-Shu Wu
Summary: It is not uncommon to find the presence of anti-interferon-gamma autoantibodies (AutoAbs-IFN-gamma) in patients with persistent non-tuberculous mycobacterial (NTM) infections characteristic of adult-onset immunodeficiency syndrome. The immune disturbances in this disorder are still not well understood. By quantifying the levels of AutoAbs-IFN-gamma and comparing them with healthy controls, it was found that the abnormal distribution of immune cell subpopulations may be correlated with the presence of AutoAbs-IFN-gamma.
JOURNAL OF CLINICAL IMMUNOLOGY
(2023)
Article
Pediatrics
Allison Guild, Jordan Fritch, Sachit Patel, Adam Reinhardt, Melissa Acquazzino
Summary: Two patients with trisomy 21 and refractory HLH were successfully treated with interventions targeting the interferon pathway. This suggests that the interferon signaling pathway, driven by interferon gamma, may be a key therapeutic target in both trisomy 21 autoimmunity and HLH.
PEDIATRIC RHEUMATOLOGY
(2022)
Article
Immunology
Sabine Jaegle, Maximilian Heeg, Sarah Gruen, Anne Rensing-Ehl, Maria Elena Maccari, Christian Klemann, Neil Jones, Kai Lehmberg, Claudia Bettoni, Klaus Warnatz, Bodo Grimbacher, Ariane Biebl, Uwe Schauer, Rosie Hague, Olaf Neth, Andrea Mauracher, Jana Pachlopnik Schmid, Alexandre Fabre, Larysa Kostyuchenko, Marita Fuehrer, Myriam Ricarda Lorenz, Klaus Schwarz, Jan Rohr, Stephan Ehl
CLINICAL IMMUNOLOGY
(2020)
Letter
Dermatology
Andreas Benedikt Weins, Martin Theiler, Bettina Bogatu, Karin Kerl, Marc Pleimes, Jana Pachlopnik-Schmid, Lisa Weibel
JOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT
(2020)
Article
Allergy
Mathieu Fusaro, Jeremie Rosain, Virginie Grandin, Nathalie Lambert, Sylvain Hanein, Cecile Fourrage, Nicholas Renaud, Marine Gil, Samuel Chevalier, Wadih Abou Chahla, Brigitte Bader-Meunier, Vincent Barlogis, Stephane Blanche, David Boutboul, Martin Castelle, Thibault Comont, Jean-Sebastien Diana, Claire Fieschi, Lionel Galicier, Olivier Hermine, Alain Lefevre-Utile, Marion Malphettes, Etienne Merlin, Eric Oksenhendler, Marlene Pasquet, Felipe Suarez, Isabelle Andre, Vivien Beziat, Genevieve de Saint Basile, Jean-Pierre De Villartay, Sven Kracker, Chantal Lagresle-Peyrou, Sylvain Latour, Frederic Rieux-Laucat, Nizar Mahlaoui, Christine Bole, Patrick Nitschke, Elisabeth Hulier-Ammar, Alain Fischer, Despina Moshous, Benedicte Neven, Alexandre Alcais, Guillaume Vogt, Jacinta Bustamante, Capucine Picard
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
(2021)
Article
Clinical Neurology
Guillaume Taieb, Elsa Kaphan, Claire Duflos, Christine Lebrun-Frenay, Valerie Rigau, Eric Thouvenot, Emeline Duhin-Gand, Romain Lefaucheur, Khe Hoang-Xuan, Sarah Coulette, Jean Christophe Ouallet, Nicolas Menjot de Champfleur, Christine Tranchant, Capucine Picard, Mathieu Fusaro, Fernando E. Sepulveda, Pierre Labauge, Genevieve de Saint Basile
Summary: In adults with CLIPPERS, a significant proportion have mutations in HLH genes. This treatable genetic condition should be considered in CLIPPERS patients, especially those with atypical presentations.
NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION
(2021)
Article
Pediatrics
Francesca Minoia, Jessica Tibaldi, Valentina Muratore, Romina Gallizzi, Claudia Bracaglia, Alessia Arduini, Elif Comak, Olga Vougiouka, Ralf Trauzeddel, Giovanni Filocamo, Antonio Mastrangelo, Concetta Micalizzi, Ozgur Kasapcopur, Erbil Unsal, Toshiyuki Kitoh, Elena Tsitsami, Mikhail Kostik, Jana Pachlopnik Schmid, Seraina Prader, Guido Laube, Despoina Maritsi, Marija Jelusic, Susan Shenoi, Sebastiaan Vastert, Gianluigi Ardissino, Randy Q. Cron, Angelo Ravelli
Summary: This study focused on the clinical characteristics, treatment, and outcomes of patients with both macrophage activation syndrome (MAS) and thrombotic microangiopathy (TMA). It found that in patients with systemic juvenile idiopathic arthritis (sJIA) who had the co-occurrence of MAS and TMA, there were more severe manifestations, higher levels of liver enzymes and coagulation parameters, and a higher need for intensive care.
JOURNAL OF PEDIATRICS
(2021)
Review
Cell Biology
Gael Menasche, Cyril Lenge, Manuela Bratti, Ulrich Blank
Summary: Mast cells release bioactive compounds upon stimulation and their granule secretion is regulated by the cytoskeleton and transport mechanisms. Recent studies have revealed a tight coupling between these two systems, offering new strategies for treating allergic and inflammatory diseases.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Allergy
David Egg, Ina Caroline Rump, Noriko Mitsuiki, Jessica Rojas-Restrepo, Maria-Elena Maccari, Charlotte Schwab, Annemarie Gabrysch, Klaus Warnatz, Sigune Goldacker, Virginia Patino, Daniel Wolff, Satoshi Okada, Seiichi Hayakawa, Yoshiaki Shikama, Kenji Kanda, Kohsuke Imai, Manabu Sotomatsu, Makoto Kuwashima, Takahiro Kamiya, Tomohiro Morio, Kazuaki Matsumoto, Takeshi Mori, Yuri Yoshimoto, Ingunn Dybedal, Maria Kanariou, Zeynep Yesim Kucuk, Hugo Chapdelaine, Lenka Petruzelkova, Hanns-Martin Lorenz, Kathleen E. Sullivan, Jennifer Heimall, Michel Moutschen, Jiri Litzman, Mike Recher, Michael H. Albert, Fabian Hauck, Suranjith Seneviratne, Jana Pachlopnik Schmid, Antonios Kolios, Gary Unglik, Christian Klemann, Scott Snapper, Lisa Giulino-Roth, Michael Svaton, Craig D. Platt, Sophie Hambleton, Olaf Neth, Geraldine Gosse, Steffen Reinsch, Dirk Holzinger, Yae-Jean Kim, Shahrzad Bakhtiar, Faranaz Atschekzei, Reinhold Schmidt, Georgios Sogkas, Shanmuganathan Chandrakasan, William Rae, Beata Derfalvi, Hanne Vibeke Marquart, Ahmet Ozen, Ayca Kiykim, Elif Karakoc-Aydiner, Pavlina Kralickova, Godelieve de Bree, Dimitra Kiritsi, Markus G. Seidel, Robin Kobbe, Jennifer Dantzer, Laia Alsina, Thais Armangue, Vassilios Lougaris, Philipp Agyeman, Sofia Nystrom, David Buchbinder, Peter D. Arkwright, Bodo Grimbacher
Summary: Heterozygous germline mutations in cytotoxic T lymphocyte-associated antigen-4 (CTLA4) can lead to life-threatening autoimmune and lymphoproliferative complications. Treatment options include systemic immunosuppressants, immunoglobulin replacement, and stem cell transplantation for potential cure.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
(2022)
Article
Chemistry, Multidisciplinary
Leishemba K. Thoidingjam, Cedric M. Blouin, Christine Gaillet, Aurelien Brion, Stephanie Solier, Supaporn Niyomchon, Ahmed El Marjou, Sara Mouasni, Fernando E. Sepulveda, Genevieve de Saint Basile, Christophe Lamaze, Raphael Rodriguez
Summary: Interferons (IFNs), important cytokines, play roles in triggering downstream biochemical events upon binding to cell surface receptors. Dysregulated IFN signaling has been linked to cancer progression and autoimmune diseases. Researchers have identified a small molecule that can block the activation of IFN signaling and further developing a more potent analogue that shows potential therapeutic effects in reducing bleeding in a model of hemorrhagic colitis. This first-in-class small molecule also inhibits other types of IFN-induced signaling. This research provides a foundation for the development of pan-IFN inhibitory drugs.
ANGEWANDTE CHEMIE-INTERNATIONAL EDITION
(2022)
Article
Allergy
Cyril Longe, Manuela Bratti, Mathieu Kurowska, Shamila Vibhushan, Pierre David, Valere Desmeure, Jian-Dong Huang, Alain Fischer, Genevieve de Saint Basile, Fernando E. Sepulveda, Ulrich Blank, Gael Menasche
Summary: This study demonstrates the crucial role of Rab44 in regulating secretory granule transport during degranulation in mast cells. Deficiency in Rab44 impairs granule translocation and degranulation, leading to reduced sensitivity to IgE-mediated allergic reactions. Rab44 can be considered a potential target for modulating MC degranulation and inhibiting allergic reactions.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
(2022)
Editorial Material
Pediatrics
Veronika Baghin, Seraina Prader, Selma Sirin, Jana Pachlopnik Schmid, Johannes Trueck
ARCHIVES OF DISEASE IN CHILDHOOD-EDUCATION AND PRACTICE EDITION
(2023)
Letter
Dermatology
J. B. Monfort, S. Deshayes, P. Dusser, R. Bourguiba, L. Savey, C. Vinit, I. Kone-Paut, G. Amaryan, K. Theodoropoulou, R. Guedri, J. Pachlopnik, A. Belot, I. Melki, N. Perveen Maldar, V. Hentgen, S. Georgin-Lavialle
JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY
(2022)
Article
Virology
Abhilash Kannan, Maarit Suomalainen, Romain Volle, Michael Bauer, Marco Amsler, Hung Trinh, Stefano Vavassori, Jana Pachlopnik Schmid, Guilherme Vilhena, Alberto Marin-Gonzalez, Ruben Perez, Andrea Franceschini, Christian von Mering, Silvio Hemmi, Urs F. Greber
Summary: This study discovered that transfection of human airway cell lines or non-transformed fibroblasts with double-stranded RNA mimicking cellular micro-RNA has strong antiviral effects against human adenovirus, influenza A virus, and SARS-CoV-2. These effects are dependent on complementary RNA strands and lead to the downregulation of certain genes and the induction of interferon-stimulated genes. The antiviral effects are mediated by specific protein receptors and RNA sensors.
Letter
Immunology
Maarja Soomann, Seraina Prader, Jana Pachlopnik Schmid, Tayfun Gungor, Johannes Truck
JOURNAL OF CLINICAL IMMUNOLOGY
(2023)
Article
Immunology
Benjamin Rolles, Andres Caballero-Oteyza, Michele Proietti, Sigune Goldacker, Klaus Warnatz, Nadezhda Camacho-Ordonez, Seraina Prader, Jana Pachlopnik Schmid, Margherita Vieri, Susanne Isfort, Robert Meyer, Martin Kirschner, Tim H. Bruemmendorf, Fabian Beier, Bodo Grimbacher
Summary: Telomere biology disorders (TBD) are genetic diseases caused by pathogenic variants in genes related to telomere maintenance. TBD can manifest in adulthood and have variable symptoms, making diagnosis complicated. Common variable immunodeficiency (CVID) is an antibody deficiency syndrome caused by dysfunctional B lymphocytes. In our study, we found that approximately 22% of CVID patients carried rare candidate variants in telomere-associated genes. We recommend including all TBD-associated genes in the genetic screening of patients with antibody deficiencies.
CLINICAL IMMUNOLOGY
(2023)