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https://www.readbyqxmd.com/read/28329174/ccr7-deficiency-modulates-t-cell-response-and-increases-susceptibility-to-yersinia-pseudotuberculosis-infection
#1
Joern Pezoldt, Fabio Pisano, Wiebke Heine, Maria Pasztoi, Maik Rosenheinrich, Aaron M Nuss, Marina C Pils, Immo Prinz, Reinhold Förster, Jochen Huehn, Petra Dersch
Background: To successfully limit pathogen dissemination an immunological link between the entry tissue of the pathogen and the underlying secondary lymphoid organs (SLO) needs to be established to prime adaptive immune responses. Here, the prerequisite of CCR7 to mount host immune responses within SLOs during gastrointestinal Yersinia pseudotuberculosis infection to limit pathogen spread was investigated. Methods: Survival, bacterial dissemination, intestinal and systemic pathology of wild type (WT) and CCR7-/- mice were assessed and correlated to the presence of immune cell subsets and cytokine responses throughout the course of infection...
January 27, 2017: Journal of Infectious Diseases
https://www.readbyqxmd.com/read/28258022/probes-for-protein-adduction-in-cholesterol-biosynthesis-disorders-alkynyl-lanosterol-as-a-viable-sterol-precursor
#2
Keri A Tallman, Hye-Young H Kim, Zeljka Korade, Thiago C Genaro-Mattos, Phillip A Wages, Wei Liu, Ned A Porter
The formation of lipid electrophile-protein adducts is associated with many disorders that involve perturbations of cellular redox status. The identities of adducted proteins and the effects of adduction on protein function are mostly unknown and an increased understanding of these factors may help to define the pathogenesis of various human disorders involving oxidative stress. 7-Dehydrocholesterol (7-DHC), the immediate biosynthetic precursor to cholesterol, is highly oxidizable and gives electrophilic oxysterols that adduct proteins readily, a sequence of events proposed to occur in Smith-Lemli-Opitz syndrome (SLOS), a human disorder resulting from an error in cholesterol biosynthesis...
February 24, 2017: Redox Biology
https://www.readbyqxmd.com/read/28174017/propagation-rate-constants-for-the-peroxidation-of-sterols-on-the-biosynthetic-pathway-to-cholesterol
#3
Connor R Lamberson, Hubert Muchalski, Kari B McDuffee, Keri A Tallman, Libin Xu, Ned A Porter
The free radical chain autoxidation of cholesterol and the oxidation products formed, i.e. oxysterols, have been the focus of intensive study for decades. The peroxidation of sterol precursors to cholesterol such as 7-dehydrocholesterol (7-DHC) and desmosterol as well as their oxysterols has received less attention. The peroxidation of these sterol precursors can become important under circumstances in which genetic conditions or exposures to small molecules leads to an increase of these biosynthetic intermediates in tissues and fluids...
February 5, 2017: Chemistry and Physics of Lipids
https://www.readbyqxmd.com/read/28166604/smith-lemli-opitz-syndrome-carrier-frequency-and-estimates-of-in-utero-mortality-rates
#4
Gabriel A Lazarin, Imran S Haque, Eric A Evans, James D Goldberg
OBJECTIVE: To tabulate individual allele frequencies and total carrier frequency for Smith-Lemli-Opitz syndrome (SLOS) and compare expected versus observed birth incidences. METHODS: 262,399 individuals with no known indication or increased probability of SLOS carrier status, primarily US-based, were screened for SLOS mutations as part of an expanded carrier screening panel. Results were retrospectively analyzed to estimate carrier frequencies in multiple ethnic groups...
February 6, 2017: Prenatal Diagnosis
https://www.readbyqxmd.com/read/28116088/radiotherapy-and-mva-muc1-il-2-vaccine-act-synergistically-for-inducing-specific-immunity-to-muc-1-tumor-antigen
#5
Gilda G Hillman, Lyndsey A Reich, Shoshana E Rothstein, Lisa M Abernathy, Matthew D Fountain, Kali Hankerd, Christopher K Yunker, Joseph T Rakowski, Eric Quemeneur, Philippe Slos
BACKGROUND: We previously demonstrated that tumor irradiation potentiates cancer vaccines using genetic modification of tumor cells in murine tumor models. To investigate whether tumor irradiation augments the immune response to MUC1 tumor antigen, we have tested the efficacy of tumor irradiation combined with an MVA-MUC1-IL2 cancer vaccine (Transgene TG4010) for murine renal adenocarcinoma (Renca) cells transfected with MUC1. METHODS: Established subcutaneous Renca-MUC1 tumors were treated with 8 Gy radiation on day 11 and peritumoral injections of MVA-MUC1-IL2 vector on day 12 and 17, or using a reverse sequence of vaccine followed by radiation...
2017: Journal for Immunotherapy of Cancer
https://www.readbyqxmd.com/read/28077266/asp0028-in-combination-with-suboptimal-dose-of-tacrolimus-in-cynomolgus-monkey-renal-transplantation-model
#6
Hao Dun, Lijun Song, Anlun Ma, Yanxin Hu, Lin Zeng, Jieying Bai, Guangzhou Zhang, Liangyan Zhang, Kumi Koide, Yohei Okada, Kaori Hanaoka, Rie Yamamoto, Jun Hirose, Tatsuaki Morokata, Pierre Daloze, Huifang Chen
FTY720, a S1P-receptor modulator, has shown to be effective in several transplant and autoimmune disease models, via modulating lymphocyte homing into secondary lymphoid organs (SLOs), and thereby reducing these cells in peripheral blood. ASP0028, a newly developed S1P1/S1P5-selective agonist, presented comparable efficacy to FTY720 and wider safety margins than FTY720. In this study, we assessed the efficacy and safety of ASP0028 co-administered with suboptimal-dose of tacrolimus in the Cynomolgus monkey renal transplantation model...
January 7, 2017: Transplant Immunology
https://www.readbyqxmd.com/read/28035134/upregulation-of-cc-chemokine-receptor-7-ccr7-enables-migration-of-xenogeneic-human-adipose-derived-mesenchymal-stem-cells-to-rat-secondary-lymphoid-organs
#7
Tian Ma, Shao-Liang Luan, Hong Huang, Xing-Kun Sun, Yan-Mei Yang, Hui Zhang, Wei-Dong Han, Hong Li, Yan Han
BACKGROUND CC chemokine receptor 7 (CCR7) expression is vital for cell migration to secondary lymphoid organs (SLOs). Our previous work showed that inducing CCR7 expression enabled syngeneic mesenchymal stem cells (MSCs) to migrate into SLOs, resulting in enhanced immunosuppressive performance in mice. Given that human adipose-derived stem cells (hASCs) are widely used in clinical therapy, we further investigated whether upregulation of CCR7 enables xenogeneic hASCs to migrate to rat SLOs. MATERIAL AND METHODS hASCs rarely express CCR7; therefore, hASCs were transfected with lentivirus encoding rat CCR7 (rCCR7) plus green fluorescence protein (GFP) or GFP alone...
December 30, 2016: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/27998976/sialylation-controls-prion-fate-in-vivo
#8
Saurabh Srivastava, Elizaveta Katorcha, Martin L Daus, Peter Lasch, Michael Beekes, Ilia V Baskakov
Prions or PrP(Sc) are proteinaceous infectious agents that consist of misfolded, self-replicating states of a sialoglycoprotein called the prion protein or PrP(C) The current work tests a new hypothesis that sialylation determines the fate of prions in an organism. To begin, we produced control PrP(Sc) from PrP(C) using protein misfolding cyclic amplification with beads (PMCAb), and also generated PrP(Sc) with reduced sialylation levels using the same method but with partially desialylated PrP(C) as a substrate (dsPMCAb)...
February 10, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/27900819/learning-outcomes-exploring-implications-of-adopting-a-different-level-of-detail
#9
H L Bateman, G I McCracken, J M Thomason, J S Ellis
INTRODUCTION: Outcome-based programmes provide a framework to support educators and learners in understanding content and end points within taught courses. Management of these outcomes in the Dental Degree at Newcastle University has been a challenge in relation to quality assurance and enhancement, having over 1500 detailed student-level outcomes (SLO). This research aimed to explore the implications of adopting a more superficial "course" level of outcome (CLO), when reviewed against a reference set of external LO requirements...
November 29, 2016: European Journal of Dental Education: Official Journal of the Association for Dental Education in Europe
https://www.readbyqxmd.com/read/27881983/high-endothelial-venules-and-lymphatic-vessels-in-tertiary-lymphoid-organs-characteristics-functions-and-regulation
#10
REVIEW
Nancy H Ruddle
High endothelial venules (HEVs) and lymphatic vessels (LVs) are essential for the function of the immune system, by providing communication between the body and lymph nodes (LNs), specialized sites of antigen presentation and recognition. HEVs bring in naïve and central memory cells and LVs transport antigen, antigen-presenting cells, and lymphocytes in and out of LNs. Tertiary lymphoid organs (TLOs) are accumulations of lymphoid and stromal cells that arise and organize at ectopic sites in response to chronic inflammation in autoimmunity, microbial infection, graft rejection, and cancer...
2016: Frontiers in Immunology
https://www.readbyqxmd.com/read/27877173/stromal-fibroblasts-in-tertiary-lymphoid-structures-a-novel-target-in-chronic-inflammation
#11
REVIEW
Francesca Barone, David H Gardner, Saba Nayar, Nathalie Steinthal, Christopher D Buckley, Sanjiv A Luther
Tertiary lymphoid structures (TLS) are organized aggregates of lymphocytes, myeloid, and stromal cells that provide ectopic hubs for acquired immune responses. TLS share phenotypical and functional features with secondary lymphoid organs (SLO); however, they require persistent inflammatory signals to arise and are often observed at target sites of autoimmune disease, chronic infection, cancer, and organ transplantation. Over the past 10 years, important progress has been made in our understanding of the role of stromal fibroblasts in SLO development, organization, and function...
2016: Frontiers in Immunology
https://www.readbyqxmd.com/read/27830060/clonal-selection-versus-clonal-cooperation-the-integrated-perception-of-immune-objects
#12
Serge Nataf
Analogies between the immune and nervous systems were first envisioned by the immunologist Niels Jerne who introduced the concepts of antigen "recognition" and immune "memory". However, since then, it appears that only the cognitive immunology paradigm proposed by Irun Cohen, attempted to further theorize the immune system functions through the prism of neurosciences. The present paper is aimed at revisiting this analogy-based reasoning. In particular, a parallel is drawn between the brain pathways of visual perception and the processes allowing the global perception of an "immune object"...
2016: F1000Research
https://www.readbyqxmd.com/read/27604178/collagenvi-cre-mice-a-new-tool-to-target-stromal-cells-in-secondary-lymphoid-organs
#13
Alejandro Prados, George Kollias, Vasiliki Koliaraki
Stromal cells in secondary lymphoid organs (SLOs) are non-hematopoietic cells involved in the regulation of adaptive immune responses. Three major stromal populations have been identified in adult SLOs: fibroblastic reticular cells (FRCs), follicular dendritic cells (FDCs) and marginal reticular cells (MRCs). The properties of these individual populations are not clearly defined, mainly due to the lack of appropriate genetic tools, especially for MRCs. Here, we analyzed stromal cell targeting in SLOs from a transgenic mouse strain that expresses Cre recombinase under the CollagenVI promoter, using lineage tracing approaches...
2016: Scientific Reports
https://www.readbyqxmd.com/read/27526097/intracranial-undifferentiated-malign-neuroglial-tumor-in-smith-lemli-opitz-syndrome-a-theory-of-a-possible-predisposing-factor-for-primary-brain-tumors-via-a-case-report
#14
Ayfer Aslan, Alp Ozgun Borcek, Selma Pamukcuoglu, M Kemali Baykaner
BACKGROUND: Smith-Lemli-Opitz Syndrome (SLOS) is a rare hereditary autosomal recessive disorder with broken cholesterol synthesis causing by 7-dehydrocholesterol reductase deficiency. Although the clinical features and pathogenesis is well-defined, it is unknown whether there is a relationship between SLOS and neoplastic processes, especially brain neoplasms. PURPOSE: We aimed to attract the attentions to any possibility of relation between SLOS and intracranial tumor development via a pediatric case with both intracranial high-grade neuroglial tumor and SLOS, and thus to contribute an additional data to the literature on togetherness of these two clinical conditions...
January 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27513191/a-placebo-controlled-trial-of-simvastatin-therapy-in-smith-lemli-opitz-syndrome
#15
Christopher A Wassif, Lisa Kratz, Susan E Sparks, Courtney Wheeler, Simona Bianconi, Andrea Gropman, Karim A Calis, Richard I Kelley, Elaine Tierney, Forbes D Porter
BACKGROUND: Smith-Lemli-Opitz syndrome (SLOS) is a multiple malformation/cognitive impairment syndrome characterized by the accumulation of 7-dehydrocholesterol, a precursor sterol of cholesterol. Simvastatin, a 3-hydroxy-3-methylglutaryl-coenzyme A reductase inhibitor that crosses the blood-brain barrier, has been proposed for the treatment of SLOS based on in vitro and in vivo studies suggesting that simvastatin increases the expression of hypomorphic DHCR7 alleles. METHODS: Safety and efficacy of simvastatin therapy in 23 patients with mild to typical SLOS were evaluated in a randomized, double-blind, placebo-controlled trial...
March 2017: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/27462110/peripheral-tolerance-can-be-modified-by-altering-klf2-regulated-treg-migration
#16
Sudheer K Pabbisetty, Whitney Rabacal, Emmanuel J Volanakis, Vrajesh V Parekh, Danyvid Olivares-Villagómez, Delphine Cendron, Kelli L Boyd, Luc Van Kaer, Eric Sebzda
Tregs are essential for maintaining peripheral tolerance, and thus targeting these cells may aid in the treatment of autoimmunity and cancer by enhancing or reducing suppressive functions, respectively. Before these cells can be harnessed for therapeutic purposes, it is necessary to understand how they maintain tolerance under physiologically relevant conditions. We now report that transcription factor Kruppel-like factor 2 (KLF2) controls naive Treg migration patterns via regulation of homeostatic and inflammatory homing receptors, and that in its absence KLF2-deficient Tregs are unable to migrate efficiently to secondary lymphoid organs (SLOs)...
August 9, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27446069/how-follicular-dendritic-cells-shape-the-b-cell-antigenome
#17
REVIEW
Jan Kranich, Nike Julia Krautler
Follicular dendritic cells (FDCs) are stromal cells residing in primary follicles and in germinal centers of secondary and tertiary lymphoid organs (SLOs and TLOs). There, they play a crucial role in B-cell activation and affinity maturation of antibodies. FDCs have the unique capacity to bind and retain native antigen in B-cell follicles for long periods of time. Therefore, FDCs shape the B-cell antigenome (the sum of all B-cell antigens) in SLOs and TLOs. In this review, we discuss recent findings that explain how this stromal cell type can arise in almost any tissue during TLO formation and, furthermore, focus on the mechanisms of antigen capture and retention involved in the generation of long-lasting antigen depots displayed on FDCs...
2016: Frontiers in Immunology
https://www.readbyqxmd.com/read/27401223/investigation-of-7-dehydrocholesterol-reductase-pathway-to-elucidate-off-target-prenatal-effects-of-pharmaceuticals-a-systematic-review
#18
REVIEW
M R Boland, N P Tatonetti
Mendelian diseases contain important biological information regarding developmental effects of gene mutations that can guide drug discovery and toxicity efforts. In this review, we focus on Smith-Lemli-Opitz syndrome (SLOS), a rare Mendelian disease characterized by compound heterozygous mutations in 7-dehydrocholesterol reductase (DHCR7) resulting in severe fetal deformities. We present a compilation of SLOS-inducing DHCR7 mutations and the geographic distribution of those mutations in healthy and diseased populations...
October 2016: Pharmacogenomics Journal
https://www.readbyqxmd.com/read/27334845/7-dehydrocholesterol-efficiently-supports-ret-signaling-in-a-mouse-model-of-smith-opitz-lemli-syndrome
#19
Myriam Gou-Fàbregas, Anna Macià, Carlos Anerillas, Marta Vaquero, Mariona Jové, Sanjay Jain, Joan Ribera, Mario Encinas
Smith-Lemli-Opitz syndrome (SLOS) is a rare disorder of cholesterol synthesis. Affected individuals exhibit growth failure, intellectual disability and a broad spectrum of developmental malformations. Among them, renal agenesis or hypoplasia, decreased innervation of the gut, and ptosis are consistent with impaired Ret signaling. Ret is a receptor tyrosine kinase that achieves full activity when recruited to lipid rafts. Mice mutant for Ret are born with no kidneys and enteric neurons, and display sympathetic nervous system defects causing ptosis...
2016: Scientific Reports
https://www.readbyqxmd.com/read/27315086/7dhc-induced-changes-of-kv1-3-operation-contributes-to-modified-t-cell-function-in-smith-lemli-opitz-syndrome
#20
András Balajthy, Sándor Somodi, Zoltán Pethő, Mária Péter, Zoltán Varga, Gabriella P Szabó, György Paragh, László Vígh, György Panyi, Péter Hajdu
In vitro manipulation of membrane sterol level affects the regulation of ion channels and consequently certain cellular functions; however, a comprehensive study that confirms the pathophysiological significance of these results is missing. The malfunction of 7-dehydrocholesterol (7DHC) reductase in Smith-Lemli-Opitz syndrome (SLOS) leads to the elevation of the 7-dehydrocholesterol level in the plasma membrane. T lymphocytes were isolated from SLOS patients to assess the effect of the in vivo altered membrane sterol composition on the operation of the voltage-gated Kv1...
August 2016: Pflügers Archiv: European Journal of Physiology
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