Results 61 to 70 of about 10,056 (300)

An immunocompetent patient with a nonsense mutation in NHEJ1 gene

BMC Medical Genetics, 2019
Background DNA double-strand breaks (DSBs) are among the most deleterious types of DNA damage. DSBs are repaired by homologous recombination or non-homologous end-joining (NHEJ).
Hossein Esmaeilzadeh, Mohammad Reza Bordbar, Zahra Hojaji, Parham Habibzadeh, Dorna Afshinfar, Mohammad Miryounesi, Majid Fardaei, Mohammad Ali Faghihi   +7 more
doaj   +1 more source

Tissue‐Resident Macrophage‐Derived E3 Ligase SMURF2 Restricts Autoimmune Inflammation by Mediating the Degradation of p‐TBK1

Advanced Science, EarlyView.
Dysregulated Tissue resident macrophage (TRMs) link to autoimmune inflammation. SMURF2 mediates Lys‐27 (K27)‐linked ubiquitination of p‐TBK1 and its degradation, which inhibits CSF1R signaling‐triggered TRM proliferation, thereby restraining the autoimmune inflammation.
Xiang An, Jun Li, Lingling Wang, Chengyuan Li, Zhenpeng Jin, Yushi Yao, Minmin Jiang, Wenlong Lin, Xiaojian Wang   +8 more
wiley   +1 more source

TRIAGEM NEONATAL DE IMUNODEFICIÊNCIAS GRAVES COMBINADAS POR MEIO DE TRECS E KRECS: SEGUNDO ESTUDO PILOTO NO BRASIL

Revista Paulista de Pediatria
RESUMO Objetivo: Validar a quantificação de T-cell receptor excision circles (TRECs) e kappa-deleting recombination circles (KRECs) por reação em cadeia de polimerase (polymerase chain reaction, PCR) em tempo real (qRT-PCR), para triagem neonatal de ...
Marilia Pyles P. Kanegae, Lucila Akune Barreiros, Jusley Lira Sousa, Marco Antônio S. Brito, Edgar Borges de Oliveira Junior, Lara Pereira Soares, Juliana Themudo L. Mazzucchelli, Débora Quiorato Fernandes, Sonia Marchezi Hadachi, Silvia Maia Holanda, Flavia Alice T. M. Guimarães, Maura Aparecida P. V. V. Boacnin, Marley Aparecida L. Pereira, Joaquina Maria C. Bueno, Anete Sevciovic Grumach, Regina Sumiko W. Di Gesu, Amélia Miyashiro N. dos Santos, Newton Bellesi, Beatriz T. Costa-Carvalho, Antonio Condino-Neto   +19 more
doaj   +1 more source

Novel Concept of CD4-Mediated Activation of Regulatory T Cells for the Treatment of Graft-Versus-Host Disease

Frontiers in Immunology, 2017
Allogeneic hematopoietic stem cell transplantation is the only curative treatment option for several hematological malignancies and immune deficiency syndromes.
Janine Schlöder, Carsten Berges, Andrea Tuettenberg, Helmut Jonuleit   +3 more
doaj   +1 more source

Neural Circuits between Nodose Ganglion and Pulmonary Neuroendocrine Cells Regulate Lung Inflammatory Responses

Advanced Science, EarlyView.
TRPA1+αCGRP+ sensory neurons in the nodose ganglion detect external insults such as lipopolysaccharide (LPS) and interact directly with pulmonary neuroendocrine cells (PNECs), promoting their activation and proliferation. This neural‐epithelial interaction amplifies lung inflammation.
Jie Chen, Shitao Xie, Zhekai Lin, Caiqi Zhao, Rujia Tao, Yingying Ma, Xiaoyan Chen, Renlan Wu, Qingjian Han, Pengfei Sui, Sheng Wang, Hongbin Ji, Hai Song, Xiaoming Zhang, Yangang Sun, Yuanlin Song, Xiao Su   +16 more
wiley   +1 more source

Clinical, Immunological, and Molecular Heterogeneity of 173 Patients With the Phenotype of Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked (IPEX) Syndrome

Frontiers in Immunology, 2018
Background: Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-linked (IPEX) Syndrome is a rare recessive disorder caused by mutations in the FOXP3 gene. In addition, there has been an increasing number of patients with wild-type FOXP3 gene and, in
Eleonora Gambineri, Eleonora Gambineri, Sara Ciullini Mannurita, Sara Ciullini Mannurita, David Hagin, Marina Vignoli, Marina Vignoli, Stephanie Anover-Sombke, Stacey DeBoer, Gesmar R. S. Segundo, Eric J. Allenspach, Claudio Favre, Hans D. Ochs, Troy R. Torgerson   +13 more
doaj   +1 more source

Extracellular Vesicle‐Delivered tRF‐His‐GTG‐1 Reprograms Neutrophil Lipophagy and Triggers Inflammation in COVID‐19

Advanced Science, EarlyView.
This study identifies platelet‐derived extracellular vesicles as key immunometabolic regulators in COVID‐19. The delivery of tRF‐His‐GTG‐1 to neutrophils activates TLR8mTOR signaling, disrupts lipophagy, and amplifies NET‐mediated inflammation. Importantly, targeting this axis restores neutrophil homeostasis, offering a potential therapeutic strategy ...
Tsai‐Ling Liao, Po‐Yu Liu, Yi‐Ming Chen, Kuo‐Tung Tang, Hung‐Jen Liu, Der‐Yuan Chen   +5 more
wiley   +1 more source

TREM2 Drives Neutrophil Extracellular Traps‐Induced Dendritic Cell Maturation and Contributes to Lupus Progression

Advanced Science, EarlyView.
TREM2 recognizes NETs‐derived MPO to promote DC maturation and antigen presentation, thereby exacerbating the autoimmune response in SLE. Mechanistically, TREM2 activation triggers the DAP12/SYK/ERK cascade and enhances NETs internalization by DCs, which in turn activates the cGAS/STING signaling pathway.
Jingxian Shu, Jiabi Liang, Linda Zeng, Shuping Zhong, Xueling Fang, Yating Xu, Yongjian Wu, Xi Huang   +7 more
wiley   +1 more source

Imunodeficiências primárias: aspectos relevantes para o pneumologista Primary immunodeficiency diseases: relevant aspects for pulmonologists

Jornal Brasileiro de Pneumologia, 2009
As imunodeficiências primárias são um grupo de doenças geneticamente heterogêneas que afetam diferentes componentes da imunidade inata e adaptativa, como neutrófilos, macrófagos, células dendríticas, proteínas do sistema complemento, células natural ...
Pérsio Roxo Júnior
doaj   +1 more source

Retinoic Acid Reprograms Mast Cells Toward a Proinflammatory State to Enhance Antitumor Immunity

Advanced Science, EarlyView.
Proinflammatory mast cells represent an MHC‐IIhigh, cytokine‐producing mast cell subset associated with improved survival and enhanced responses to anti‐PD‐1 therapy. Retinoic acid drives their polarization, enabling antigen uptake and presentation, and T cell recruitment and activation, collectively promoting adaptive anti‐tumor immunity.
Lizao Zhang, Siqi Ren, Yuhui Li, Rongxi Chen, Ling Qiu, Yongmei Tan, Suling Chen, Huiqian Wu, Lianxi Mai, Xiao Tan, Xin Liu, Peisheng Liang, Shijia Kuang, Liansheng Wang, Jingkang Liu, Jintao Li, Yanyan Li, Qiuping Xu, Yongzhi Su, Yuehai Luo, Binyan Wu, Zijing Huang, Haotian Cao, Qunxing Li, Jinsong Li, Tianjun Lan   +25 more
wiley   +1 more source