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Disrupted Ca^(2+) h homeostasis and immunodeficiency in patients with functional IP_(3) receptor subtype 3 defects

Cellular & Molecular Immunology 2023年第1期20卷 11-25页

作者： Julika Neumann Erika Van Nieuwenhove Lara E.Terry Frederik Staels Taylor R.Knebel Kirsten Welkenhuyzen Kourosh Ahmadzadeh Mariah R.Baker Margaux Gerbaux mathijs willemsen John S.Barber Irina I.Serysheva Liesbeth De Waele François Vermeulen Susan Schlenner Isabelle Meyts David IYule Geert Bultynck Rik Schrijvers Stephanie Humblet-Baron Adrian Liston VIB Center for Brain and Disease Research LeuvenBelgium Department of Microbiology and Immunology KU LeuvenLeuvenBelgium UZ Leuven LeuvenBelgium Department of Pharmacology and Physiology University of RochesterRochesterNY14526USA Laboratory of Molecular and Cellular Signaling Department of Cellular and Molecular MedicineLeuven KankerinstituutKU LeuvenLeuvenBelgium Laboratory of Immunobiology Department Microbiology and ImmunologyRega InstituteKU LeuvenLeuvenBelgium Department of Biochemistry and Molecular Biology Structural Biology Imaging CenterMcGovern Medical School at The University of Texas Health Science Center at HoustonHoustonTX77030USA Pediatric Department Academic Children Hospital Queen FabiolaUniversitéLibre de BruxellesBrusselsBelgium Department of Pediatric Neurology University Hospitals LeuvenLeuvenBelgium Department of Pulmonology University Hospitals LeuvenLeuvenBelgium Laboratory for Inborn Errors of Immunity Department of Immunology and MicrobiologyKU LeuvenLeuvenBelgium Laboratory for Allergy and Clinical Immunology and Immunogenetics Research Group Department of MicrobiologyImmunology and TransplantationKU LeuvenLeuvenBelgium Immunology Programme The Babraham InstituteBabraham Research CampusCambridgeCB223ATUK

Calcium signaling is essential for lymphocyte activation, with genetic disruptions of store-operated calcium (Ca^(2+)) entry resulting in severe immunodeficiency. The inositol 1,4,5-trisphosphate receptor (IP_(3)R), a homo- or heterotetramer of the IP_(3)R1-3 isoforms, amplifies lymphocyte signaling by releasing Ca^(2+) from endoplasmic reticulum stores following antigen stimulation. Although knockout of all IP_(3)R isoforms in mice causes immunodeficiency, the seeming redundancy of the isoforms is thought to explain the absence of variants in human immunodeficiency. In this study, we identified compound heterozygous variants of ITPR3 (a gene encoding IP_(3)R subtype 3) in two unrelated Caucasian patients presenting with immunodeficiency. To determine whether ITPR3 variants act in a nonredundant manner and disrupt human immune responses, we characterized the Ca^(2+) signaling capacity, the lymphocyte response, and the clinical phenotype of these patients. We observed disrupted Ca^(2+) signaling in patient-derived fibroblasts and immune cells, with abnormal proliferation and activation responses following T-cell receptor stimulation. Reconstitution of IP_(3)R3 in IP_(3)R knockout cell lines led to the identification of variants as functional hypomorphs that showed reduced ability to discriminate between homeostatic and induced states, validating a genotype–phenotype link. These results demonstrate a functional link between defective endoplasmic reticulum Ca^(2+) channels and immunodeficiency and identify IP_(3)Rs as diagnostic targets for patients with specific inborn errors of immunity. These results also extend the known cause of Ca^(2+)-associated immunodeficiency from store-operated entry to impaired Ca^(2+) mobilization from the endoplasmic reticulum, revealing a broad sensitivity of lymphocytes to genetic defects in Ca^(2+) signaling.

关键词： Primary immunodeficiency Calcium signalling Whole exome sequencing

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Author Correction: Disrupted Ca2+ homeostasis and immunodeficiency in patients with functional IP3 receptor subtype 3 defects

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Cellular & Molecular Immunology 2023年第1期20卷 114-114页

作者： Julika Neumann Erika Van Nieuwenhove Lara E.Terry Frederik Staels Taylor R.Knebel Kirsten Welkenhuyzen Kourosh Ahmadzadeh Mariah R.Baker Margaux Gerbaux mathijs willemsen John S.Barber Irina I.Serysheva Liesbeth De Waele François Vermeulen Susan Schlenner Isabelle Meyts David I.Yule Geert Bultynck Rik Schrijvers Stephanie Humblet-Baron Adrian Liston VIB Center for Brain and Disease Research Leuven Belgium Department of Microbiology and Immunology KU Leuven Leuven Belgium UZ Leuven Leuven Belgium Department of Pharmacology and Physiology University of Rochester Rochester NY 14526 USA Laboratory of Molecular and Cellular Signaling Department of Cellular and Molecular Medicine Leuven Kankerinstituut KU Leuven Leuven Belgium Laboratory of Immunobiology Department Microbiology and Immunology Rega Institute KU Leuven Leuven Belgium Department of Biochemistry and Molecular Biology Structural Biology Imaging Center McGovern Medical School at The University of Texas Health Science Center at Houston Houston TX 77030 USA Pediatric Department Academic Children Hospital Queen Fabiola Université Libre de Bruxelles Brussels Belgium Department of Pediatric Neurology University Hospitals Leuven Leuven Belgium Department of Pulmonology University Hospitals Leuven Leuven Belgium Laboratory for Inborn Errors of Immunity Department of Immunology and Microbiology KU Leuven Leuven Belgium Laboratory for Allergy and Clinical Immunology and Immunogenetics Research Group Department of Microbiology Immunology and Transplantation KU Leuven Leuven Belgium Immunology Programme The Babraham Institute Babraham Research Campus Cambridge CB22 3AT UK

In this article the statement in the Acknowledgements section was incorrectly given as “MRB and IIS are supported by the NIH R01GM080139 grant (to IIS), the Welch Foundation Research Grant AU-2014-20190331 (to IIS), and the American Heart Association grant 18CDA34110086 (to MRB)” and should have been, “MRB and IIS are supported by the NIH R01GM072804 grant (to IIS), the Welch Foundation Research Grant AU-2014-20190331 (to IIS), and the American Heart Association grant 18CDA34110086 (to MRB).”

关键词： IIS statement knowledge

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Recognition and Enforcement of Chinese Money Judgments in Civil and Commercial Matters in the Netherlands: Law and Practice

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中国国际私法与比较法年刊 2017年第2期20卷 125-133页

作者： mathijs H.Ten Wolde the University of Groningen The Netherlands the Ulrik Huber Institute for Private International Law

Ⅰ.Enforcement of foreign judgments? Article 431 DCCP In the absence of a treaty in w hich the mutual recognition and enforcement of judgments in civil and commercial matters is regulated,the recognition and enforceme... 详细信息

关键词： mutual governed judgment

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T(11;18)(q21;q21)-positive gastrointestinal MALT lymphomas are heterogeneous with respect to the V_H gene mutation status

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World Journal of Gastrointestinal Oncology 2011年第2期3卷 24-32页

作者： Xavier Sagaert Vera Vanhentenrijk Gert De Hertogh Karel Geboes Thomas Tousseyn Brigitte Maes mathijs Baens Eric Van Cutsem Department of Morphology and Molecular Pathology University Hospitals Leuven Department of Morphology and Molecular Pathology University Hospitals Leuven Department of Laboratory Medicine Jessa Hospital Department for Molecular and Developmental Genetics Flanders Institute for Biotechnology (VIB) Department of Human Genetics Catholic University of Leuven Department of Digestive Oncology University Hospitals Leuven

AIM: To investigate how t(11;18)(q21;q21)-positive gastrointestinal MALT lymphomas relate to other marginal zone lymphomas with respect to the somatic mutation pattern of the VH genes and the expression of the marker CD27. METHODS: The VH gene of 7 t(11;18)(q21;q21)-positive gastrointestinal MALT lymphomas was amplif iedby PCR using family specif ic VH primers and a consensus JH primer. PCR products were sequenced and mutation analysis of the CDR and the FR regions was performed. All cases were immunostained for CD27. RESULTS: One case showed unmutated VH genes while the others showed mutated VH genes with mutation frequencies ranging from 1.3 to 14.7% and with evidence of antigen selection in 2 cases. These data suggest that the translocation t(11;18)(q21;q21) can target either B-cells at different stages of differentiation or naive B-cells that retain the capacity to differentiate upon antigen stimulation. All cases but one displayed weak to strong CD27 expression which did not correlate with the VH gene mutation status. CONCLUSION: t(11;18)(q21;q21)-positive gastro- intestinal MALT lymphomas are heterogeneous with respect to the VH mutation status and CD27 is not a marker of somatically mutated B-cells.

关键词： Gastrointestinal MALT lymphoma t(11 18) (q21 q21) VH mutation Marginal zone CD27

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荷兰的工业设计

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深圳大学学报（人文社会科学版） 2010年第2期27卷 154-155页

作者：马泰斯·波特荷兰CQ工业设计事务所

设计改变都市,创意铸就品牌。创意设计是提高产品品牌价值,创造产业高附加值的重要手段;是改进人们的生活方式,提升人们生存质量的重要途径;也是塑造都市的文化形象,开发与推广城市品牌必不可少的方法。当深圳被联合国教科文组织批准加... 详细信息

设计改变都市,创意铸就品牌。创意设计是提高产品品牌价值,创造产业高附加值的重要手段;是改进人们的生活方式,提升人们生存质量的重要途径;也是塑造都市的文化形象,开发与推广城市品牌必不可少的方法。当深圳被联合国教科文组织批准加入全球创意城市网络,并获得"设计之都"称号之后,大力推动与发展创意设计不仅成为人们的共识,而且成为深圳市文化产业发展的一个重要战略。深圳获取"设计之都"的称号,使深圳的创意设计的发展迎来了新的机遇,同时也面临着一些新的亟待解决的课题:创意设计如何在中国语境与国际视域中科学发展;如何彰显深圳文化的创新品质,强化深圳先锋、时尚、创意的城市色彩;如何树立起深圳自己的"设计之都"的品牌形象,让深圳尽快走在世界设计之都的前列,这些问题值得人们关注和深入思考。为了明确深圳文化建设的定位及设计之都的发展战略,作为深圳创意十二月序曲的"2009深圳设计论坛暨设计展"于2009年12月2日在深圳大学开幕。这是继2005、2007两届深圳设计论坛暨设计展成功举办后的又一次国际性、高规格的设计界学术盛会,有国内外36所院校的近百位专家学者、设计界精英共聚一堂,以"设计之都与都市设计"为主题,以"在中国的都市化和现代进程中设计对于都市建设的重要作用及面临的紧迫问题"、"他国都市设计的启示"、"深圳设计之都建设的策略"、"设计教育与都市设计"等为参考议题,展开了深入的研讨与精彩的演讲。演讲嘉宾中既有理论功底扎实的学者,也有实践经验丰富的著名设计师,他们的演讲或体现出严谨的理性深度,或迸发出感性的思想火花,或从宏观上阐发其对设计之都与创意设计的发展构想,或从微观上述说自己的设计理念与作品蕴含……虽因演讲者的文化视野、知识结构、设计体验等存在种种差异,但在不同观点、不同文化取向的碰撞中,却展现出论坛内容的丰富性。为了使更多的人能够领略到这场设计论坛的余韵,我们从中摘选十位演讲嘉宾的主要观点,以期引发人们对这些问题的思考和讨论。

关键词：工业设计联合国教科文组织文化产业发展创意设计都市建设荷兰深圳市品牌价值

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Glutamate reduces experimental intestinal hyperpermeability and facilitates glutamine support of gut integrity

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World Journal of Gastroenterology 2011年第12期17卷 1569-1573页

作者： Mechteld AR Vermeulen Jeffrey de Jong mathijs J Vaessen Paul AM van Leeuwen Alexander PJ Houdijk Department of Surgery VU University Medical Center1007 MB AmsterdamThe Netherlands Department of Surgery Gemini Hospital1782 GZ Den HelderThe Netherlands Department of Surgery Medical Center Alkmaar1815 JD AlkmaarThe Netherlands

AIM:To assess whether glutamate plays a similar role to glutamine in preserving gut wall ***:The effects of glutamine and glutamate on induced hyperpermeability in intestinal cell lines were *** hyperpermeability was induced in *** and HT-29CL.19A cell lines by adding phorbol-12,13-dibutyrate(PDB) apically,after which the effects of glutamine and glutamate on horseradish peroxidase(HRP) diffusion were *** inhibitor of glutamate transport(L-trans-pyrrolidine-2,4-dicarboxylic acid:trans-PDC) and an irreversible blocker(acivicin) of the extracellular glutamine to glutamate converting enzyme,γ-glutamyltransferase,were ***:Apical to basolateral HRP flux increased significantly compared to controls not exposed to PDB (n=30,P<0.001).Glutamine application reduced hyperpermeability by 19%and 39%in the respective cell *** application reduced hyperpermeability by 30%and 20%,*** of HT29CL.19A cells with acivicin and subsequent PDB and glutamine addition increased permeability *** of *** cells with trans-PDC followed by PDB and glutamate addition also resulted in high permeability ***:Apical glutamate-similar to glutaminecan decrease induced paracellular *** conversion of glutamine to glutamate and subsequent uptake of glutamate could be a pivotal step in the mechanism underlying the protective effect of glutamine.

关键词： Apical Basolateral Flux Glutamate Glutamine Gut protection Gut wall integrity Intestine Permeability

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Decentralised Governance of Wetland Resources in the Lake Victoria Basin of Uganda

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Natural Resources 2013年第1期4卷 55-64页

作者： Alice Nakiyemba Were Moses Isabirye Jean Poesen Miet Maertens Jozef Deckers Erik mathijs Department of Earth and Environmental Sciences Katholieke Universiteit Leuven Leuven Belgium Department of Earth and Environmental Sciences Katholieke Universiteit Leuven Leuven Belgium Faculty of Natural Resources and Environment Busitema University Namasagali Campus Tororo Uganda Faculty of Natural Resources and Environment Busitema University Namasagali Campus Tororo Uganda

Recent studies on decentralized wetland governance in Uganda have focused mainly on the relationship between central and local governments. Less attention has been given to the relationship between agricultural systems, local governments, and water bodies. This study aims at assessing decentralized wetlands governance in the upper river Rwizi and Iguluibi micro catchments, Lake Victoria Basin Uganda, in relation to farming practices. Qualitative and quantitative methods were used to provide a comprehensive understanding of the contribution of decentralized governance to the management of wetlands resources. Quantitative survey data were analyzed using descriptive summary statistics with the help of Statistical package for Social Sciences (SPSS) soft ware. Qualitative data analysis involved the categorization of verbal and behavioral data for purposes of classification with the use of Nvivo soft ware. Data were analyzed at two levels: the descriptive level of analysis which is the account of the data in terms of what was said, documented or observed with nothing assumed about it. The second level of analysis was interpretive, where data is transformed into what is meant by the responses and conclusions are drawn. All recorded interviews were transcribed into a written report. Findings revealed that decentralized wetlands governance has brought ecosystem services nearer to communities in form of community-based management planning. However, results indicated inadequate institutional capacity, knowledge gaps of existing legal frameworks, limited political support and accountability for local leaders, lack of cooperation and coordination, and inadequate funding. In all, the policy has not fully realized its intended outcomes.

关键词： Agricultural Systems Decentralized Governance Lake Victoria Wetland Degradation Uganda

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Differentiation of malignant and benign proximal bile duct strictures: The diagnostic dilemma

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World Journal of Gastroenterology 2008年第32期14卷 5032-5038页

作者： Jaap Jacob Kloek Otto Marinus van Delden Deha Erdogan Fibo Jan ten Kate Erik Anthoni Rauws Olivier Robert Busch Dirk Joan Gouma Thomas mathijs van Gulik Department of Surgery Academic Medical Center University of Amsterdam Department of Radiology Academic Medical Center University of Amsterdam Department of Pathology Academic Medical Center University of Amsterdam Department of Gastroenterology Academic Medical Centre University of Amsterdam

AIM: To identify the criteria for the differentiation of hilar cholangiocarcinoma (HCCA) from benign strictures. METHODS: A total of 68 patients underwent resection of lesions suspicious for HCCA between 1998 and 2006. The results of laboratory investigations, imaging studies and brush cytology were collected. These fi ndings were analyzed to obtain the fi nal diagnosis. RESULTS: Histological examination of the resected specimens confirmed HCCA in 58 patients (85%, group Ⅰ) whereas 10 patients (15%, groupⅡ) were diagnosed to have benign strictures. The most common presenting symptom was obstructive jaundice in 77% patients (79% group Ⅰvs 60% group Ⅱ P = 0.23). Laboratory findings showed greater elevation of transaminase levels in group Ⅰcompared to group Ⅱ The various imaging modalities showed vascular involvement exclusively in the malignant group (36%,P < 0.05). Brush cytology was positive for malignant cells in only 50% patients in group Ⅰ whereas none in group Ⅱ showed malignant cells. CONCLUSION: Despite improvements in imaging techniques, 10 patients (15%) with a presumptive diagnosis of HCCA were ultimately found to have benign strictures. Except for vascular involvement which was associated significantly with malignancy, there were no conclusive features of malignancy on regular imaging modalities. This uncertainty should be taken into account when patients with a suspicious lesion at the liver hilum are considered for resection.

关键词： Biliary stricture Hilar cholangiocarcinoma Benign Radiological Vascular involvement cholangio

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Controlling the gelation temperature of biomimetic polyisocyanides

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Chinese Chemical Letters 2018年第2期29卷 281-284页

作者： Paul H.J. Kouwer Paula de Almeida Onno ven den Boomen Zaskia H. Eksteen-Akeroyd Roel Hammink Maarten Jaspers Stijn Kragt mathijs F.J. Mabesoone RoelandJ.M. Nolte Alan E. Rowan Martin G.T.A. Rutten Vincent A.A. Le Sage Daniel C. Schoenmakers Chengfen Xing Jialiang xu Institute for Molecules and Materials Radboud University Nijmegen 6525 A J The Netherlands

Thermosensitive polymers show an entropy-driven transition from a well-solvated to a poorly solvated polymer chain, resulting in a more compact globular conformation. The transition at the lower critical solution temperature（LCST） is often sharp, which allows for a wide range of smart material *** the LCST, oligo（ethylene glycol）-substituted polyisocyanides（PICs） form soft hydrogels, composed of polymer bundles similar to biological gels, such as actin, fibrin and intermediate filaments. Here, we show that the LCST of PICs strongly depends linearly on the length of the ethylene glycol（EG） tails; every EG group increases the LCSTand thus the gelation temperature by nearly 30 ℃. Using a copolymerisation approach, we demonstrate that we can precisely tailor the gelation temperature between 10 ℃ and 60 ℃and, consequently, tune the mechanical properties of the PIC gels.

关键词： Smart materials Lower critical solution temperature Polyisocyanides Mechanical properties Biomimetic polymers

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新研究确定了与肝病和脂肪代谢有关的肝细胞突变

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中西医结合肝病杂志 2021年第11期31卷 1011-1011页

作者： Stanley W. K. Ng Foad J. Rouhani Simon F. Brunner Natalia Brzozowska Federico Abascal Luiza Moore Lia Chappell Daniel Leongamornlert Aleksandra Ivovic Philip Robinson Timothy Butler mathijs A. Sanders Nicholas Williams Tim H. H. Coorens Jon Teague Keiran Raine Adam P. Butler Yvette Hooks Beverley Wilson Natalie Birtchnell Michael R. Stratton Iñigo Martincorena Raheleh Rahbari Peter J. Campbell Huw Naylor Sarah J. Aitken Matthew Hoare Susan E. Davies Ming Yang Efterpi Nikitopoulou Christian Frezza Cancer Genome Project Wellcome Sanger Institute Hinxton UK Department of Surgery Addenbrooke’s Hospital Cambridge UK CRUK Cambridge Institute Cambridge UK Department of Pathology Addenbrooke’s Hospital Cambridge UK MRC Toxicology Unit University of Cambridge Cambridge UK MRC Cancer Unit University of Cambridge Cambridge UK Department of Hematology Erasmus University Medical Center Rotterdam The Netherlands Department of Medicine University of Cambridge Addenbrooke’s Hospital Cambridge UK Stem Cell Institute University of Cambridge Cambridge UK

一项来自英国韦尔科姆基金会桑格研究所和剑桥大学等研究机构的研究人员的新研究分析了来自34例患者肝脏样本的1590个基因组,包括健康对照、酒精相关肝病和非酒精性脂肪肝疾病患者的肝脏样本,结果发现ACVR2A、TNRC6B、NEAT1、FOXO1和GP... 详细信息

一项来自英国韦尔科姆基金会桑格研究所和剑桥大学等研究机构的研究人员的新研究分析了来自34例患者肝脏样本的1590个基因组,包括健康对照、酒精相关肝病和非酒精性脂肪肝疾病患者的肝脏样本,结果发现ACVR2A、TNRC6B、NEAT1、FOXO1和GPAM 五个基因在肝病患者的肝细胞中发生了突变,过程如图a所示。而其中的FOXO1、CIDEB、GPAM三个基因是对肝细胞如何代谢脂肪和对胰岛素作出反应有直接影响的基因。

关键词： FOXO1 肝病患者细胞突变肝细胞脂肪代谢胰岛素 GPA 剑桥大学

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