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第十届全国免疫学学术大会

作者： Xuan Zhang Rongxin Dai Hongqiang Du Guangjin Luo Junfeng Wu Wenxia Song Linlin Niu Yunfei An Zhiyong Zhang Yuan Ding Xiaodong Zhao Division of Immunology Children’s Hospital of Chongqing Medical University Department of Cell Biology and Molecular Genetics University of Maryland

Objects:To analyze the impact of WAS gene mutation on the number and function of follicular helper T cells in children with Wiskott-Aldrich syndrome(WAS)and WAS KO mice.Methods:Fresh blood samples of 35 WAS patients and 35 age-matched healthy children(HC)were collected.The frequency of circulating Tfh cells,subsets of circulating Tfh cells,and IL21-secreting Tfh were detected by flow cytometry(FCM).CD4+T cells of WAS patients and HC were sorted by FCM.The m RNA expression of Bcl6 and Blimp-1 in CD4+T cells of WAS patients and HC were detected by q PCR.Ten WAS KO mices and10 WT mices were randomly chosen as immune group and NS control group respectively.Each group enrolled5 mice.For immunization,400μL NS and 400μg NP-KLH was injected s.***.on the mouse flanks at d1 and d30 separately.All of the animals were sacrificed 5 days after secondary immunization.The frequency of Tfh cells in blood and spleen cells was detected by FCM.R e s u l t s:In WAS patients,the number of circulating Tfh cells was significantly reduced.Meanwhile BCL6 expression was decreased in CD4+T cells of WAS patients compared to controls.Although cytokine IL21 secreting was not affected,circulating Tfh cells in WAS patients were T helper(h)2 and Th17 polarized and with higher expression of inducible T-cell co-stimulator(ICOS).The frequency of Tfh cells in both the blood and spleen of WAS knockout(KO)mice was significantly decreased after secondary immunization,as was the frequency of Bcl6+Tfh cells in spleen,but the frequency of Icos+germinal center Tfh cells in both blood and spleen was significantly increased.Conclusions:Similar alterations in Tfh cells from both WAS patients and WAS ko mice suggest WASp contributes to the development and memo-ry response of Tfh cells,by regulating signaling that maintains the transcription level of BCL6.

关键词： T follicular helper cell Wiskott-Aldrich syndrome ICOS BCL6 memory response

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TCR diversity is selectively skewed in T-cell populations of Wiskott-Aldrich syndrome patients

TCR diversity is selectively skewed in T-cell populations of...

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第九届全国免疫学学术大会

作者： Junfeng Wu Dawei Liu Wenwei Tu Wenxia Song Xiaodong Zhao Ministry of Education Key Laboratory of Child Development and Disorders Children's Hospital of Chongqing Medical University Department of Paediatrics & Adolescent Medicine LKS Faculty of Medicinethe University of Hong Kong Department of Cell Biology & Molecular Genetics University of MarylandCollege ParkMD 20742U.S.A

Background:Wiskott-Aldrich syndrome(WAS)is a severe disorder characterized by thrombocytopenia,eczema,immunodeficiency,and increased risk of autoimmune disease and lymphoid malignancies.The immunodeficiency caused by lack of WAS protein(WASP)expression has been mainly attributed to defective T-cell functions.Whether WAS mutations differentially influence the T cell receptor(TCR)diversity of different T-cell subsets is unknown.Objective:We aimed to identify the degree and the pattern of skewing in the variable region of theβ-chain(Vβ)TCR repertoire in different T-cell subsets from patients with WAS.Methods:The TCR repertoire diversity in total peripheral T cells,sorted CD4+and CD8+T cells,CD45RA+(CD45RA+CD45RO-)and CD45RO+(CD45RA-CD45RO+)CD4+and CD8+T cells from patients with WAS and age-matched healthy controls were analyzed and compared,using spectratyping,of complementarity-determining region3(CDR3);and the CDR3 region of TCRβtranscripts in CD45RA+CD4+and CD8+T cells,CD45RO+CD4+T cells,CD8+terminally differentiated effctor memory T cells(Temra)and naive CD8+T(CD8+CD45RO-CCR7+)cells from patients and controls were analyzed and compared by high-throughput sequencing(HTS).Results:The TCR repertoire diversity in CD45RO+CD4+T cells and CD8+Temra of WAS patients was significantly skewed,compared to that in age-matched controls.Conclusion:Our results indicate that WAS gene mutations selectively influence the repertoire development or expansion of CD45RO+(memory)CD4+T cells.

关键词： Wiskott-Aldrich syndrome TCR diversity CDR3 spectratyping high-throughput sequencing

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Gene therapy and genome editing for primary immunodeficiency diseases

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Genes & Diseases 2020年第1期7卷 38-51页

作者： Zhi-Yong Zhang Adrian J.Thrasher Fang Zhang Molecular and Cellular Immunology Great Ormond Street Institute of Child HealthUniversity Colleage LondonUK Department of Immunology and Rheumatology Children’s Hospital of Chongqing Medical UniversityChina

In past two decades the gene therapy using genetic modified autologous hematopoietic stem cells(HSCs)transduced with the viral vector has become a promising alternative option for treating primary immunodeficiency diseases(PIDs).Despite of some pitfalls at early stage clinical trials,the field of gene therapy has advanced significantly in the last decade with improvements in viral vector safety,preparatory regime for manufacturing high quality virus,automated CD34 cell purification.Hence,the overall outcome from the clinical trials for the different PIDs has been very encouraging.In addition to the viral vector based gene therapy,the recent fast moving forward developments in genome editing using engineered nucleases in HSCs has provided a new promising platform for the treatment of PIDs.This review provides an overall outcome and progress in gene therapy clinical trials for SCID-X,ADA-SCID,WAS,X-CGD,and the recent developments in genome editing technology applied in HSCs for developing potential therapy,particular in the key studies for PIDs.

关键词： Adenosine deaminase deficient Chronic granulomatous disease Gene therapy Genome editing Hematopoietic progenitor stem cells Primary immunodeficiency diseases Wiskott-Aldrich syndrome X-liked severe combined immunodeficiency

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减低毒性预处理方案在造血干细胞移植治疗WAS中的疗效及安全性评估

减低毒性预处理方案在造血干细胞移植治疗WAS中的疗效及安全性评估

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作者：罗明静重庆医科大学

学位级别：硕士

目的:评估白消安联合氟达拉滨预处理方案在湿疹-血小板减少伴免疫缺陷综合征(Wiskott-Aldrich syndrome,WAS)患者行异基因造血干细胞移植治疗中的有效性及安全性。方法:在2014年1月至2017年2月重庆医科大学附属儿童医院血液肿瘤科行异... 详细信息

目的:评估白消安联合氟达拉滨预处理方案在湿疹-血小板减少伴免疫缺陷综合征(Wiskott-Aldrich syndrome,WAS)患者行异基因造血干细胞移植治疗中的有效性及安全性。方法:在2014年1月至2017年2月重庆医科大学附属儿童医院血液肿瘤科行异基因造血干细胞移植治疗WAS的患者中,纳入回输人白细胞抗原(Human leukocyte antigen,HLA)高分辨配型全相合或1个位点不合的无关供者外周血移植患者,实验组采用由氟达拉滨、白消安、兔抗人胸腺免疫球蛋白组成(FluBuATG)的减低毒性清髓性预处理方案(7例),对照组采用传统白消安和环磷酰胺(BuCy)的清髓方案(7例)。比较两组患者移植后植入、移植后并发症和预处理不良反应发生情况。结果:Bu Cy组与FluBuATG组总体生存率分别为85.7%,71.4%(P=0.205)。BuCy组与FluBu ATG组移植后中性粒细胞植入时间、血小板植入时间、嵌合度、总体生存率方面无统计学差异。BuCy组患者III°V°急性移植物抗宿主病和慢性移植物抗宿主反应发生率与Flu BuATG组均无统计学差异。Flu BuATG组发生移植后AIHA 1例(14.3%),而BuCy组5例(71.4%)(P=0.103)。移植后EBV感染发生率高,FluBuATG组和BuCy组EBV感染率分别为85.7%、57.1%(P=0.559)。两组不同预处理方案对各脏器的不良反应中肝功能损害发生率最高,Bu Cy组肝功能损害严重程度重于FluBuATG组,但无统计学差异。结论:含FluBuATG的减低毒性预处理方案能使异基因造血干细胞植入稳定,不良反应少,具有较好耐受性。

关键词：减低毒性预处理造血干细胞移植 Wiskott-Aldrich syndrome

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