keyword
https://read.qxmd.com/read/38504982/case-report-a-case-of-novel-homozygous-lrba-variant-induced-by-chromosomal-segmental-uniparental-disomy-genetic-and-clinical-insights
#1
Lihua Jiang, Sen Chen
OBJECTIVE: The study aims to report a rare case of a novel homozygous variant in the LRBA gene, originating from uniparental disomy of paternal origin. This case contributes new clinical data to the LRBA gene variant database. METHODS: The study details the case of a 2-year-old child diagnosed in May 2023 at our center with a homozygous LRBA gene variant. Detailed clinical data of the patient were collected, including whole-exome sequencing of peripheral blood mononuclear cells, with parental genetic verification...
2024: Frontiers in Immunology
https://read.qxmd.com/read/38422768/the-xpo1-inhibitor-selinexor-ameliorates-bleomycin-induced-pulmonary-fibrosis-in-mice-via-gbp5-nlrp3-inflammasome-signaling
#2
JOURNAL ARTICLE
Jia Zhang, Yihua Zhang, Qi Chen, Yong Qi, Xiaoju Zhang
Pulmonary fibrosis is an irreversible and progressive lung disease with limited treatments available. Selinexor (Sel), an orally available, small-molecule, selective inhibitor of XPO1, exhibits notable antitumor, anti-inflammatory and antiviral activities. However, its potential role in treating pulmonary fibrosis is unknown. C57BL/6J mice were used to establish a pulmonary fibrosis model by intratracheal administration of bleomycin (BLM). Subsequently, Sel was administered intraperitoneally. Our data demonstrated that Sel administration ameliorated BLM-induced pulmonary fibrosis by increasing mouse body weights; reducing H&E staining, Masson staining scores, and shadows in mouse lung computed tomography (CT) images, decreasing the total cell and neutrophil counts in the lung and bronchoalveolar lavage fluid (BALF); and decreasing the levels of TGF-β1...
February 28, 2024: International Immunopharmacology
https://read.qxmd.com/read/38053293/highly-efficient-genome-editing-via-crispr-cas9-ribonucleoprotein-rnp-delivery-in-mesenchymal-stem-cells
#3
A Reum Han, Ha Rim Shin, Jiyeon Kweon, Soo Been Lee, Sang Eun Lee, Eun-Young Kim, Eun-Ju Chang, Yongsub Kim, Seong Who Kim
The CRISPR-Cas9 system has significantly advanced regenerative medicine research by enabling genome editing in stem cells. Due to their desirable properties, mesenchymal stem cells (MSCs) have recently emerged as highly promising therapeutic agents, which properties include differentiation abilit and cytokine production. While CRISPR-Cas9 technology is applied to develop MSC-based therapeutics, MSCs exhibit inefficient genome editing, and susceptibility to plasmid DNA. In this study, we compared and optimized plasmid DNA and RNP approaches for efficient genome engineering in MSCs...
December 6, 2023: BMB Reports
https://read.qxmd.com/read/37400575/higher-cd4-cd40-%C3%A2-t-cells-th40-cells-associate-with-systemic-lupus-erythematosus-activity
#4
JOURNAL ARTICLE
Lihua Zhu, Guangmei Song, Xiaohui Chen, Yue Zhang, Yanjie Cui, Jie Qiao, Xinran Huang, Xueqin Li, Xiaoen Liu, Xiangbo Zeng, Yangqiu Li, Liang Wang, Bo Li
The aim of this study was to investigate the characteristics of CD4+ CD40+  T cells (Th40 cells) in Chinese systemic lupus erythematosus (SLE) patients. Flow cytometry was used to identify the percentage of Th40 cells in peripheral blood from 24 SLE patients and 24 healthy individuals and the level of IL-2, IL-4, IL-6, IL-10, IFN-r, and TNF-α in serum (22 cases) from the SLE patients. Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2000) was used to assess the SLE disease active state...
July 3, 2023: Scientific Reports
https://read.qxmd.com/read/37329903/investigation-of-a-subunit-protein-vaccine-for-hfrs-based-on-a-consensus-sequence-between-envelope-glycoproteins-of-htnv-and-seov
#5
JOURNAL ARTICLE
Rongrong Liu, Yunhua Lv, Wenjie Sun, Min Li, Ningning Ge, Cheng Zhu, Yaxin Ding, Ziyu Liu, Ruixue Ma, Yuxiao Huang, Shiyuan Hou, Qikang Ying, Tianle Gu, Fang Wang, Lingling Nie, Youchun Wang, Weijin Huang, Jiayi Shu, Xingan Wu
Due to the global resurgence of hemorrhagic fever with renal syndrome (HFRS), more attention is being focused on this dangerous illness. In China and Korea, the only vaccine available is the virus-inactivated vaccine against Hantaan virus (HTNV) or Seoul virus (SEOV), but their efficacy and safety of these vaccines are inadequate. Therefore, it is important to develop new vaccines that are safer and more efficient to neutralize and regulate areas with a high prevalence of HFRS. We employed bioinformatics methods to design a recombinant protein vaccine based on conserved regions of protein consensus sequences in HTNV and SEOV membranes...
June 15, 2023: Virus Research
https://read.qxmd.com/read/37209277/daphnetin-attenuates-intestinal-inflammation-oxidative-stress-and-apoptosis-in-ulcerative-colitis-via-inhibiting-reg3a-dependent-jak2-stat3-signaling-pathway
#6
JOURNAL ARTICLE
Zhi He, Jingjing Liu, Yang Liu
Daphnetin is a natural coumarin compound with anti-inflammatory, anti-oxidant, and anti-apoptotic effects, which has been previously demonstrated to ameliorate DSS-induced ulcerative colitis (UC). However, the molecular mechanism involved in the daphnetin-mediated pathological process of UC remains unclarified. The current study used DSS-induced mice and LPS-challenged Caco-2 cells as UC models. Bodyweight, disease activity index (DAI) score, and colon length were used to evaluate the severity of colitis. The histological changes in colon tissues were observed using H&E and PAS staining...
May 20, 2023: Environmental Toxicology
https://read.qxmd.com/read/37169953/combinatorial-immunotherapy-with-gemcitabine-and-ex-vivo-expanded-nk-cells-induces-anti-tumor-effects-in-pancreatic-cancer
#7
JOURNAL ARTICLE
Eun-Kyoung Koh, Hong-Rae Lee, Woo-Chang Son, Ga-Young Park, Juhee Kim, Jae-Ho Bae, You-Soo Park
Pancreatic cancer is difficult to diagnose at the initial stage and is often discovered after metastasis to nearby organs. Gemcitabine is currently used as a standard treatment for pancreatic cancer. However, since chemotherapy for pancreatic cancer has not yet reached satisfactory therapeutic results, adjuvant chemotherapy methods are attempted. It can be expected that combining immune cell therapy with existing anticancer drug combination treatment will prevent cancer recurrence and increase survival rates...
May 11, 2023: Scientific Reports
https://read.qxmd.com/read/36851279/immune-responses-to-hbv-vaccine-in-people-living-with-hiv-plwhs-who-achieved-successful-treatment-a-prospective-cohort-study
#8
JOURNAL ARTICLE
Ling Xu, Li Zhang, Shuang Kang, Xiaodi Li, Lianfeng Lu, Xiaosheng Liu, Xiaojing Song, Yanling Li, Xiaoxia Li, Wei Lyu, Wei Cao, Zhengyin Liu, Taisheng Li
BACKGROUND: Understanding immune responses after HBV vaccination is important to prevent HBV infection in PLWH and to achieve successful treatment. METHODS: Thirty-two PLWHs with CD4+ cell count > 350 cells/µL and HIV RNA < 200 copies/mL were vaccinated with 20 µg of HBV vaccine at weeks 0, 4, and 24 in this prospective study. We measured total HIV DNA levels, HBsAb titers and HBsAg-specific T-cell responses during follow-up time. RESULTS: All patients achieved protective HBsAb titer after immunization...
February 9, 2023: Vaccines
https://read.qxmd.com/read/36195489/th1-th2-cytokine-levels-a-potential-diagnostic-tool-for-patients-with-necrotizing-fasciitis
#9
JOURNAL ARTICLE
Xiang-Wei Ling, Ting-Ting Zhang, Meng-Meng Ling, Wei-Hao Chen, Chun-Hui Huang, Guo-Liang Shen
INTRODUCTION: Necrotizing fasciitis (NF) has emerged as rare but rapidly progressive, life-threatening severe skin and soft tissue infection. We conducted a study to investigate whether Th1/Th2 cytokines could serve as biomarkers to distinguish NF from class III skin and soft tissue infections (SSTIs). METHODS: A retrospective review was performed for 155 patients suffering from serious skin and soft tissue infections from October 2020 to February 2022. Th1/Th2 cytokines were obtained from peripheral blood and wound drainage fluid samples...
September 3, 2022: Burns
https://read.qxmd.com/read/35965593/early-changes-in-peripheral-blood-cytokine-levels-after-the-treatment-of-metastatic-hepatic-carcinoma-with-callispheres-microspheres-drug-eluting-beads-transcatheter-arterial-chemoembolization
#10
JOURNAL ARTICLE
Ying Liu, Song Liu, Guang Sheng Zhao, Xiang Li, Fei Gao, Zhi Zhong Ren, Jie Bian, Jian Lin Wu, Yue Wei Zhang
Objective: To observe the early changes in peripheral blood cytokine levels after treatment of metastatic hepatic carcinoma (MHC) with CalliSpheres microspheres drug-eluting beads (DEB) transcatheter arterial chemoembolization (CSM-TACE). Methods: Twenty-eight patients with refractory MHC who underwent CSM-TACE were selected prospectively, and 5mL of peripheral blood was collected before CSM-TACE and on the 2nd and 5th day after CSM-TACE. Flow cytometry was used to detect immunological indicators...
2022: Frontiers in Oncology
https://read.qxmd.com/read/35556653/virus-like-vesicles-vlvs-as-therapeutic-vaccine-vectors-for-hepatocellular-carcinoma
#11
JOURNAL ARTICLE
Lei Yang, Kien Pham, Timur O Yarovinsky, Samuel M Defina, John K Rose, Chen Liu
Hepatocellular Carcinoma (HCC) is the most common form of liver cancer and is the leading cause of cancer-related mortality globally. Despite the substantial progress that has been made in current treatments, HCC remains malignancy with a high recurrence rate due to a poor prognosis. Therefore, developing novel immunotherapy approaches to treat HCC is crucial to solving this global public health problem. Anti-tumor vaccines are promising strategies to elicit immune responses for anti-tumor immunity. In this study, we designed and cloned an alphavirus-vesiculovirus hybrid vaccine vector VLV-GPC3 that can generate virus-like vesicles (VLVs) as self-amplifying RNA replicons to additionally express GPC3, a cell-surface heparan sulfate proteoglycan is highly expressed in HCC...
May 2022: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://read.qxmd.com/read/34691022/il-27-mediates-pro-inflammatory-effects-via-the-erk-signaling-pathway-during-preterm-labor
#12
JOURNAL ARTICLE
Dongni Huang, Yuxin Ran, Zheng Liu, Jie He, Nanlin Yin, Hongbo Qi
Preterm labor (PTL) is a multifactorial syndrome that results in birth prior to 37 weeks of gestation. However, the specific molecular mechanisms underlying this condition have yet to be elucidated. Previous research demonstrated that the abnormal expression of IL-27, and its receptors, played a role in the pathophysiology of preterm labor. In the present study, we established a Lipopolysaccharide (LPS)-stimulated, infection-induced, preterm mouse model based on wild-type C57BL/6 mice and WSX-1-/- C57BL/6 mice...
2021: Frontiers in Immunology
https://read.qxmd.com/read/34675597/interferon-driven-immune-dysregulation-in-down-syndrome-a-review-of-the-evidence
#13
REVIEW
Howard Chung, Peter H R Green, Timothy C Wang, Xiao-Fei Kong
Down syndrome (DS) is a unique genetic disease caused by the presence of an extra copy of chromosome 21, which carries four of the six interferon receptor (IFN-R) genes on its long arm. Recent studies reporting higher levels of interferon-stimulated gene (ISG) expression in primary immune cells studied ex vivo have suggested that the additional copies of the IFN-R genes in DS result in mild interferonopathy. In this review, we analyze the potential clinical and immunological impacts of this interferonopathy in DS...
2021: Journal of Inflammation Research
https://read.qxmd.com/read/34599024/activation-of-gm-csf-and-tlr2-signaling-synergistically-enhances-antigen-specific-antitumor-immunity-and-modulates-the-tumor-microenvironment
#14
JOURNAL ARTICLE
Wan-Lun Yan, Chiao-Chieh Wu, Kuan-Yin Shen, Shih-Jen Liu
BACKGROUND: The major challenge of antitumor immunotherapy is dealing with the immunosuppressive tumor microenvironment, which involves immature myeloid cell accumulation that results in T cell dysfunction. Myeloid cell activation is induced by Toll-like receptor agonists. Additionally, granulocyte/macrophage colony stimulating factor (GM-CSF) promotes myelopoiesis and recruits myeloid cells. Here, we combined the Toll-like receptor 2 (TLR2) agonist lipoprotein and GM-CSF to assess whether this bifunctional immunotherapy has synergistic effects on myeloid cells and could be further developed as a therapeutic intervention that enhances the antitumor response...
October 2021: Journal for Immunotherapy of Cancer
https://read.qxmd.com/read/33446504/prior-activation-state-shapes-the-microglia-response-to-antihuman-trem2-in-a-mouse-model-of-alzheimer-s-disease
#15
JOURNAL ARTICLE
Daniel C Ellwanger, Shoutang Wang, Simone Brioschi, Zhifei Shao, Lydia Green, Ryan Case, Daniel Yoo, Dawn Weishuhn, Palaniswami Rathanaswami, Jodi Bradley, Sara Rao, Diana Cha, Peng Luan, Shilpa Sambashivan, Susan Gilfillan, Samuel A Hasson, Ian N Foltz, Menno van Lookeren Campagne, Marco Colonna
Triggering receptor expressed on myeloid cells 2 (TREM2) sustains microglia response to brain injury stimuli including apoptotic cells, myelin damage, and amyloid β (Aβ). Alzheimer's disease (AD) risk is associated with the TREM2 R47H variant, which impairs ligand binding and consequently microglia responses to Aβ pathology. Here, we show that TREM2 engagement by the mAb hT2AB as surrogate ligand activates microglia in 5XFAD transgenic mice that accumulate Aβ and express either the common TREM2 variant ( TREM2 CV ) or TREM2 R47H scRNA-seq of microglia from TREM2 CV -5XFAD mice treated once with control hIgG1 exposed four distinct trajectories of microglia activation leading to disease-associated (DAM), interferon-responsive (IFN-R), cycling (Cyc-M), and MHC-II expressing (MHC-II) microglia types...
January 19, 2021: Proceedings of the National Academy of Sciences of the United States of America
https://read.qxmd.com/read/33357705/comparative-analysis-of-key-immune-protection-factors-in-h9n2-avian-influenza-viruses-infected-and-immunized-specific-pathogen-free-chicken
#16
JOURNAL ARTICLE
Manman Dai, Shibing Li, Keyi Shi, Hui Sun, Li Zhao, Deshui Yu, Jiayu Liao, Chenggang Xu, Ming Liao
H9N2 avian influenza viruses (AIV) continue to circulate in vaccinated chicken flocks in China, which prompted us to investigate the differential immune protection factors induced by H9N2 AIV infection and immunization for analyzing the reason of protection deficiency of H9N2 AIV inactivated vaccine. In this study, we firstly explored virus-induced optimal immune responses in chicken after H9N2 AIV infection. And, we found that H9N2 hemagglutination inhibition (HI) antibody level, antiviral interferon-stimulated genes including 2',5'-oligoadenylate synthetase-like and myxovirus resistance 1, CD8+ T cell response in peripheral blood lymphocytes (PBL) accompanied by the cytotoxicity-associated genes, including poly (ADP-ribose) polymerase and IFN-r play important roles in defending against H9N2 infection...
January 2021: Poultry Science
https://read.qxmd.com/read/33123600/the-effectiveness-of-rapamycin-combined-with-eltrombopag-in-murine-models-of-immune-mediated-bone-marrow-failure
#17
JOURNAL ARTICLE
Shaoxue Ding, Xiaowei Liang, Tian Zhang, Rong Fu
Severe aplastic anemia (SAA) is a rare disease characterized by severe pancytopenia and bone marrow failure. Most patients with AA respond to immunosuppressive therapy (IST), usually as antithymocyte globulin (ATG) and cyclosporine (CsA), but some relapse on CsA withdrawal or require long-term administration of CsA to maintain blood counts. Recent research has found that rapamycin (Rapa) was an effective therapy in mouse models of immune-mediated bone marrow failure. However, it has not achieved a satisfactory effect in clinical application...
2020: Journal of Immunology Research
https://read.qxmd.com/read/32953887/effect-of-pingchuan-formula-on-toll-like-receptors-and-dendritic-cells-in-an-asthmatic-mouse-model
#18
JOURNAL ARTICLE
Fei Liu, Li Bai, Zheng Xue, Wei Pan, Jianer Yu
Pingchuan formula (PCF) was created by Professor Yu Jianer. The purpose of this study was to investigate the effect of PCF on dendritic cells (DCs) and toll-like receptors (TLRs) in initiating immunity. A bronchial asthma BALB/c mouse model was established using an OVA excitation method. PCF was immediately administered by gavage after the first excitation. After 7 d, hematoxylin and eosin (HE) staining was used to observe the pathological changes in the asthma model. Eosinophil infiltration and concentrations of IL-4, IFN-r, IL-12, and IFN- α in BALF were determined by enzyme-linked immunosorbent assay (ELISA)...
2020: BioMed Research International
https://read.qxmd.com/read/32744026/bushen-jiangzhi-formula-reduces-atherosclerosis-in-apoe-mice-through-autophagy
#19
JOURNAL ARTICLE
Hui Cao, Qingling Jia, Dingzhu Shen, Li Yan, Chuan Chen, Sanli Xing, Fangfang Dou
OBJECTIVE: To study the effect of Bushen Jiangzhi formula (BSJZF) on atherosclerosis (AS) in apolipoprotein E knockout (apoE-/-) mice and the underlying mechanism. METHODS: We used a high fat diet to induce AS in apoE-/- mice. The mice were randomly divided into four groups: model, BSJZF, atorvastatin, and 3-methyladenine groups. Syngeneic C57BL/6 mice of the same age were used for the control group. Autophagosomes in the aorta were examined by transmission electron microscopy...
August 2020: Journal of Traditional Chinese Medicine
https://read.qxmd.com/read/32572726/three-copies-of-four-interferon-receptor-genes-underlie-a-mild-type-i-interferonopathy-in-down-syndrome
#20
JOURNAL ARTICLE
Xiao-Fei Kong, Lisa Worley, Darawan Rinchai, Vincent Bondet, Puthen Veettil Jithesh, Marie Goulet, Emilie Nonnotte, Anne Sophie Rebillat, Martine Conte, Clotilde Mircher, Nicolas Gürtler, Luyan Liu, Mélanie Migaud, Mohammed Elanbari, Tanwir Habib, Cindy S Ma, Jacinta Bustamante, Laurent Abel, Aimé Ravel, Stanislas Lyonnet, Arnold Munnich, Darragh Duffy, Damien Chaussabel, Jean-Laurent Casanova, Stuart G Tangye, Stéphanie Boisson-Dupuis, Anne Puel
Down syndrome (DS) is characterized by the occurrence of three copies of human chromosome 21 (HSA21). HSA21 contains a cluster of four interferon receptor (IFN-R) genes: IFNAR1, IFNAR2, IFNGR2, and IL10RB. DS patients often develop mucocutaneous infections and autoimmune diseases, mimicking patients with heterozygous gain-of-function (GOF) STAT1 mutations, which enhance cellular responses to three types of interferon (IFN). A gene dosage effect at these four loci may contribute to the infectious and autoimmune manifestations observed in individuals with DS...
August 2020: Journal of Clinical Immunology
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