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Systemic sclerosis animal model

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https://www.readbyqxmd.com/read/29053787/humanized-mutant-fus-drives-progressive-motor-neuron-degeneration-without-aggregation-in-fusdelta14-knockin-mice
#1
Anny Devoy, Bernadett Kalmar, Michelle Stewart, Heesoon Park, Beverley Burke, Suzanna J Noy, Yushi Redhead, Jack Humphrey, Kitty Lo, Julian Jaeger, Alan Mejia Maza, Prasanth Sivakumar, Cinzia Bertolin, Gianni Soraru, Vincent Plagnol, Linda Greensmith, Abraham Acevedo Arozena, Adrian M Isaacs, Benjamin Davies, Pietro Fratta, Elizabeth M C Fisher
Mutations in FUS are causative for amyotrophic lateral sclerosis with a dominant mode of inheritance. In trying to model FUS-amyotrophic lateral sclerosis (ALS) in mouse it is clear that FUS is dosage-sensitive and effects arise from overexpression per se in transgenic strains. Novel models are required that maintain physiological levels of FUS expression and that recapitulate the human disease-with progressive loss of motor neurons in heterozygous animals. Here, we describe a new humanized FUS-ALS mouse with a frameshift mutation, which fulfils both criteria: the FUS Delta14 mouse...
October 7, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29029628/erythropoietin-reduces-experimental-autoimmune-encephalomyelitis-severity-via-neuroprotective-mechanisms
#2
M Moransard, M Bednar, K Frei, M Gassmann, O O Ogunshola
BACKGROUND: Treatment with erythropoietin (Epo) in experimental autoimmune encephalomyelitis (EAE), the rodent model of multiple sclerosis (MS), has consistently been shown to ameliorate disease progression and improve overall outcome. The effect has been attributed to modulation of the immune response and/or preservation of the central nervous system (CNS) tissue integrity. It remains unclear, however, if (a) Epo acts primarily in the CNS or the periphery and if (b) Epo's beneficial effect in EAE is mainly due to maintaining CNS tissue integrity or to modulation of the immune response...
October 13, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/29018331/distinctive-roles-for-%C3%AE-7-and-%C3%AE-9-nicotinic-acetylcholine-receptors-in-inflammatory-and-autoimmune-responses-in-the-murine-experimental-autoimmune-encephalomyelitis-model-of-multiple-sclerosis
#3
Qiang Liu, Paul Whiteaker, Barbara J Morley, Fu-Dong Shi, Ronald J Lukas
Previous studies have demonstrated immunosuppressive and anti-inflammatory effects of nicotine, including in the experimental autoimmune encephalomyelitis (EAE) model in mice of some forms of multiple sclerosis (MS). Other studies using knock-out (KO) mice have implicated nicotinic acetylcholine (ACh) receptors containing α7, α9, or β2 subunits (α7*-, α9*- or β2*-nAChR) in different, disease-exacerbating or disease-ameliorating processes. These outcomes are in harmony with gene expression analyses showing nAChR subunit mRNA in many classes of immune system cell types...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28990564/-mirnas-new-actors-in-the-physiopathology-of-multiple-sclerosis
#4
Ferdinand Jagot, Nathalie Davoust
Multiple sclerosis (MS) is an auto-immune demyelinating disorder characterized by a chronic neuro-inflammatory process associated with an infiltration of the central nervous system (CNS) by autoreactive lymphocytes. The etiology of the disease remains unclear but the recent discovery of a dysregulated miRNA network in both cells and extracellular fluids of MS patients has brought new insights on the pathophysiological mechanisms involved in this disorder. miRNAs can induce a T cell polarization towards a pathological Th17 or Th1 phenotype and a deleterious activation of microglia, the CNS-resident macrophages...
June 2017: Médecine Sciences: M/S
https://www.readbyqxmd.com/read/28989911/the-effect-of-levothyroxine-on-serum-levels-of-interleukin-10-and-interferon-gamma-in-rat-model-of-multiple-sclerosis
#5
Cobra Payghani, Fatemeh Khani, Aryan Rafiee Zadeh, Parham Reisi, Hojjatallah Alaei, Bahman Rashidi
BACKGROUND: There is an increase in inflammatory and a reduction in anti-inflammatory cytokines in multiple sclerosis (MS). Considering the role of thyroid hormones in the development and regulation of both neural and immune systems, the aim of this study was to evaluate the effects of levothyroxine on serum concentrations of interleukin-10 (IL-10) and interferon gamma (IFN-γ) in animal models of MS. MATERIALS AND METHODS: To induce demyelination in male Wistar rats, lysolecithin was injected into the optic chiasm...
2017: Advanced Biomedical Research
https://www.readbyqxmd.com/read/28984112/effects-of-green-tea-epigallocatechin-3-gallate-on-the-proteolipid-protein-and-oligodendrocyte-transcription-factor-1-messenger-rna-gene-expression-in-a-mouse-model-of-multiple-sclerosis
#6
Mohammadreza Semnani, Farhad Mashayekhi, Mahnaz Azarnia, Zivar Salehi
The cuprizone multiple sclerosis (MS) animal model is characteristic for toxic demyelination and represents a reversible demyelination and remyelination system. It has been shown that green tea epigallocatechin-3-gallate (EGCG) might be effective in improving the symptoms and pathological conditions associated with autoimmune inflammatory diseases in several animal models. In this study the effects of EGCG on proteolipid protein (PLP) and oligodendrocyte transcription factor 1 (Olig1) expression in the cerebral cortex of a murine model of cuprizone-induced demyelination was investigated...
2017: Folia Neuropathologica
https://www.readbyqxmd.com/read/28965011/anti-encephalitogenic-effects-of-ethyl-pyruvate-are-reflected-in-the-central-nervous-system-and-the-gut
#7
Neda Djedović, Suzana Stanisavljevic, Bojan Jevtić, Miljana Momčilović, Irena Lavrnja, Djordje Miljković
Ethyl pyruvate is a redox analogue of dimethyl fumarate (Tecfidera), a drug for multiple sclerosis treatment. We have recently shown that ethyl pyruvate ameliorates experimental autoimmune encephalomyelitis (EAE), an animal model of multiple sclerosis. It affects encephalitogenic T cells and macrophages in vitro, as well as in lymph nodes draining the site of encephalitogenic immunization and within the central nervous system (CNS). Here, in vivo effects of ethyl pyruvate on EAE are thoroughly investigated in the CNS and within the gut associated lymphoid tissue...
September 28, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28964257/the-contribution-of-the-acute-phase-response-to-the-pathogenesis-of-relapse-in-chronic-relapsing-experimental-autoimmune-encephalitis-models-of-multiple-sclerosis
#8
Silvy Mardiguian, Emma Ladds, Roberta Turner, Hazel Shepherd, Sandra J Campbell, Daniel C Anthony
BACKGROUND: Increased relapse rates in multiple sclerosis (MS) as a consequence of peripheral immune system activation, owing to infection for example, have been widely reported, but the mechanism remains unclear. Acute brain injury models can be exacerbated by augmenting the hepatic acute phase response (APR). Here, we explored the contribution of the hepatic APR to relapse in two rodent models of MS. METHODS: Mice with MOG-CFA-induced chronic relapsing experimental autoimmune encephalitis (CR-EAE) were killed before, during and after the first phase of disease, and the brain and liver chemokine, cytokine and acute phase protein (APP) mRNA expression profile was determined...
September 30, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/28962999/nr4a1-plays-a-crucial-modulatory-role-in-th1-th17-cell-responses-and-cns-autoimmunity
#9
Li-Mei Wang, Yuan Zhang, Xing Li, Ming-Liang Zhang, Lin Zhu, Guang-Xian Zhang, Yu-Ming Xu
Nuclear receptor4 group A1 (Nr4a1), an orphan nuclear receptor, is down-regulated in peripheral blood mononuclear cells (MNCs) of individuals with multiple sclerosis (MS), and Nr4a1 deficiency results in severe experimental autoimmune encephalomyelitis (EAE), an animal model of MS, caused by increased macrophage infiltration into the central nervous system (CNS). However, the role of Nr4a1 in macrophage phenotype and T cell responses remains poorly understood. In the present study we show that macrophages/microglia of Nr4a1(-/-) mice, which exhibited earlier onset and more severe clinical EAE, were polarized to an enhanced type 1 (M1) phenotype and produced higher levels of IL-12 and TNF-α than wild type mice...
September 26, 2017: Brain, Behavior, and Immunity
https://www.readbyqxmd.com/read/28961183/splitting-the-unsplittable-dissecting-resident-and-infiltrating-macrophages-in-experimental-autoimmune-encephalomyelitis
#10
REVIEW
Tobias Koeniger, Stefanie Kuerten
Macrophages predominate the inflammatory landscape within multiple sclerosis (MS) lesions, not only regarding cellularity but also with respect to the diverse functions this cell fraction provides during disease progression and remission. Researchers have been well aware of the fact that the macrophage pool during central nervous system (CNS) autoimmunity consists of a mixture of myeloid cells. Yet, separating these populations to define their unique contribution to disease pathology has long been challenging due to their similar marker expression...
September 29, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28946394/bifidobacterium-animalis-in-combination-with-human-origin-of-lactobacillus-plantarum-ameliorate-neuroinflammation-in-experimental-model-of-multiple-sclerosis-by-altering-cd4-t-cell-subset-balance
#11
Zohre Salehipour, Dariush Haghmorad, Mojtaba Sankian, Maryam Rastin, Reza Nosratabadi, Mohammad Mehdi Soltan Dallal, Nafiseh Tabasi, Mahdieh Khazaee, Leila Roozbeh Nasiraii, Mahmoud Mahmoudi
BACKGROUND: Multiple Sclerosis (MS) is a chronic inflammatory autoimmune disease of the central nervous system (CNS). Recent reports have shown that probiotics can induce immunomodulatory activity with promising effects in inflammatory diseases. This study was designed to reveal the molecular and cellular mechanisms underlying the effect of Lactobacillus plantarum A7, which comprises human commensal bacteria, and Bifidobacterium animalis, a potential probiotic strain, on alleviation of experimental autoimmune encephalomyelitis (EAE), an animal model of MS...
September 21, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28928747/modulation-of-multiple-sclerosis-and-its-animal-model-experimental-autoimmune-encephalomyelitis-by-food-and-gut-microbiota
#12
REVIEW
Ward J van den Hoogen, Jon D Laman, Bert A 't Hart
Multiple sclerosis (MS) is an autoimmune neurological disease characterized by chronic inflammation of the central nervous system (CNS), leading to demyelination, axonal damage, and symptoms such as fatigue and disability. Although the cause of MS is not known, the infiltration of peripherally activated immune cells into the CNS has a key pathogenic role. Accumulating evidence supports an important role of diet and gut microbiota in immune-mediated diseases. Preclinical as well as clinical studies suggest a role for gut microbiota and dietary components in MS...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28921467/mir-155-dysregulation-and-therapeutic-intervention-in-multiple-sclerosis
#13
Claire E McCoy
microRNAs play a fundamental role in the immune system. One particular microRNA, miR-155 plays a critical role in hematopoietic cell development and tightly regulates innate and adaptive immune responses in response to infection. However, its dysregulation, more specifically its overexpression, is closely associated with various inflammatory disorders. The purpose of this review is to consolidate how miR-155 underpins a variety of processes that contribute to the pathology of multiple sclerosis (MS). In particular, the impact of miR-155 is discussed with respect to human pathology and animal models...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28919455/is-there-a-role-for-il-17-in-the-pathogenesis-of-systemic-sclerosis
#14
REVIEW
Carlo Chizzolini, Aleksandra Maria Dufour, Nicolò Costantino Brembilla
In systemic sclerosis (SSc) immuno-inflammatory events are central to disease development. Amongst other mediators of inflammation, interleukin 17 (IL-17) and Th17 cells have been reported to be increased in the peripheral blood and target organs including involved skin in SSc. They participate and amplify inflammatory responses by inducing the production of cytokines such as IL-6, chemokines such as CCL2 and CXCL8 (IL-8), matrix metalloproteinases-1, -2, -9 and the expression of adhesion molecules in stromal cells including fibroblasts and endothelial cells...
September 15, 2017: Immunology Letters
https://www.readbyqxmd.com/read/28919108/the-renin-angiotensin-system-regulates-neurodegeneration-in-a-mouse-model-of-optic-neuritis
#15
Xiaoli Guo, Kazuhiko Namekata, Atsuko Kimura, Chikako Harada, Takayuki Harada
The major role of the renin-angiotensin system (RAS), including that of angiotensin II (Ang II), the principal effector molecule, in the cardiovascular system is well known. Increasing evidence suggests that the RAS also plays a role in the development of autoimmune diseases. Optic neuritis (ie, inflammation of the optic nerve, with retinal ganglion cell loss) is strongly associated with multiple sclerosis. We investigated the effects of candesartan, an Ang II receptor antagonist, on optic neuritis in experimental autoimmune encephalomyelitis (EAE), an animal model of multiple sclerosis...
September 15, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28915963/hepcidin-and-metallothioneins-as-molecular-base-for-sex-dependent-differences-in-clinical-course-of-experimental-autoimmune-encephalomyelitis-in-chronic-iron-overload
#16
Božena Ćurko-Cofek, Tanja Grubić Kezele, Vesna Barac-Latas
Multiple sclerosis is a chronic demyelinating disease of the central nervous system characterised by inflammatory and degenerative changes. It is considered that disease arises from the influence of environmental factors on genetically susceptible individuals. Recent researches, using magnetic resonance imaging, connected iron deposits in different brain regions with demyelinating process in multiple sclerosis patients. Although iron is an essential trace element important for many biological functions it could be harmful because iron excess can induce the production of reactive oxygen species, development of oxidative stress and lipid peroxidation which leads to demyelination...
September 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28914804/the-role-of-erk-signaling-in-experimental-autoimmune-encephalomyelitis
#17
Katharina Birkner, Beatrice Wasser, Julia Loos, Alexander Plotnikov, Rony Seger, Frauke Zipp, Esther Witsch, Stefan Bittner
Extracellular signal-regulated kinase (ERK) signaling plays a crucial role in regulating immune cell function and has been implicated in autoimmune disorders. To date, all commercially available inhibitors of ERK target upstream components, such as mitogen-activated protein (MAP) kinase/ERK kinase (MEKs), but not ERK itself. Here, we directly inhibit nuclear ERK translocation by a novel pharmacological approach (Glu-Pro-Glu (EPE) peptide), leading to an increase in cytosolic ERK phosphorylation during T helper (Th)17 cell differentiation...
September 15, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28913726/diagnosis-and-treatment-of-progressive-multifocal-leukoencephalopathy-associated-with-multiple-sclerosis-therapies
#18
REVIEW
Eric M L Williamson, Joseph R Berger
Progressive multifocal leukoencephalopathy (PML) is a rare, but serious, complication encountered in patients treated with a select number of disease-modifying therapies (DMTs) utilized in treating multiple sclerosis (MS). PML results from a viral infection in the brain for which the only demonstrated effective therapy is restoring the perturbed immune system-typically achieved in the patient with MS by removing the offending therapeutic agent or, in the case of HIV-associated PML, treatment with highly active antiretroviral therapies...
September 14, 2017: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
https://www.readbyqxmd.com/read/28904861/a-modified-graft-versus-host-induced-model-for-systemic-sclerosis-with-pulmonary-fibrosis-in-rag2-deficient-mice
#19
Xue Yang, Chi Liu, Masayuki Fujino, Ji Yang, Xiao-Kang Li, Hejian Zou
Systemic sclerosis (SSc) is a connective tissue disease that results in fibrosis in multiple organs. Various animal models for this disease have been developed, both genetic and induced. One of the induced models, sclerodermatous graft-versus-host disease (scl-GvHD), exhibits the main characteristics of SSc, but involves lethal γ-irradiation of recipients. We sought to develop a modified scl-GvHD model. Spleen cells from B10.D2 donor mice were transplanted into immunodeficient Rag-2 recipients on the BALB/c genetic background...
September 2017: FEBS Open Bio
https://www.readbyqxmd.com/read/28891414/therapeutic-effects-of-walnut-oil-on-the-animal-model-of-multiple-sclerosis
#20
Ali Ganji, Iman Farahani, Mohammad Reza Palizvan, Ali Ghazavi, Mostafa Ejtehadifar, Mohsen Ebrahimimonfared, Mana Shojapour, Ghasem Mosayebi
OBJECTIVES: Therapeutic approaches for multiple sclerosis (MS), an autoimmune disease of the central nervous system (CNS), are accompanied by various undesirable side effects. Owing to the anti-inflammatory and antioxidant effects of walnut, we investigated its effects on the experimental autoimmune encephalomyelitis (EAE) mouse model of MS. METHODS: After EAE induction in mice, the treated group was gavaged daily with walnut oil. The weights and clinical symptoms were monitored daily for 21 days following the onset of symptoms...
September 11, 2017: Nutritional Neuroscience
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