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https://www.readbyqxmd.com/read/29790918/temporal-and-tissue-specific-variability-of-smn-protein-levels-in-mouse-models-of-spinal-muscular-atrophy
#1
Ewout Jn Groen, Elena Perenthaler, Natalie L Courtney, Crispin Y Jordan, Hannah K Shorrock, Dinja van der Hoorn, Yu-Ting Huang, Lyndsay M Murray, Gabriella Viero, Thomas H Gillingwater
Spinal muscular atrophy (SMA) is a progressive motor neuron disease caused by deleterious variants in SMN1 that lead to a marked decrease in survival motor neuron (SMN) protein expression. Humans have a second SMN gene (SMN2) that is almost identical to SMN1. However, due to alternative splicing the majority of SMN2 mRNA is translated into a truncated, unstable protein that is quickly degraded. Because the presence of SMN2 provides a unique opportunity for therapy development in SMA patients, the mechanisms that regulate SMN2 splicing and mRNA expression have been elucidated in great detail...
May 22, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29784903/exercise-ameliorates-endoplasmic-reticulum-stress-mediated-vascular-dysfunction-in-mesenteric-arteries-in-atherosclerosis
#2
Junyoung Hong, Kwangchan Kim, Eunkyung Park, Jonghae Lee, Melissa M Markofski, Sean P Marrelli, Yoonjung Park
Endoplasmic reticulum (ER) stress is closely associated with atherosclerosis, but the effects of exercise on ER stress-mediated endothelial dysfunction in atherosclerosis is not yet fully understood. We assessed endothelium-dependent vasodilation in isolated mesenteric arteries from wild type (WT), WT with exercise (WT-EX), ApoE knockout (ApoE KO), and ApoE KO mice with exercise (ApoE KO-EX). Vasodilation to acetylcholine (ACh) was elicited in the presence of inhibitors of ER stress, eNOS, caspase-1, and UCP-2 (Tudca, L-NAME, AC-YVARD-cmk, and Genipin, respectively) and the ER stress inducer (Tunicamycin)...
May 21, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29775572/gene-therapy-with-antisense-oligonucleotides-silencing-c-myc-reduces-neointima-formation-and-vessel-wall-thickness-in-a-mouse-model-of-vein-graft-disease
#3
Christina Maria Steger, Nikolaos Bonaros, Ralf Joachim Rieker, Johannes Bonatti, Thomas Schachner
Gene therapy for avoiding intimal hyperplasia of vein grafts after coronary artery bypass grafting is still discussed controversially. A promising application of gene therapy in vein grafts is the use of antisense oligonucleotides to block the expression of genes encoding cell cycle regulatory proteins in vascular smooth muscle cells. C-myc, either directly or by regulating the expression of other proteins, controls cell proliferation, apoptosis and cell survival, tissue remodeling, angiogenesis, cell metabolism, production of inflammatory and anti-inflammatory cytokines, and also participates in cell transformation...
May 15, 2018: Experimental and Molecular Pathology
https://www.readbyqxmd.com/read/29770156/probing-into-the-mechanism-of-alkaline-citrus-extract-promoted-apoptosis-in-pulmonary-fibroblasts-of-bleomycin-induced-pulmonary-fibrosis-mice
#4
Qi Wu, Yao Zhou, Fan-Chao Feng, Yi-Han Jin, Zhi-Chao Wang, Xian-Mei Zhou
We extracted the primary pulmonary fibroblasts of the normal and bleomycin-induced pulmonary fibrosis mice and investigated the functioning mechanism of citrus alkaline extract (CAE) in the induction of pulmonary fibroblast apoptosis. The expression intensity of vimentin of the pulmonary fibroblasts in the model mice was higher than that in the normal mice. Meanwhile, the positive expression rate and expression intensity of alpha smooth muscle actin ( α -SMA) of the pulmonary fibroblasts in the model mice were higher than those in the normal mice...
2018: Evidence-based Complementary and Alternative Medicine: ECAM
https://www.readbyqxmd.com/read/29768198/myocardin-related-transcription-factor-a-promotes-recruitment-of-itga5-profibrotic-progenitors-during-obesity-induced-adipose-tissue-fibrosis
#5
Jean Z Lin, Nabil Rabhi, Stephen R Farmer
Adipose tissue fibrosis is associated with inflammation and insulin resistance in human obesity. In particular, visceral fat fibrosis is correlated with hyperlipidemia and ectopic fat accumulation. Myocardin-related transcription factor A (MRTFA) is an important coactivator that mediates the transcription of extracellular matrix and other fibrogenic genes. Here, we examine the role of MRTFA in the development of adipose tissue fibrosis and identify a signaling pathway that regulates the fate of vascular progenitors...
May 15, 2018: Cell Reports
https://www.readbyqxmd.com/read/29767748/glial-activation-and-central-synapse-loss-but-not-motoneuron-degeneration-are-prevented-by-the-sigma-1-receptor-agonist-pre-084-in-the-smn2b-mouse-model-of-spinal-muscular-atrophy
#6
Clàudia Cerveró, Alba Blasco, Olga Tarabal, Anna Casanovas, Lídia Piedrafita, Xavier Navarro, Josep E Esquerda, Jordi Calderó
Spinal muscular atrophy (SMA) is characterized by the loss of α-motoneurons (MNs) with concomitant muscle denervation. MN excitability and vulnerability to disease are particularly regulated by cholinergic synaptic afferents (C-boutons), in which Sigma-1 receptor (Sig1R) is concentrated. Alterations in Sig1R have been associated with MN degeneration. Here, we investigated whether a chronic treatment with the Sig1R agonist PRE-084 was able to exert beneficial effects on SMA. We used a model of intermediate SMA, the Smn2B/- mouse, in which we performed a detailed characterization of the histopathological changes that occur throughout the disease...
May 14, 2018: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29757584/effects-of-nerve-growth-factor-shrna-inhibition-on-asthma-phenotypes-in-a-mouse-model-of-asthma
#7
Jinhong Wu, Bing Chen, Wenxian Li, Yanan Xiao
Nerve growth factor (NGF) plays an important role in airway hyper-responsiveness (AHR). In this study, we aimed at investigating the effect of NGF inhibition on AHR and other asthma phenotypes in a mouse model of asthma. 12 mice in each group were injected with lentiviral vectors expressing non-targeting shRNA (sham shRNA), targeting NGF (shRNA-1 and shRNA-2), or normal saline for control before the asthma models were established. Peak inspiratory pressure (PIP), NGF levels in bronchoalveolar lavage fluid (BALF), and bronchoconstriction in response to acetylcholine (ACh) were measured...
April 2018: Iranian Journal of Allergy, Asthma, and Immunology
https://www.readbyqxmd.com/read/29752580/loss-of-hox5-function-results-in-myofibroblast-mislocalization-and-distal-lung-matrix-defects-during-postnatal-development
#8
Steven M Hrycaj, Leilani Marty-Santos, Andrew J Rasky, Nicholas W Lukacs, Deneen M Wellik
Alveologenesis is the final stage of lung development and is responsible for the formation of the principle gas exchange units called alveoli. The lung mesenchyme, in particular the alveolar myofibroblasts, are drivers of alveolar development, however, few key regulators that govern the proper distribution and behavior of these cells in the distal lung during alveologenesis have been identified. While Hox5 triple mutants (Hox5 aabbcc) exhibit neonatal lethality, four-allele, compound mutant mice (Hox5 AabbCc) are born in Mendelian ratios and are phenotypically normal at birth...
April 27, 2018: Science China. Life Sciences
https://www.readbyqxmd.com/read/29749461/upregulation-of-allograft-inflammatory-factor%C3%A2-1-expression-and-secretion-by-macrophages-stimulated-with-aldosterone-promotes-renal-fibroblasts-to-a-profibrotic-phenotype
#9
Yushu Li, Xingzhi Wang, Lei Zhang, Xueying Yuan, Jianbing Hao, Jie Ni, Lirong Hao
Macrophages have been identified as a key cell type in the pathogenesis of renal interstitial fibrosis (RIF). However, the mechanism through which macrophages drive fibrosis remains unclear. The current study focuses on the effects and possible underlying mechanism of allograft inflammatory factor‑1 (AIF‑1), an inflammation‑responsive scaffold protein expressed and secreted by macrophages, in promoting fibroblasts to a profibrotic phenotype. In vivo experiments indicated that AIF‑1, CD68 and α‑smooth muscle actin (α‑SMA) were upregulated in kidney tissues of mice subjected to unilateral ureteric obstruction, while their expressions were inhibited by an aldosterone receptor antagonist, spironolactone...
May 10, 2018: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/29747347/-the-establishment-of-the-immortalized-mouse-brain-microvascular-pericytes-model-and-its-preliminary-application-in-screening-of-cerebrovascular-toxicants
#10
H P Zhao, Y F Gao, D Xia, Z Q Zhao, S Wu, X H Wang, H X Liu, C Xiao, X M Xing, Y He
Objective: To establish the immortalized mouse brain microvascular pericytes model and to apply to the cerebrovascular toxicants screening study. Methods: Brain pericytes were isolated from 3 weeks of mice by tissue digestion. Immortalized pericyte cell line was constructed by infecting with LT retrovirus. Monoclone was selected to purify the immortalized pericyte cell line. The pericyte characteristics and purity were explored by immunocytochemistry. Cell proliferation was measured by using the Pomega MTS cell Proliferation Colorimetric Assay Kit...
May 6, 2018: Zhonghua Yu Fang Yi Xue za Zhi [Chinese Journal of Preventive Medicine]
https://www.readbyqxmd.com/read/29747160/targeting-liver-cancer-and-associated-pathologies-in-mice-with-a-mitochondrial-vdac1-based-peptide
#11
Srinivas Pittala, Yakov Krelin, Varda Shoshan-Barmatz
Hepatocellular carcinoma (HCC) is the third most lethal cancer worldwide. Despite progress in identifying risk factors, the incidence of HCC is increasing. Moreover, therapeutic options are limited and survival is poor. Therefore, alternative and innovative therapeutic strategies are urgently required. R-Tf-D-LP4, a cell-penetrating peptide derived from the mitochondrial multifunctional protein the voltage-dependent anion channel (VDAC1), is identified here as a highly effective liver cancer treatment. Recently, we demonstrated that R-Tf-D-LP4 induced apoptosis and inhibited tumor growth in mouse models...
May 7, 2018: Neoplasia: An International Journal for Oncology Research
https://www.readbyqxmd.com/read/29743985/andrographolide-ameliorates-liver-fibrosis-in-mice-involvement-of-tlr4-nf-%C3%AE%C2%BA-b-and-tgf-%C3%AE-1-smad2-signaling-pathways
#12
Liteng Lin, Rui Li, Mingyue Cai, Jingjun Huang, Wensou Huang, Yongjian Guo, Liuhong Yang, Guizhi Yang, Tian Lan, Kangshun Zhu
Liver fibrosis is characterized by activated hepatic stellate cells (HSC) and extracellular matrix accumulation. Blocking the activation of HSC and the inflammation response are two major effective therapeutic strategies for liver fibrosis. In addition to the long history of using andrographolide (Andro) for inflammatory disorders, we aimed at elucidating the pharmacological effects and potential mechanism of Andro on liver fibrosis. In this study, liver fibrosis was induced by carbon tetrachloride (CCl4 ) and the mice were intraperitoneally injected with Andro for 6 weeks...
2018: Oxidative Medicine and Cellular Longevity
https://www.readbyqxmd.com/read/29743924/emodin-alleviates-liver-fibrosis-of-mice-by-reducing-infiltration-of-gr1-hi-monocytes
#13
Xiang-An Zhao, Guangmei Chen, Yong Liu, Hongyan Wu, Jin Chen, Yali Xiong, Chen Tian, Bei Jia, Guiyang Wang, Juan Xia, Yuxin Chen, Jian Wang, Xiaomin Yan, Zhaoping Zhang, Rui Huang, Chao Wu
Emodin, as a major active component of Rheum palmatum L. and Polygonum cuspidatum , has been reported to have antifibrotic effect. However, the mechanism of emodin on antifibrotic effect for liver fibrosis was still obscure. In the present study, we aimed to investigate whether emodin can alleviate carbon tetrachloride- (CCl4 -) induced liver fibrosis through reducing infiltration of Gr1hi monocytes. Liver fibrosis was induced by intraperitoneal CCl4 injection in mice. Mice in the emodin group received emodin treatment by gavage...
2018: Evidence-based Complementary and Alternative Medicine: ECAM
https://www.readbyqxmd.com/read/29740320/amifostine-analog-drde-30-attenuates-bleomycin-induced-pulmonary-fibrosis-in-mice
#14
Aastha Arora, Vikas Bhuria, Puja P Hazari, Uma Pathak, Sweta Mathur, Bal G Roy, Rajat Sandhir, Ravi Soni, Bilikere S Dwarakanath, Anant N Bhatt
Bleomycin (BLM) is an effective curative option in the management of several malignancies including pleural effusions; but pulmonary toxicity, comprising of pneumonitis and fibrosis, poses challenge in its use as a front-line chemotherapeutic. Although Amifostine has been found to protect lungs from the toxic effects of radiation and BLM, its application is limited due to associated toxicity and unfavorable route of administration. Therefore, there is a need for selective, potent, and safe anti-fibrotic drugs...
2018: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/29740240/antifibrotic-effects-of-crocin-on-thioacetamide-induced-liver-fibrosis-in-mice
#15
Mardi M Algandaby
Liver fibrosis is a major health concern that results in significant morbidity and mortality. Up-to-date, there is no standard treatment for fibrosis because of its complex pathogenesis. Crocin is one of the main nutraceuticals isolated from the stigma of Crocus sativus with antioxidant and anti-inflammatory activities. The current study aimed at evaluating the potential antifibrotic activity of crocin against thioacetamide (TAA)-induced liver fibrosis in mice as well as the underlying mechanism using silymarin as a reference antifibrotic product...
May 2018: Saudi Journal of Biological Sciences
https://www.readbyqxmd.com/read/29735415/interventions-targeting-glucocorticoid-kr%C3%A3-ppel-like-factor-15-branched-chain-amino-acid-signaling-improve-disease-phenotypes-in-spinal-muscular-atrophy-mice
#16
Lisa M Walter, Marc-Olivier Deguise, Katharina E Meijboom, Corinne A Betts, Nina Ahlskog, Tirsa L E van Westering, Gareth Hazell, Emily McFall, Anna Kordala, Suzan M Hammond, Frank Abendroth, Lyndsay M Murray, Hannah K Shorrock, Domenick A Prosdocimo, Saptarsi M Haldar, Mukesh K Jain, Thomas H Gillingwater, Peter Claus, Rashmi Kothary, Matthew J A Wood, Melissa Bowerman
The circadian glucocorticoid-Krüppel-like factor 15-branched-chain amino acid (GC-KLF15-BCAA) signaling pathway is a key regulatory axis in muscle, whose imbalance has wide-reaching effects on metabolic homeostasis. Spinal muscular atrophy (SMA) is a neuromuscular disorder also characterized by intrinsic muscle pathologies, metabolic abnormalities and disrupted sleep patterns, which can influence or be influenced by circadian regulatory networks that control behavioral and metabolic rhythms. We therefore set out to investigate the contribution of the GC-KLF15-BCAA pathway in SMA pathophysiology of Taiwanese Smn-/- ;SMN2 and Smn2B/- mouse models...
May 4, 2018: EBioMedicine
https://www.readbyqxmd.com/read/29729496/progressive-increase-in-allergen-concentration-abrogates-immune-tolerance-in-ovalbumin-induced-murine-model-of-chronic-asthma
#17
Gurupreet S Sethi, Amarjit S Naura
Persistent inflammation and remodeling of airways are the major hallmarks of asthma. Though airway inflammation diminishes in ovalbumin (OVA)-based mouse model of chronic asthma owing to immune-tolerance linked with repeated allergen exposure, which limits the application of the disease model. Accordingly, the present study was designed to develop a murine model of chronic asthma which presents persistent airway inflammation coupled with remodeling traits. Herein, OVA-sensitized BALB/c mice were challenged with increasing (modified protocol) or constant concentration (conventional protocol) of the allergen for 6 weeks; 3 times/week...
May 2, 2018: International Immunopharmacology
https://www.readbyqxmd.com/read/29723529/suppression-of-elp2-prevents-renal-fibrosis-and-inflammation-induced-by-unilateral-ureter-obstruction-uuo-via-inactivating-stat3-regulated-tgf-%C3%AE-1-and-nf-%C3%AE%C2%BAb-pathways
#18
Shan Lu, Hong-Wu Fan, Kai Li, Xiao-Di Fan
Renal fibrosis and inflammation are common underlying processes of progressive kidney diseases. Elongator protein 2 (Elp2), identical to signal transducer and activator of transcription-3 (STAT-3)-interacting protein-1 (Stip1), is a component of the Elongator complex that regulates RNA polymerase II. Elp2 regulates STAT-3 activation to control various cellular processes. The mechanisms of Elp2 prevention in renal interstitial fibrosis and inflammation remain unknown. In the study, Elp2 transgenic knockout (KO) and wild type (WT) mice were employed to investigate the effects of Elp2 on renal fibrosis and inflammation development after unilateral ureter obstruction (UUO) surgery...
April 30, 2018: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/29722566/inhibition-of-raf1-ameliorates-bleomycin-induced-pulmonary-fibrosis-through-attenuation-of-tgf%C3%AE-1-signaling
#19
Shuang Li, Jia Liu, Jiangning Tan, Lian Li, Mary J Kaltreider, Jing Zhao, Daniel J Kass, Dong Shang, Yutong Zhao
Idiopathic Pulmonary Fibrosis (IPF) is a fatal fibrotic lung disease associated with aberrant activation and differentiation of fibroblasts, leading to abnormal extracellular matrix (ECM) production. Currently, it is still an untreatable disease except lung transplantation. Here, we demonstrate that the Raf1 inhibitor GW5074 ameliorates lung fibrosis in bleomycin-induced pulmonary fibrosis. Post-treatment with GW5074 reduced fibronectin (FN) expression, collagen deposition, and inflammatory cell infiltration in bleomycin-challenged mice, suggesting an anti-fibrotic property of GW5074...
May 3, 2018: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29721568/hepatoprotection-of-auraptene-from-peels-of-citrus-fruits-against-thioacetamide-induced-hepatic-fibrosis-in-mice-by-activating-farnesoid-x-receptor
#20
Xiaoguang Gao, Changyuan Wang, Chenqing Ning, Kexin Liu, Xinyuan Wang, Zhihao Liu, Huijun Sun, Xiaodong Ma, Pengyuan Sun, Qiang Meng
Hepatic fibrosis is a pathological process that eventually leads to the development of cirrhosis and liver cancer by various types of chronic liver disease. To date, there is no standard treatment for the progression of liver fibrosis. This study aims to investigate the hepatoprotection of auraptene (AUR), a simple coumarin contained in the peels of citrus fruits such as grapefruit, against thioacetamide (TAA)-induced hepatic fibrosis in mice. The involvement of farnesoid X receptor (FXR) in the anti-fibrotic effect of AUR was further elucidated using in vivo and in vitro experiments...
May 3, 2018: Food & Function
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