Results 81 to 90 of about 4,729,170 (302)

Ikaros family zinc finger 1 regulates dendritic cell development and function in humans

Nature Communications, 2018
IKZF1 is a transcription factor known to regulate mammalian B-cell development. Here the authors show that IKZF1 is required for human pDC development and regulation of DC cytokine production in patients with IKZF1 haploinsufficiency, findings which are ...
Urszula Cytlak, Anastasia Resteu, Delfien Bogaert, Hye Sun Kuehn, Thomas Altmann, Andrew Gennery, Graham Jackson, Attila Kumanovics, Karl V. Voelkerding, Seraina Prader, Melissa Dullaers, Janine Reichenbach, Harry Hill, Filomeen Haerynck, Sergio D. Rosenzweig, Matthew Collin, Venetia Bigley   +16 more
doaj   +1 more source

Optimization of B Cell Responses in Human Immune System Mice Through Organoid Based Screening

Advanced Science, EarlyView.
Human immune system (HIS) mice show limited B cell responses to immunization. Using HIS mouse spleen organoids, this study identifies cytokine combinations and innate immune signals that drive human B cell expansion, differentiation, and class‐switch. Applying these signals in vivo through temporal separation enables antigen‐specific IgG responses and ...
Haiqiao Sun, He Li, Xu Zhu, Zijian Zhang, Deshan Ren, Shuai Ding, Yan Li   +6 more
wiley   +1 more source

14 Years after Discovery: Clinical Follow-up on 15 Patients with Inducible Co-Stimulator Deficiency

Frontiers in Immunology, 2017
BackgroundInducible co-stimulator (ICOS) deficiency was the first monogenic defect reported to cause common variable immunodeficiency (CVID)-like disease in 2003.
Johanna Schepp, Janet Chou, Andrea Skrabl-Baumgartner, Peter D. Arkwright, Karin R. Engelhardt, Sophie Hambleton, Sophie Hambleton, Tomohiro Morio, Ekkehard Röther, Klaus Warnatz, Raif Geha, Bodo Grimbacher, Bodo Grimbacher   +12 more
doaj   +1 more source

Single‐Cell Profiling Identifies SLC2A5‐Mediated Fructose Metabolism as a Vulnerability in Primary CNS Lymphoma

Advanced Science, EarlyView.
Glucose deprivation in the primary CNS lymphoma (PCNSL) tumor microenvironment drives SLC2A5 (encoding GLUT5)‐dependent fructose metabolism in tumor cells, while hypoxia induces HIF‐mediated SLC2A5 expression in tumor‐supportive macrophages, revealing SLC2A5‐driven fructose utilization as a shared and targetable metabolic vulnerability across malignant
Qiaoli Wu, Qianru Zhang, Wenqiang Yan, Lu Sun, Yiran Cui, Bolin Liu, Guoqing Han, Feng Zhang, Ke Pu, Mingchao Zhang, Xiaobing Zhang, Qingguo Li, Gang An, Yuxuan Liu   +13 more
wiley   +1 more source

Early-onset primary antibody deficiency resembling common variable immunodeficiency challenges the diagnosis of Wiedeman-Steiner and Roifman syndromes

Scientific Reports, 2017
Syndromic primary immunodeficiencies are rare genetic disorders that affect both the immune system and other organ systems. More often, the immune defect is not the major clinical problem and is sometimes only recognized after a diagnosis has been made ...
Delfien J. Bogaert, Melissa Dullaers, Hye Sun Kuehn, Bart P. Leroy, Julie E. Niemela, Hans De Wilde, Sarah De Schryver, Marieke De Bruyne, Frauke Coppieters, Bart N. Lambrecht, Frans De Baets, Sergio D. Rosenzweig, Elfride De Baere, Filomeen Haerynck   +13 more
doaj   +1 more source

First Year of Israeli Newborn Screening for Severe Combined Immunodeficiency—Clinical Achievements and Insights

Frontiers in Immunology, 2017
Severe combined immunodeficiency (SCID), the most severe form of T cell immunodeficiency, is detectable through quantification of T cell receptor excision circles (TRECs) in dried blood spots obtained at birth.
Erez Rechavi, Atar Lev, Amos J. Simon, Tali Stauber, Suha Daas, Talia Saraf-Levy, Arnon Broides, Arnon Broides, Amit Nahum, Amit Nahum, Nufar Marcus, Nufar Marcus, Suhair Hanna, Suhair Hanna, Polina Stepensky, Polina Stepensky, Ori Toker, Ori Toker, Ilan Dalal, Ilan Dalal, Ilan Dalal, Ilan Dalal, Amos Etzioni, Amos Etzioni, Shlomo Almashanu, Raz Somech, Raz Somech, Raz Somech   +27 more
doaj   +1 more source

Targeting the ATX‐LPA Axis Overcomes TKI Resistance and Immunosuppression in Renal Cell Carcinoma via Dual Inhibition of AKT/mTOR and TBK1/IRF3 Pathways

Advanced Science, EarlyView.
ABSTRACT Background Therapeutic resistance limits durable survival in advanced/metastatic renal cell carcinoma (RCC) treated with first‐line tyrosine kinase inhibitor (TKI) plus immune checkpoint inhibitor (ICI). We sought to define key resistance drivers and actionable targets.
Jinchen Luo, Hansen Lin, Haoqian Feng, Lei Tan, Xi Liu, Yong Huang, Junjie Cen, Jiajie Chen, Xinwei Zhou, Mingjie Lin, Wuyuan Liao, Zheyu Ai, Minyu Chen, Yinghan Wang, Wei Chen, Junhang Luo, Yanping Liang   +16 more
wiley   +1 more source

Penfluridol Triggers GSDME‐Mediated Immunogenic Pyroptosis to Potentiate Antitumor Immunotherapy

Advanced Science, EarlyView.
A high‐throughput screen of FDA‐approved antipsychotics identifies penfluridol as a potent pyroptosis inducer acting via direct TTI1 inhibition. This triggers TNFA‐NFKB signaling and caspase‐8/‐3‐dependent GSDME cleavage. The compound stimulates antitumor immunity alone and synergizes with anti‐PD‐1 therapy, while low TTI1 expression emerges as a ...
Linfeng Li, Daishi Li, Danyao Chen, Yating Dian, Lei Yao, Hui Su, Ziyu Guo, Deze Zhao, Zihua Wu, Furong Zeng, Chunfang Zhang, Guangtong Deng   +11 more
wiley   +1 more source

Defective germinal center selection results in persistence of self-reactive B cells from the primary to the secondary repertoire in Primary Antiphospholipid Syndrome

Nature Communications
Primary antiphospholipid syndrome (PAPS) is a life-threatening clotting disorder mediated by pathogenic autoantibodies. Here we dissect the origin of self-reactive B cells in human PAPS using peripheral blood and bone marrow of patients with triple ...
Yannick Dieudonné, Raquel Lorenzetti, Julien Rottura, Iga Janowska, Quentin Frenger, Léa Jacquel, Olivier Vollmer, Francesco Carbone, Zhu Chengsong, Marine Luka, Sabine Depauw, Nadège Wadier, Stéphane Giorgiutti, Benoît Nespola, Agathe Herb, Reinhard Edmund Voll, Aurélien Guffroy, Vincent Poindron, Mickaël Ménager, Thierry Martin, Pauline Soulas-Sprauel, Marta Rizzi, Anne-Sophie Korganow, Vincent Gies   +23 more
doaj   +1 more source

Immune defects in active mycobacterial diseases in patients with primary immunodeficiency diseases (PIDs)

Journal of the Formosan Medical Association, 2011
Natural human immunity to the mycobacteria group, including Mycobacterium tuberculosis, Bacille Calmette-Guérin (BCG) or nontuberculous mycobacteria (NTM), and/or Salmonella species, relies on the functional IL-12/23-IFN-γ integrity of macrophages ...
Wen-I Lee, Jing-Long Huang, Kuo-Wei Yeh, Tang-Her Jaing, Tzou-Yien Lin, Yhu-Chering Huang, Cheng-Hsun Chiu   +6 more
doaj   +1 more source