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https://www.readbyqxmd.com/read/28790360/comprehensive-validation-of-t-and-b-cell-deficiency-in-rag1-null-zebrafish-implication-for-the-robust-innate-defense-mechanisms-of-teleosts
#1
Yumie Tokunaga, Masamichi Shirouzu, Ryota Sugahara, Yasutoshi Yoshiura, Ikunari Kiryu, Mitsuru Ototake, Takahiro Nagasawa, Tomonori Somamoto, Miki Nakao
rag1 (-/-) zebrafish have been employed in immunological research as a useful immunodeficient vertebrate model, but with only fragmentary evidence for the lack of functional adaptive immunity. rag1-null zebrafish exhibit differences from their human and murine counterparts in that they can be maintained without any specific pathogen-free conditions. To define the immunodeficient status of rag1 (-/-) zebrafish, we obtained further functional evidence on T- and B-cell deficiency in the fish at the protein, cellular, and organism levels...
August 8, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28783691/characterization-of-t-and-b-cell-repertoire-diversity-in-patients-with-rag-deficiency
#2
Yu Nee Lee, Francesco Frugoni, Kerry Dobbs, Irit Tirosh, Likun Du, Francesca A Ververs, Heng Ru, Lisa Ott de Bruin, Mehdi Adeli, Jacob H Bleesing, David Buchbinder, Manish J Butte, Caterina Cancrini, Karin Chen, Sharon Choo, Reem A Elfeky, Andrea Finocchi, Ramsay L Fuleihan, Andrew R Gennery, Dalia H El-Ghoneimy, Lauren A Henderson, Waleed Al-Herz, Elham Hossny, Robert P Nelson, Sung-Yun Pai, Niraj C Patel, Shereen M Reda, Pere Soler-Palacin, Raz Somech, Paolo Palma, Hao Wu, Silvia Giliani, Jolan E Walter, Luigi D Notarangelo
Recombination-activating genes 1 and 2 (RAG1 and RAG2) play a critical role in T and B cell development by initiating the recombination process that controls the expression of T cell receptor (TCR) and immunoglobulin genes. Mutations in the RAG1 and RAG2 genes in humans cause a broad spectrum of phenotypes, including severe combined immunodeficiency (SCID) with lack of T and B cells, Omenn syndrome, leaky SCID, and combined immunodeficiency with granulomas or autoimmunity (CID-G/AI). Using next-generation sequencing, we analyzed the TCR and B cell receptor (BCR) repertoire in 12 patients with RAG mutations presenting with Omenn syndrome (n = 5), leaky SCID (n = 3), or CID-G/AI (n = 4)...
December 16, 2016: Science Immunology
https://www.readbyqxmd.com/read/28783681/the-orphan-nuclear-receptor-ror%C3%AE-and-group-3-innate-lymphoid-cells-drive-fibrosis-in-a-mouse-model-of-crohn-s-disease
#3
Bernard C Lo, Matthew J Gold, Michael R Hughes, Frann Antignano, Yanet Valdez, Colby Zaph, Kenneth W Harder, Kelly M McNagny
Fibrosis is the result of dysregulated tissue regeneration and is characterized by excessive accumulation of matrix proteins that become detrimental to tissue function. In Crohn's disease, this manifests itself as recurrent gastrointestinal strictures for which there is no effective therapy beyond surgical intervention. Using a model of infection-induced chronic gut inflammation, we show that Rora-deficient mice are protected from fibrosis; infected intestinal tissues display diminished pathology, attenuated collagen deposition, and reduced fibroblast accumulation...
September 2, 2016: Science Immunology
https://www.readbyqxmd.com/read/28783045/macrophage-derived-il-10-mediates-mucosal-repair-by-epithelial-wisp-1-signaling
#4
Miguel Quiros, Hikaru Nishio, Philipp A Neumann, Dorothee Siuda, Jennifer C Brazil, Veronica Azcutia, Roland Hilgarth, Monique N O'Leary, Vicky Garcia-Hernandez, Giovanna Leoni, Mingli Feng, Gabriela Bernal, Holly Williams, Priya H Dedhia, Christian Gerner-Smidt, Jason Spence, Charles A Parkos, Timothy L Denning, Asma Nusrat
In response to injury, epithelial cells migrate and proliferate to cover denuded mucosal surfaces and repair the barrier defect. This process is orchestrated by dynamic crosstalk between immune cells and the epithelium; however, the mechanisms involved remain incompletely understood. Here, we report that IL-10 was rapidly induced following intestinal mucosal injury and was required for optimal intestinal mucosal wound closure. Conditional deletion of IL-10 specifically in CD11c-expressing cells in vivo implicated macrophages as a critical innate immune contributor to IL-10-induced wound closure...
August 7, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28776702/species-richness-in-the-african-pike-genus-hepsetus-a-perfect-match-between-genetics-and-morphology
#5
E Decru, E Vreven, K De Gelas, E Verheyen, J Snoeks
In this study, morphological differences were found that corroborate earlier results that showed the existence of six species within the previously monospecific African pike genus Hepsetus. Additional genetic data (coI, mtDNA and rag1, nDNA) confirm the morphology-based species delineations. Deep genetic divergences imply a relatively old age for diversification within the genus. An identification key for the six species is provided in the present study.
August 2017: Journal of Fish Biology
https://www.readbyqxmd.com/read/28775298/sexual-and-vertical-transmission-of-zika-virus-in-anti-interferon-receptor-treated-rag1-deficient-mice
#6
Clayton W Winkler, Tyson A Woods, Rebecca Rosenke, Dana P Scott, Sonja M Best, Karin E Peterson
Although Zika virus (ZIKV) is primarily transmitted to humans by the Aedes aegypti mosquito, human-to-human transmission has also been observed from males-to-females as well as mother-to-offspring. In the current study, we studied both sexual transmission (STx) and vertical transmission (VTx) of ZIKV using anti-IFNAR1-treatment of Rag1 (-/-) (AIR) mice. These mice have suppressed type I IFN responses and lack adaptive immune responses, leading to a prolonged infection prior to clinical disease. STx of ZIKV from infected AIR males to naive Ifnar1 (-/-) females was observed with greater than 50% incidence, with infection observed in the vaginal tract at early time points...
August 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28769923/natural-killer-cells-from-patients-with-recombinase-activating-gene-and-non-homologous-end-joining-gene-defects-comprise-a-higher-frequency-of-cd56-bright-nkg2a-cells-and-yet-display-increased-degranulation-and-higher-perforin-content
#7
Kerry Dobbs, Giovanna Tabellini, Enrica Calzoni, Ornella Patrizi, Paula Martinez, Silvia Clara Giliani, Daniele Moratto, Waleed Al-Herz, Caterina Cancrini, Morton Cowan, Jacob Bleesing, Claire Booth, David Buchbinder, Siobhan O Burns, Talal A Chatila, Janet Chou, Vanessa Daza-Cajigal, Lisa M Ott de Bruin, MaiteTeresa de la Morena, Gigliola Di Matteo, Andrea Finocchi, Raif Geha, Rakesh K Goyal, Anthony Hayward, Steven Holland, Chiung-Hui Huang, Maria G Kanariou, Alejandra King, Blanka Kaplan, Anastasiya Kleva, Taco W Kuijpers, Bee Wah Lee, Vassilios Lougaris, Michel Massaad, Isabelle Meyts, Megan Morsheimer, Benedicte Neven, Sung-Yun Pai, Alessandro Plebani, Susan Prockop, Ismail Reisli, Jian Yi Soh, Raz Somech, Troy R Torgerson, Yae-Jaen Kim, Jolan E Walter, Andrew R Gennery, Sevgi Keles, John P Manis, Emanuela Marcenaro, Alessandro Moretta, Silvia Parolini, Luigi D Notarangelo
Mutations of the recombinase-activating genes 1 and 2 (RAG1 and RAG2) in humans are associated with a broad range of phenotypes. For patients with severe clinical presentation, hematopoietic stem cell transplantation (HSCT) represents the only curative treatment; however, high rates of graft failure and incomplete immune reconstitution have been observed, especially after unconditioned haploidentical transplantation. Studies in mice have shown that Rag(-/-) natural killer (NK) cells have a mature phenotype, reduced fitness, and increased cytotoxicity...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28759039/elevated-tumor-ldlr-expression-accelerates-ldl-cholesterol-mediated-breast-cancer-growth-in-mouse-models-of-hyperlipidemia
#8
E J Gallagher, Z Zelenko, B A Neel, I M Antoniou, L Rajan, N Kase, D LeRoith
Obesity is associated with an increase in cancer-specific mortality in women with breast cancer. Elevated cholesterol, particularly low-density lipoprotein cholesterol (LDL-C), is frequently seen in obese women. Here, we aimed to determine the importance of elevated circulating LDL, and LDL receptor (LDLR) expression in tumor cells, on the growth of breast cancer using mouse models of hyperlipidemia. We describe two novel immunodeficient mouse models of hyperlipidemia (Rag1(-/)(-)/LDLR(-/)(-) and Rag1(-/)(-)/ApoE (apolipoprotein E)(-/)(-) mice) in addition to established immunocompetent LDLR(-/)(-) and ApoE(-/)(-) mice...
July 31, 2017: Oncogene
https://www.readbyqxmd.com/read/28756234/anti-tumor-necrosis-factor-with-a-glyco-engineered-fc-region-has-increased-efficacy-in-mice-with-colitis
#9
Felicia M Bloemendaal, Alon D Levin, Manon E Wildenberg, Pim J Koelink, Bradford L McRae, Jochen Salfeld, Jenifer Lum, Marijn van der Neut Kolfschoten, Jill W Claassens, Remco Visser, Arthur Bentlage, Geert R A M D'Haens, J Sjef Verbeek, Gestur Vidarsson, Gijs R van den Brink
BACKGROUND & AIMS: Although tumor necrosis factor (TNF) antagonists reduce many clinical features of inflammatory bowel disease (IBD), complete mucosal healing occurs in less than 50% of patients. The Fc region of monoclonal antibodies against TNF has immunosuppressive properties via effects on macrophage polarization. We examined the interaction between the anti-TNF Fc region and Fcγ receptors (FcγR), and whether the absence of the Fc core fucose (which increases binding to FcγRIIIa) increases the efficacy of anti-TNF in mice with colitis...
July 26, 2017: Gastroenterology
https://www.readbyqxmd.com/read/28747913/family-history-of-early-infant-death-correlates-with-earlier-age-at-diagnosis-but-not-shorter-time-to-diagnosis-for-severe-combined-immunodeficiency
#10
Anderson Dik Wai Luk, Pamela P Lee, Huawei Mao, Koon-Wing Chan, Xiang Yuan Chen, Tong-Xin Chen, Jian Xin He, Nadia Kechout, Deepti Suri, Yin Bo Tao, Yong Bin Xu, Li Ping Jiang, Woei Kang Liew, Orathai Jirapongsananuruk, Tassalapa Daengsuwan, Anju Gupta, Surjit Singh, Amit Rawat, Amir Hamzah Abdul Latiff, Anselm Chi Wai Lee, Lynette P Shek, Thi Van Anh Nguyen, Tek Jee Chin, Yin Hsiu Chien, Zarina Abdul Latiff, Thi Minh Huong Le, Nguyen Ngoc Quynh Le, Bee Wah Lee, Qiang Li, Dinesh Raj, Mohamed-Ridha Barbouche, Meow-Keong Thong, Maria Carmen D Ang, Xiao Chuan Wang, Chen Guang Xu, Hai Guo Yu, Hsin-Hui Yu, Tsz Leung Lee, Felix Yat Sun Yau, Wilfred Hing-Sang Wong, Wenwei Tu, Wangling Yang, Patrick Chun Yin Chong, Marco Hok Kung Ho, Yu Lung Lau
BACKGROUND: Severe combined immunodeficiency (SCID) is fatal unless treated with hematopoietic stem cell transplant. Delay in diagnosis is common without newborn screening. Family history of infant death due to infection or known SCID (FH) has been associated with earlier diagnosis. OBJECTIVE: The aim of this study was to identify the clinical features that affect age at diagnosis (AD) and time to the diagnosis of SCID. METHODS: From 2005 to 2016, 147 SCID patients were referred to the Asian Primary Immunodeficiency Network...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28747605/omenn-syndrome-and-dna-recombination-defects
#11
Akihiro Yachie
  Mutations in the RAG1/RAG2 genes are associated with a broad spectrum of clinical phenotypes, ranging from severe combined immunodeficiency to various autoimmune diseases. The diversity of the clinical symptoms is determined not only by the residual RAG recombinase enzyme activity as determined by the mutations, but also by multiple environmental factors and, in rare cases, by second site mutations within the RAG1/RAG2 genes. The residual recombinase activity is responsible for the oligoclonal expansion of autoreactive T cells...
2017: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28747508/mutations-in-the-e2-glycoprotein-and-the-3-untranslated-region-enhance-chikungunya-virus-virulence-in-mice
#12
David W Hawman, Kathryn S Carpentier, Julie M Fox, Nicholas A May, Wes Sanders, Stephanie A Montgomery, Nathaniel J Moorman, Michael S Diamond, Thomas E Morrison
Chikungunya virus (CHIKV) is a mosquito-transmitted alphavirus that causes debilitating musculoskeletal pain and inflammation and can persist for months to years after acute infection. Although studies in humans and experimentally-infected animals suggest that CHIKV infection persists in musculoskeletal tissues, the mechanisms for this remain poorly understood. To evaluate this further, we isolated CHIKV from the serum of persistently infected Rag1(-/-) mice at day 28. When inoculated into naïve WT mice, this persistently circulating CHIKV strain displayed a capacity for earlier dissemination and greater pathogenicity compared with the parental virus...
July 26, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28747346/modulation-of-roquin-function-in-myeloid-cells-reduces-mycobacterium-tuberculosis-induced-inflammation
#13
Gayathri Nagalingam, Carola G Vinuesa, Warwick J Britton, Bernadette M Saunders
Damaging inflammation is a hallmark of Mycobacterium tuberculosis infection, and understanding how this is regulated is important for the development of new therapies to limit excessive inflammation. The E3 ubiquitin ligase, Roquin, is involved in immune regulation; however, its role in immunity to M. tuberculosis is unknown. To address this, we infected mice with a point mutation in Roquin1/Rc3h1 (sanroque). Aerosol-infected sanroque mice showed enhanced control of M. tuberculosis infection associated with delayed bacterial dissemination and upregulated TNF production in the lungs after 2 wk...
July 26, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28739882/zebrafish-b-cell-development-without-a-pre-b-cell-stage-revealed-by-cd79-fluorescence-reporter-transgenes
#14
Xingjun Liu, Yue-Sheng Li, Susan A Shinton, Jennifer Rhodes, Lingjuan Tang, Hui Feng, Cicely A Jette, A Thomas Look, Kyoko Hayakawa, Richard R Hardy
CD79a and CD79b proteins associate with Ig receptors as integral signaling components of the B cell Ag receptor complex. To study B cell development in zebrafish, we isolated orthologs of these genes and performed in situ hybridization, finding that their expression colocalized with IgH-μ in the kidney, which is the site of B cell development. CD79 transgenic lines were made by linking the promoter and upstream regulatory segments of CD79a and CD79b to enhanced GFP to identify B cells, as demonstrated by PCR analysis of IgH-μ expression in sorted cells...
July 24, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28710254/the-plasticity-and-stability-of-regulatory-t-cells-during-viral-induced-inflammatory-lesions
#15
Siddheshvar Bhela, Siva Karthik Varanasi, Ujjaldeep Jaggi, Sarah S Sloan, Naveen K Rajasagi, Barry T Rouse
Ocular infection with HSV causes a chronic T cell-mediated inflammatory lesion in the cornea. Lesion severity is affected by the balance of different CD4 T cell subsets, with greater severity occurring when the activity of regulatory T cells (Tregs) is compromised. In this study, fate-mapping mice were used to assess the stability of Treg function in ocular lesions. We show that cells that were once Foxp3(+) functional Tregs may lose Foxp3 and become Th1 cells that could contribute to lesion expression. The instability primarily occurred with IL-2R(lo) Tregs and was shown, in part, to be the consequence of exposure to IL-12...
August 15, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28710252/an-unbiased-linkage-approach-reveals-that-the-p53-pathway-is-coupled-to-nk-cell-maturation
#16
Roxanne Collin, Charles St-Pierre, Lorie Guilbault, Victor Mullins-Dansereau, Antonia Policheni, Fanny Guimont-Desrochers, Adam-Nicolas Pelletier, Daniel H Gray, Elliot Drobetsky, Claude Perreault, Erin E Hillhouse, Sylvie Lesage
Natural killer cells constitute potent innate lymphoid cells that play a major role in both tumor immunosurveillance and viral clearance via their effector functions. A four-stage model of NK cell functional maturation has been established according to the expression of CD11b and CD27, separating mature NK (mNK) cells into distinct populations that exhibit specific phenotypic and functional properties. To identify genetic factors involved in the regulation of NK cell functional maturation, we performed a linkage analysis on F2 (B6...
July 14, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28706113/pd-l1-programmed-death-ligand-1-protects-against-experimental-intracerebral-hemorrhage-induced-brain-injury
#17
Ranran Han, Jiaying Luo, Yanchao Shi, Yang Yao, Junwei Hao
BACKGROUND AND PURPOSE: Intracerebral hemorrhage (ICH) is a neurologically destructive stroke, for which no valid treatment is available. This preclinical study examined the therapeutic effect of PD-L1 (programmed death ligand 1), a B7 family member and a ligand for both PD-1 (programmed death 1) and B7-1 (CD80), in a murine ICH model. METHODS: ICH was induced by injecting autologous blood into 252 male C57BL/6 and Rag1(-/-) mice. One hour later, ICH mice were randomly assigned to receive an intraperitoneal injection of vehicle, PD-L1, or anti-PD-L1 antibody...
July 13, 2017: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/28686482/outer-membrane-vesicles-blebbing-contributes-to-b-vulgatus-mpk-mediated-immune-response-silencing
#18
Jan Kevin Maerz, Alex Steimle, Anna Lange, Annika Bender, Birgit Fehrenbacher, Julia-Stefanie Frick
The Gram negative intestinal symbiont Bacteroides vulgatus mpk is able to prevent from induction of colonic inflammation in Rag1(-/-) mice and promotes immune balance in Il2(-/-) mice. These inflammation-silencing effects are associated with B. vulgatus mpk-mediated induction of semi-mature dendritic cells, especially in the colonic lamina propria (cLP). However the beneficial interaction of bacteria with host immune cells is limited due to the existence of a large mucus layer covering the intestinal epithelium...
July 7, 2017: Gut Microbes
https://www.readbyqxmd.com/read/28673996/host-mediated-impairment-of-parasite-maturation-during-blood-stage-plasmodium-infection
#19
David S Khoury, Deborah Cromer, Jasmin Akter, Ismail Sebina, Trish Elliott, Bryce S Thomas, Megan S F Soon, Kylie R James, Shannon E Best, Ashraful Haque, Miles P Davenport
Severe malaria and associated high parasite burdens occur more frequently in humans lacking robust adaptive immunity to Plasmodium falciparum Nevertheless, the host may partly control blood-stage parasite numbers while adaptive immunity is gradually established. Parasite control has typically been attributed to enhanced removal of parasites by the host, although in vivo quantification of this phenomenon remains challenging. We used a unique in vivo approach to determine the fate of a single cohort of semisynchronous, Plasmodium berghei ANKA- or Plasmodium yoelii 17XNL-parasitized red blood cells (pRBCs) after transfusion into naive or acutely infected mice...
July 18, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28670633/the-orphan-nuclear-receptor-ror-alpha-and-group-3-innate-lymphoid-cells-drive-fibrosis-in-a-mouse-model-of-crohn-s-disease
#20
Bernard C Lo, Matthew J Gold, Michael R Hughes, Frann Antignano, Yanet Valdez, Colby Zaph, Kenneth W Harder, Kelly M McNagny
Fibrosis is the result of dysregulated tissue regeneration and is characterized by excessive accumulation of matrix proteins that become detrimental to tissue function. In Crohn's disease, this manifests itself as recurrent gastrointestinal strictures for which there is no effective therapy beyond surgical intervention. Using a model of infection-induced chronic gut inflammation, we show that Rora-deficient mice are protected from fibrosis; infected intestinal tissues display diminished pathology, attenuated collagen deposition and reduced fibroblast accumulation...
September 2, 2016: Science Immunology
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