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《Cell reports》2020,30(1):153-163.e5
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Yafei Tian Yongping Zhang Shaoyan Hu Lilan Yao Yijian Zhu Shenglong Qiao Daru Lu Junjie Fan 《Blood and Genomics》2021,664(1):63-67
MYH9-related diseases (MYH9-RD) are a group of autosomal dominant diseases caused by mutations in the MYH9 gene, which are featured by thrombocytopenia, giant platelets and granulocyte cytoplasmic inclusion bodies. MYH9-RD patients generally suffer from bleeding syndromes, progressive kidney disease, deafness, or cataracts. Here, we reported on a case of MYH9-RD. A novel heterozygous mutation of MYH9 (c.2344-2345delGTinsTA, p.T782Y) was discovered by targeted sequencing technology. Immunofluorescence analysis of neutrophils confirmed abnormal aggregation of MYH9 protein. The results of this study should expand the MYH9 gene mutation spectrum and provide reference for subsequent researchers and genetic counseling. 相似文献
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Dun-Feng Xu Yu-Jian Liu Yan-Fei Mao Yan Wang Chu-Fan Xu Xiao-Yan Zhu Lai Jiang 《Journal of cellular and molecular medicine》2021,25(8):4124-4135
Thrombocytopenia is independently related with increased mortality in severe septic patients. Renin-angiotensin system (RAS) is elevated in septic subjects; accumulating studies show that angiotensin II (Ang II) stimulate the intrinsic apoptosis pathway by promoting reactive oxygen species (ROS) production. However, the mechanisms underlying the relationship of platelet apoptosis and RAS system in sepsis have not been fully elucidated. The present study aimed to elucidate whether the RAS was involved in the pathogenesis of sepsis-associated thrombocytopenia and explore the underlying mechanisms. We found that elevated plasma Ang II was associated with decreased platelet count in both patients with sepsis and experimental animals exposed to lipopolysaccharide (LPS). Besides, Ang II treatment induced platelet apoptosis in a concentration-dependent manner in primary isolated platelets, which was blocked by angiotensin II type 1 receptor (AT1R) antagonist losartan, but not by angiotensin II type 2 receptor (AT2R) antagonist PD123319. Moreover, inhibiting AT1R by losartan attenuated LPS-induced platelet apoptosis and alleviated sepsis-associated thrombocytopenia. Furthermore, Ang II treatment induced oxidative stress level in a concentration-dependent manner in primary isolated platelets, which was partially reversed by the AT1R antagonist losartan. The present study demonstrated that elevated Ang II directly stimulated platelet apoptosis through promoting oxidative stress in an AT1R-dependent manner in sepsis-associated thrombocytopenia. The results would helpful for understanding the role of RAS system in sepsis-associated thrombocytopenia. 相似文献
4.
Sihua Liu Hongyun Liu Keke Zhang Xueping Li Yuqin Duan Zhiyun Wang Tao Wang 《中国病毒学》2019,34(5):572-582
Severe fever with thrombocytopenia syndrome(SFTS) is an emerging hemorrhagic fever disease caused by SFTSV, a newly discovered phlebovirus that is named after the disease. Currently, no effective vaccines or drugs are available for use against SFTSV infection, as our understanding of the viral pathogenesis is limited. Bortezomib(PS-341), a dipeptideboronic acid analog, is the first clinically approved proteasome inhibitor for use in humans. In this study, the antiviral efficacy of PS-341 against SFTSV infection was tested in human embryonic kidney HEK293 T(293 T) cells. We employed four different assays to analyze the antiviral ability of PS-341 and determined that PS-341 inhibited the proliferation of SFTSV in 293 T cells under various treatment conditions. Although PS-341 did not affect the virus absorption, PS-341 treatment within a non-toxic concentration range resulted in a significant reduction of progeny viral titers in infected cells.Dual-luciferase reporter assays and Western blot analysis revealed that PS-341 could reverse the SFTSV-encoded nonstructural protein(NS) mediated degradation of retinoic acid-inducible gene-1(RIG-I), thereby antagonizing the inhibitory effect of NSs on interferons and blocking virus replication. In addition, we observed that inhibition of apoptosis promotes virus replication. These results indicate that targeting of cellular interferon pathways and apoptosis during acute infection might serve as the bases of future therapeutics for the treatment of SFTSV infections. 相似文献
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Nicola Vannini Vasco Campos Mukul Girotra Vincent Trachsel Shanti Rojas-Sutterlin Josefine Tratwal Simone Ragusa Evangelos Stefanidis Dongryeol Ryu Pernille Y. Rainer Gena Nikitin Sonja Giger Terytty Y. Li Aikaterini Semilietof Aurelien Oggier Yannick Yersin Loïc Tauzin Eija Pirinen Olaia Naveiras 《Cell Stem Cell》2019,24(3):405-418.e7
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Ping Zhang Guoyang Zhang Xiaoyan Liu Hongyun Liu Pengfeng Yang Liping Ma 《Journal of cellular biochemistry》2019,120(7):11274-11283
Immune thrombocytopenia (ITP) is a common autoimmune bleeding disorder. The breakdown of immune tolerance (regulatory T [Treg] cells and suppressor cytokines) plays an important role in ITP pathophysiology, especially in refractory ITP. Bone marrow–derived mesenchymal stem cells (BM-MSCs) show immunomodulatory properties and have been extensively utilized for autoimmune diseases. However, it has not been fully elucidated how BM-MSCs affect ITP. In this study, we explore the therapeutic mechanism of BM-MSCs on ITP in mice. Dose-escalation passive ITP mice were inducted by injection of MWReg30. BALB/c mice were randomly divided into two groups: ITP with BM-MSC transplantation and ITP controls. The serum levels of cytokines (interleukin 10 [IL-10] and transforming growth factor-β1 [TGF-β1]) were examined by enzyme-linked immunosorbent assays. The frequency of Treg cells in both peripheral blood and spleen mononuclear cells was analyzed by flow cytometry, and the forkhead box P3 (Foxp3) messenger RNA (mRNA) level was measured by real-time polymerase chain reaction. After BM-MSC treatment, the platelet (PLT) counts were significantly elevated. Meanwhile, cytokines (TGF-β1 and IL-10), the ratios of Treg cells, and the Foxp3 mRNA expression level were significantly higher in the BM-MSC group. Our results show that BM-MSCs can improve PLT counts mainly by secreting suppressive cytokines and upregulating Tregs, which may provide new therapeutic potential for human ITP. 相似文献
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摘要 目的:为探讨淋巴细胞及其亚群、NK细胞与儿童原发性免疫性血小板减少症(ITP)复发的关系,评估其预测价值,为临床评估患者预后提供理论支持。方法:回顾性分析2017年12月-2021年12月于新疆医科大学第一附属医院儿科中心初发首诊为原发性免疫性血小板减少症的165例患儿,根据是否复发分为复发组与无复发组,评估ITP复发的影响因素,利用ROC曲线评估淋巴细胞计数绝对值对儿童ITP复发的预测价值,运用Kaplan-Meier法绘制与淋巴细胞计数绝对值相关的儿童ITP无复发生存曲线。结果:共纳入165名ITP患儿,复发率24.8%。淋巴细胞计数绝对值对儿童ITP无复发的ROC曲线下面积为0.704,95%CI为0.613-0.795(P<0.05),最佳截断值为3.21×109/L。儿童ITP是否复发与年龄、淋巴细胞计数、出血评分、ESR有关,两组比较差异有统计学意义(P<0.05)。儿童ITP是否复发与CD3+CD19-细胞计数、CD3+CD4+细胞计数、CD3+CD8+细胞计数、CD4+/CD8+细胞比例、NK细胞计数有关,两组比较差异有统计学意义(P<0.05)。结论:淋巴细胞计数绝对值可作为评估儿童ITP复发的预测指标,儿童ITP复发与初始T淋巴细胞亚群、NK细胞计数具有一定相关性。 相似文献
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《Microbes and infection / Institut Pasteur》2015,17(2):149-154
Severe fever with thrombocytopenia syndrome (SFTS) is an emerging hemorrhagic fever in East Asia with case fatality up to 50%. SFTS is caused by SFTSV, a tick borne bunyavirus. In endemic area in China 1%–3% population was infected with SFTSV, but age is critical risk factor for hospitalization and death of SFTS patients. 相似文献
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重组人血小板生成素早期干预救治重症急性放射病猴的实验研究 总被引:1,自引:0,他引:1
重组人血小板生成素(rhTPO)是一种能促进巨核系祖细胞增殖、分化生成血小板的造血因子,研究表明它能促进射线照射小鼠造血功能恢复,前期工作证明rhTPO早期干预可显著提高致死剂量照射小鼠的活存率.本文以7.0Gy照射恒河猴为重度骨髓型急性放射病(ARS)模型,研究了rhTPO早期干预对重症ARS的治疗作用,并与WR2721和"500"的辐射防护作用进行了比较,结果发现rhTPO早期干预可明显促进ARS猴造血功能恢复,改善ARS猴症状,简化对症治疗措施,提高重度骨髓型ARS猴活存率,其对重度骨髓型ARS的防治作用优于现有的辐射防护药WR2721和"500",有望开发成安全有效的新型辐射防治药物. 相似文献
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