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https://www.readbyqxmd.com/read/28646679/hypoxia-induced-adam-17-expression-is-mediated-by-rsk1-dependent-c-ebp%C3%AE-activation-in-human-lung-fibroblasts
#1
Jing-Yun Chen, Chien-Huang Lin, Bing-Chang Chen
Hypoxia was identified as a mediator of lung fibrosis in patients with chronic obstructive asthma (COA). Overexpression of a disintegrin and metalloproteinase 17 (ADAM 17) and connective tissue growth factor (CTGF) leads to development of tissue fibrosis. However, the signaling pathway in hypoxia-induced ADAM 17 expression remains poorly defined. In this study, we investigated the roles that ribosomal S-6 kinase 1 (RSK1)/CCAAT/enhancer-binding protein β (C/EBPβ)-dependent ADAM 17 expression plays in hypoxia-induced CTGF expression in human lung fibroblasts...
June 21, 2017: Molecular Immunology
https://www.readbyqxmd.com/read/28646623/pregnancy-outcome-in-women-with-cystic-fibrosis-related-diabetes
#2
Quitterie Reynaud, Stéphanie Poupon-Bourdy, Muriel Rabilloud, Lina Al Mufti, Christine Rousset Jablonski, Lydie Lemonnier, Raphaële Nove-Josserand, Sandrine Touzet, Isabelle Durieu
With increasing life expectancy more women with cystic fibrosis and diabetes mellitus become pregnant. We investigated how pre-gestational diabetes (cystic fibrosis related diabetes) influenced pregnancy outcome and the clinical status of these women MATERIAL AND METHODS: We analyzed all pregnancies reported to the French cystic fibrosis registry between 2001 and 2012, and compared forced expiratory volume (FEV1 ) and body mass index before and after pregnancy in women with and without pre-gestational diabetes having a first delivery RESULTS: A total 249 women delivered 314 infants...
June 24, 2017: Acta Obstetricia et Gynecologica Scandinavica
https://www.readbyqxmd.com/read/28646553/epithelial-mesenchymal-transition-emt-a-spectrum-of-states-role-in-lung-development-homeostasis-and-disease
#3
REVIEW
Mohit Kumar Jolly, Chris Ward, Mathew Suji Eapen, Stephen Myers, Oskar Hallgren, Herbert Levine, Sukhwinder Singh Sohal
Epithelial Mesenchymal Transition (EMT) plays key roles during lung development and many lung diseases such as Chronic Obstructive Pulmonary Disease (COPD), lung cancer and pulmonary fibrosis. Here, integrating morphological observations with underlying molecular mechanisms, we highlight the functional role of EMT in lung development and injury repair, and discuss how it can contribute to pathogenesis of chronic lung disease. We discuss the evidence of manifestation of EMT and its potential driving role in COPD, idiopathic pulmonary fibrosis (IPF), bronchiolitis obliterans syndrome (BOS), and lung cancer, while noting that all cells need not display a full EMT in any of these contexts, i...
June 24, 2017: Developmental Dynamics: An Official Publication of the American Association of Anatomists
https://www.readbyqxmd.com/read/28646530/pirfenidone-reduces-subchondral-bone-loss-and-fibrosis-after-murine-knee-cartilage-injury
#4
Deva D Chan, Jun Li, Wei Luo, Dan N Predescu, Brian J Cole, Anna Plaas
Pirfenidone is an anti-inflammatory and anti-fibrotic drug that has shown efficacy in lung and kidney fibrosis. Because inflammation and fibrosis have been linked to the progression of osteoarthritis, we investigated the effects of oral Pirfenidone in a mouse model of cartilage injury, which results in chronic inflammation and joint-wide fibrosis in mice that lack hyaluronan synthase 1 (Has1(-/-) ) in comparison to wild-type. Femoral cartilage was surgically injured in wild-type and Has1(-/-) mice, and Pirfenidone was administered in food starting after 3 days...
June 23, 2017: Journal of Orthopaedic Research: Official Publication of the Orthopaedic Research Society
https://www.readbyqxmd.com/read/28646304/ndrg2-knockdown-promotes-fibrosis-in-renal-tubular-epithelial-cells-through-tgf-%C3%AE-1-smad3-pathway
#5
Zhibo Jin, Chaohui Gu, Fengyan Tian, Zhankui Jia, Jinjian Yang
Renal fibrosis is a common pathological pathway of various chronic kidney diseases progressing to end-stage renal disease and is characterized by tubular atrophy, fibroblast/myofibroblast activation and excessive deposition of extracellular matrix (ECM). N-Myc downstream-regulated gene-2 (NDRG2) is reported to be associated with liver fibrosis in rats. However, the biological function of NDRG2 in renal fibrosis remains unclear. Therefore, we investigate the effect of NDRG2 on renal fibrosis and the underlying mechanism of NDRG2 in TGF-β1-induced renal tubular epithelial cells (HK-2)...
June 23, 2017: Cell and Tissue Research
https://www.readbyqxmd.com/read/28646244/increased-soluble-vcam-1-and-normal-p-selectin-in-cystic-fibrosis-a-cross-sectional-study
#6
Jan K Nowak, Irena Wojsyk-Banaszak, Edyta Mądry, Andrzej Wykrętowicz, Patrycja Krzyżanowska, Sławomira Drzymała-Czyż, Agata Nowicka, Andrzej Pogorzelski, Ewa Sapiejka, Wojciech Skorupa, Mariusz Szczepanik, Aleksandra Lisowska, Jaroslaw Walkowiak
PURPOSE: As life expectancy in cystic fibrosis (CF) increases, questions regarding its potential impact on cardiovascular health arise. Soluble vascular cell adhesion molecule 1 (sVCAM-1), P-selectin (sP-selectin) are proposed as biomarkers of cardiovascular disease. We aimed to: compare their concentrations in clinically stable CF patients and healthy subjects (HS) and verify whether they independently correlate with CF characteristics. METHODS: Serum sVCAM-1 and sP-selectin levels were measured using ELISA...
June 23, 2017: Lung
https://www.readbyqxmd.com/read/28646221/aberrant-activation-of-the-human-sex-determining-gene-in-early-embryonic-development-results-in-postnatal-growth-retardation-and-lethality-in-mice
#7
Tatsuo Kido, Zhaoyu Sun, Yun-Fai Chris Lau
Sexual dimorphisms are prevalent in development, physiology and diseases in humans. Currently, the contributions of the genes on the male-specific region of the Y chromosome (MSY) in these processes are uncertain. Using a transgene activation system, the human sex-determining gene hSRY is activated in the single-cell embryos of the mouse. Pups with hSRY activated (hSRY(ON)) are born of similar sizes as those of non-activated controls. However, they retard significantly in postnatal growth and development and all die of multi-organ failure before two weeks of age...
June 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28646122/nintedanib-a-triple-tyrosine-kinase-inhibitor-attenuates-renal-fibrosis-in-chronic-kidney-disease
#8
Feng Liu, Li Wang, Hualin Qi, Jun Wang, Wei Jiang, Yi Wang, Liuqing Xu, Na Liu, Shougang Zhuang
<span style="margin: 0px; color: black; line-height: 200%; font-family: 'Times New Roman',serif; font-size: 12pt;">Nintedanib (BIBF1120) is a triple kinase inhibitor</span><span style="margin: 0px; color: black; line-height: 200%; font-family: 'Times New Roman',serif; font-size: 12pt;"> of </span><span style="margin: 0px; color: black; line-height: 200%; font-family: 'Times New Roman',serif; font-size: 12pt;">platelet derived growth factor receptor (PDGFR), fibroblast growth factor receptors (FGFR)</span><span style="margin: 0px; color: black; line-height: 200%; font-family: 'Times New Roman',serif; font-size: 12pt;">, </span><span style="margin: 0px; color: black; line-height: 200%; font-family: 'Times New Roman',serif; font-size: 12pt;">vascular endothelial growth factor receptor (VEGFR)</span><span style="margin: 0px; color: black; line-height: 200%; font-family: 'Times New Roman',serif; font-size: 12pt;"> and Src family kinase, </span><span style="margin: 0px; color: black; line-height: 200%; font-family: 'Times New Roman',serif; font-size: 12pt;">that has recently been approved by FDA to treat idiopathic pulmonary fibrosis...
June 23, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/28646120/mir-223-represents-a-biomarker-in-acute-and-chronic-liver-injury
#9
Florian Schueller, Sanchari Roy, Sven Heiko Loosen, Jan Alder, Christiane Koppe, Anne Theres Schneider, Franziska Wandrer, Heike Bantel, Mihael Vucur, Qing-Sheng Mi, Christian Trautwein, Tom Luedde, Christoph Roderburg
Background: Dysregulation of miRNAs has been described in tissue and serum from patients with acute and chronic liver diseases. However, only little information on the role of miR-223 in the pathophysiology of acute liver failure (ALF) and liver cirrhosis is available.  Methods: We analysed cell and tissue specific expression levels as well as serum concentrations of miR-223 in mouse models of acute (hepatic ischemia and reperfusion, single CCl4 injection) and chronic (repetitive CCl4 injection, bile duct ligation) liver diseases...
June 23, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/28646026/exosomes-promising-sacks-for-treating-ischemic-heart-disease
#10
Gui-Hao Chen, Jun Xu, Yue-Jin Yang
Ischemic heart disease(IHD) is the leading cause of death worldwide. Despite development of continuously improving therapeutic strategies, morbidity and mortality of patients with IHD remains relatively high. Exosomes are a subpopulation of vesicles that are universally recognized as major mediators in intercellular communication. Numerous preclinical studies showed that these tiny vesicles were protective in IHD, through such actions as alleviating myocardial ischemia/reperfusion injury, promoting angiogenesis, inhibiting fibrosis and facilitating cardiac regeneration...
June 23, 2017: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/28645798/left-atrial-mechanics-and-integrated-calibrated-backscatter-in-anthracycline-treated-long-term-survivors-of-childhood-cancers
#11
Vivian Wing-Yi Li, Clare Tik-Man Lai, Anthony Pak-Yin Liu, Daniel Ka-Leung Cheuk, Yiu-Fai Cheung
We tested the hypothesis that left atrial (LA) mechanics and myocardial calibrated integrated backscatter (cIB) are altered in anthracycline-treated long-term survivors of childhood cancers. Forty-nine survivors and 25 controls were studied. Survivors had significantly smaller maximal (p = 0.009) and minimal (p = 0.017) LA volumes and lower peak negative LA strains (p = 0.011). For left ventricular (LV) indices, survivors had significantly lower shortening fraction (p < 0.001), ejection fraction (p < 0...
June 20, 2017: Ultrasound in Medicine & Biology
https://www.readbyqxmd.com/read/28645738/human-germline-hedgehog-pathway-mutations-predispose-to-fatty-liver
#12
Maria J Guillen Sacoto, Ariel F Martinez, Yu Abe, Paul Kruszka, Karin Weiss, Joshua L Everson, Ramon Bataller, David E Kleiner, Jerrold M Ward, Kathleen K Sulik, Robert J Lipinski, Benjamin D Solomon, Maximilian Muenke
BACKGROUND & AIMS: Non-alcoholic fatty liver disease (NAFLD) is the most common form of liver disease. Activation of hedgehog (Hh) signaling has been implicated in the progression of NAFLD and proposed as a therapeutic target; however, the effects of Hh signaling inhibition have not been studied in humans with germline mutations affecting this pathway. METHODS: Patients with holoprosencephaly (HPE), a disorder associated with germline mutations disrupting Sonic hedgehog (SHH) signaling, were clinically evaluated for NAFLD...
June 20, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28645539/gli1-labeled-adult-mesenchymal-stem-progenitor-cells-and-hedgehog-signaling-contribute-to-endochondral-heterotopic-ossification
#13
Chen Kan, Lijun Chen, Yangyang Hu, Na Ding, Yuyun Li, Tammy L McGuire, Haimei Lu, John A Kessler, Lixin Kan
Heterotopic ossification (HO), acquired or hereditary, endochondral or intramembranous, is the formation of true bone outside the normal skeleton. Since perivascular Gli1+ progenitors contribute to injury induced organ fibrosis, and CD133 is expressed by a variety of populations adult stem cells, this study utilized Cre-lox based genetic lineage tracing to test the contribution to endochondral HO of adult stem/progenitor cells that expressed either Gli1 or CD133. We found that both lineages contributed broadly to different normal tissues with distinct patterns, but that only Gli1-creERT labeled stem/progenitor cells contributed to all stages of endochondral HO in a BMP dependent, injury induced, transgenic mouse model...
June 20, 2017: Bone
https://www.readbyqxmd.com/read/28645253/a-case-report-of-thoracolumbar-paraspinal-myopathy-as-the-cause-of-camptocormia-in-a-patient-with-atypical-parkinsonism
#14
Yoon Kim, Ahro Kim, Aryun Kim, Beomseok Jeon
BACKGROUND: Camptocormia is severe flexion of the thoracolumbar spine, exaggerated during standing and walking but minimized in supine position. Even though camptocormia is a relatively common condition during the course of Parkinson's disease, there is ongoing controversy concerning its mechanisms. The most widely accepted and yet still disputed one is dystonia. However, based on myopathic changes observed in the paraspinal muscle biopsies of some PD patients with camptocormia, the attempt to attribute camptocormia to myopathy has continued...
June 23, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28645157/secretory-iga-response-against-pseudomonas-aeruginosa-in-the-upper-airways-and-the-link-with-chronic-lung-infection-in-cystic-fibrosis
#15
Renan Mauch, Claudio Rossi, Talita Aiello, José Ribeiro, Antonio Ribeiro, Niels Høiby, Carlos Levy
We assessed the diagnostic ability of an ELISA test for measurement of specific secretory IgA (sIgA) in saliva to identify Cystic Fibrosis (CF) patients with P. aeruginosa chronic lung infection and intermittent lung colonization. A total of 102 Brazilian CF patients and 53 healthy controls were included. Specific serum IgG response was used as a surrogate to distinguish CF patients according to their P. aeruginosa colonization/infection status. The rate of sIgA positivity was 87.1% in CF chronically infected patients (Median value = 181...
June 22, 2017: Pathogens and Disease
https://www.readbyqxmd.com/read/28645009/case-report-of-robotic-dor-fundoplication-for-scleroderma-esophagus-with-aperistalsis-on-manometry
#16
Alonso Andrade, Matthew K Folstein, Brian R Davis
Scleroderma is a systemic disease of collagen deposition resulting in fibrosis of small arteries and arterioles. It commonly affects the skin, lungs, and gastrointestinal tract. The most common site of GI tract involvement is the esophagus. We present the case report of a 44year old female with scleroderma esophagus and severe reflux which was successfully treated with robotic dor fundoplication. Because of the wide variety of symptoms with which this problem can present, a tailored approach taking into consideration the patient's symptomatology and findings during diagnostic work-up was implemented with good results...
June 16, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28644870/effects-of-nicotine-on-corneal-wound-healing-following-acute-alkali-burn
#17
Jong Won Kim, Chae Woong Lim, Bumseok Kim
Epidemiological studies have indicated that smoking is a pivotal risk factor for the progression of several chronic diseases. Nicotine, the addictive component of cigarettes, has powerful pathophysiological properties in the body. Although the effects of cigarette smoking on corneal re-epithelialization have been studied, the effects of nicotine on corneal wound healing-related neovascularization and fibrosis have not been fully demonstrated. The aim of this study was to evaluate the effects of chronic administration of nicotine on corneal wound healing following acute insult induced by an alkali burn...
2017: PloS One
https://www.readbyqxmd.com/read/28644868/electrophysiological-alterations-in-a-murine-model-of-chronic-coxsackievirus-b3-myocarditis
#18
Sven Kaese, Robert Larbig, Matthias Rohrbeck, Gerrit Frommeyer, Dirk Dechering, Jan Olligs, Sabine Schönhofer-Merl, Rainer Wessely, Karin Klingel, Guiscard Seebohm, Lars Eckardt
INTRODUCTION: Coxsackievirus B3 (CVB3) is known to induce acute and chronic myocarditis. Most infections are clinically unapparent but some patients suffer from ventricular arrhythmias (VA) and sudden cardiac death (SCD). Studies showed that acute CVB3 infection may cause impaired function of cardiac ion channels, creating a proarrhythmic substrate. However, it is unknown whether low level CVB3+ expression in myocytes may cause altered cardiac electrophysiology leading to VA. METHODS: Cellular electrophysiology was used to analyze cellular action potentials (APs) and occurrence of afterdepolarizations from isolated cardiomyocytes of wildtype (WT) and transgenic CVB3ΔVP0 (CVB3+) mice...
2017: PloS One
https://www.readbyqxmd.com/read/28644845/lipo-pge1-suppresses-collagen-production-in-human-dermal-fibroblasts-via-the-erk-ets-1-signaling-pathway
#19
Yoolhee Yang, Hee Jung Kim, Kyong-Je Woo, Daeho Cho, Sa Ik Bang
Dysregulation of collagen production contributes to various pathological processes, including tissue fibrosis as well as impaired wound healing. Lipo-prostaglandin E1 (Lipo-PGE1), a lipid microsphere-incorporated prostaglandin E1, is used as a vasodilator for the treatment of peripheral vascular diseases. Lipo-PGE1 was recently shown to enhance human dermal fibroblast (HDF) migration and in vivo wound healing. No published study has characterized the role of Lipo-PGE1 in collagen regulation in HDFs. Here, we investigated the cellular signaling mechanism by which Lipo-PGE1 regulates collagen in HDFs...
2017: PloS One
https://www.readbyqxmd.com/read/28644693/supplemental-oxygen-in-interstitial-lung-disease-an-art-in-need-of-science
#20
Kerri A Johannson, Sachin R Pendharkar, Kirk Mathison, Charlene D Fell, Jordan A Guenette, Meena Kalluri, Martin Kolb, Christopher J Ryerson
Interstitial lung disease (ILD) comprises a large and heterogeneous group of disorders that often lead to progressive fibrosis and premature death. Oxygen supplementation is typically used in advanced lung disease patients with resting hypoxemia; however, there is a paucity of evidence guiding the use of supplemental oxygen in ILD, and significant heterogeneity in clinical practice. It remains unclear whether supplemental oxygen improves clinically meaningful outcomes, and the role of ambulatory oxygen supplementation in isolated exertional hypoxemia is particularly controversial...
June 23, 2017: Annals of the American Thoracic Society
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