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https://www.readbyqxmd.com/read/28922846/different-complicated-brain-pathologies-in-monozygotic-twins-with-gerstmann-str%C3%A3-ussler-scheinker-disease
#1
Hiroyuki Honda, Kensuke Sasaki, Hiroshi Takashima, Daisuke Mori, Sachiko Koyama, Satoshi O Suzuki, Toru Iwaki
Gerstmann-Sträussler-Scheinker disease (GSS) is an autosomal, dominantly inherited prion disease. In this study, we present different complicated brain pathologies determined postmortem of monozygotic GSS twin sisters. Case 1 showed cerebellar ataxia at the age of 58 years, and died at 66 years. Case 2 became symptomatic at the age of 75 years, and died at 79 years. There was a 17-year difference in the age of onset between the twins. Postmortem examination revealed numerous prion protein (PrP) plaques in the brains of both cases...
October 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28922766/altered-accumulation-of-osa-mir171b-contributes-to-rice-stripe-virus-infection-by-regulating-disease-symptoms
#2
Aizi Tong, Quan Yuan, Shu Wang, Jiejun Peng, Yuwen Lu, Hongying Zheng, Lin Lin, Hairu Chen, Yifu Gong, Jianping Chen, Fei Yan
Viral infection affects the pattern of plant miRNA expression. It has been presumed that reduction of miR171 and several other miRNAs influences viral symptoms in plants. We here experimentally demonstrate the association of osa-miR171b with rice stripe virus (RSV) symptoms in rice. Inhibition of osa-miR171b caused stunting with reduced chlorophyll content in leaves similar to viral symptoms. Overexpression of osa-miR171b by an artificial miRNA extended vegetative growth and enhanced chlorophyll accumulation in leaves...
July 10, 2017: Journal of Experimental Botany
https://www.readbyqxmd.com/read/28922704/direct-acting-antivirals-for-chronic-hepatitis-c
#3
REVIEW
Janus C Jakobsen, Emil Eik Nielsen, Joshua Feinberg, Kiran Kumar Katakam, Kristina Fobian, Goran Hauser, Goran Poropat, Snezana Djurisic, Karl Heinz Weiss, Milica Bjelakovic, Goran Bjelakovic, Sarah Louise Klingenberg, Jian Ping Liu, Dimitrinka Nikolova, Ronald L Koretz, Christian Gluud
BACKGROUND: Millions of people worldwide suffer from hepatitis C, which can lead to severe liver disease, liver cancer, and death. Direct-acting antivirals (DAAs), e.g. sofosbuvir, are relatively new and expensive interventions for chronic hepatitis C, and preliminary results suggest that DAAs may eradicate hepatitis C virus (HCV) from the blood (sustained virological response). Sustained virological response (SVR) is used by investigators and regulatory agencies as a surrogate outcome for morbidity and mortality, based solely on observational evidence...
September 18, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28922692/phosphodiesterase-4-inhibitors-for-chronic-obstructive-pulmonary-disease
#4
REVIEW
Jimmy Chong, Bonnie Leung, Phillippa Poole
BACKGROUND: Chronic obstructive pulmonary disease (COPD) is associated with cough, sputum production or dyspnoea and a reduction in lung function, quality of life and life expectancy. Apart from smoking cessation, there are no other treatments that slow lung function decline. Roflumilast and cilomilast are oral phosphodiesterase 4 (PDE4) inhibitors proposed to reduce the airway inflammation and bronchoconstriction seen in COPD. This is an update of a Cochrane review first published in 2011 and updated in 2013...
September 19, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28922609/serum-proteomic-variability-associated-with-clinical-phenotype-in-familial-transthyretin-amyloidosis-attrm
#5
Gloria G Chan, Clarissa M Koch, Lawreen H Connors
Transthyretin (TTR), normally a plasma circulating protein, can become misfolded and aggregated, ultimately leading to extracellular deposition of amyloid fibrils usually targeted to heart or nerve tissues. Referred to as TTR-associated amyloidoses (ATTR), this group of diseases is frequently life threatening and fatal if untreated. ATTR, caused by amyloid-forming variant TTR proteins (ATTRm) which arise from point mutations in the TTR gene, were classically referred to as familial amyloid cardiomyopathy (FAC) or familial amyloid polyneuropathy (FAP) reflecting the clinical phenotype...
September 18, 2017: Journal of Proteome Research
https://www.readbyqxmd.com/read/28922593/cysteine-silac-mass-spectrometry-enables-the-identification-and-quantitation-of-scrambled-inter-chain-disulfide-bonds-preservation-of-native-heavy-light-chain-pairing-in-bispecific-iggs-generated-by-controlled-fab-arm-exchange
#6
Ewald T J van den Bremer, Aran F Labrijn, Ramon van den Boogaard, Patrick Priem, Kai Scheffler, Joost P M Melis, Janine Schuurman, Paul Parren, Rob N de Jong
Bispecific antibodies (bsAbs) are one of the most versatile and promising pharmaceutical innovations for countering heterogeneous and refractory disease by virtue of their ability to bind two distinct antigens. One critical quality attribute of bsAb formation requiring investigation is the potential randomization of cognate heavy chain (H)/light (L) chain pairing, which could occur to a varying extent dependent on bsAb format and the production platform. To assess the content of such HL chain-swapped reaction products with high sensitivity, we developed cysteine-SILAC, a method that facilitates the detailed characterization of disulfide-bridged peptides by mass-spectrometry...
September 18, 2017: Analytical Chemistry
https://www.readbyqxmd.com/read/28922566/streptococcus-pneumoniae-and-chronic-endobronchial-infections-in-childhood
#7
Kim M Hare, Amanda J Leach, Heidi C Smith-Vaughan, Anne B Chang, Keith Grimwood
Streptococcus pneumoniae (pneumococcus) is the main cause of bacterial pneumonia worldwide and has been studied extensively in this context. However, its role in chronic endobronchial infections and accompanying lower airway neutrophilic infiltration has received little attention. Severe and recurrent pneumonia are risk factors for chronic suppurative lung disease (CSLD) and bronchiectasis; the latter causes considerable morbidity and, in some populations, premature death in children and adults. Protracted bacterial bronchitis (PBB) is another chronic endobronchial infection associated with substantial morbidity...
September 18, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28922525/describing-the-quality-of-life-of-boys-with-haemophilia-in-china-results-of-a-multicentre-study-using-the-cho-klat
#8
L Tang, W Xu, C G Li, F Hou, X Q Feng, H Wang, X J Li, W L Li, J P Liu, L R Sun, S H Wang, J Jin, Q Fang, K H Luke, M C Poon, V S Blanchette, K Usuba, N L Young, R Wu
INTRODUCTION: The treatment of haemophilia varies across countries and across regions within some countries. Similar variation has been observed in health-related quality of life (HR-QoL). Relatively little is known about the HR-QoL of boys with haemophilia in China. AIM: The aim of this study was to describe the HR-QoL of boys with haemophilia in China using the Canadian Haemophilia Outcomes-Kids Life Assessment Tool (CHO-KLAT). METHODS: Boys (4-18 years of age) with haemophilia and their parents were enroled in a cross-sectional study...
September 18, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28922494/magnetic-nanotransducers-in-biomedicine
#9
Agostina Grillone, Gianni Ciofani
Owing to their abilities to identify diseased conditions, to modulate biological process, and to control cellular activities, magnetic nanoparticles have become one of the most popular nanomaterials exploited in the biomedical field. Targeted drug delivery, controlled drug release, hyperthermia treatments, imaging, and stimulation of several biological entities are just some of the several tasks that can be accomplished by taking advantage of magnetic nanoparticles in tandem with magnetic fields. The huge interest towards this class of nanomaterials rises from the possibility to physically drive their spatiotemporal localization inside the body, and to deliver an externally applied stimulation at a target site...
September 18, 2017: Chemistry: a European Journal
https://www.readbyqxmd.com/read/28922492/highly-sensitive-detection-of-protein-biomarkers-with-organic-electrochemical-transistors
#10
Ying Fu, Naixiang Wang, Anneng Yang, Helen Ka-Wai Law, Li Li, Feng Yan
The analysis of protein biomarkers is of great importance in the diagnosis of diseases. Although many convenient and low-cost electrochemical approaches have been extensively investigated, they are not sensitive enough in the detection of protein biomarkers with low concentrations in physiological environments. Here, this study reports a novel organic-electrochemical-transistor-based biosensor that can successfully detect cancer protein biomarkers with ultrahigh sensitivity. The devices are operated by detecting electrochemical activity on gate electrodes, which is dependent on the concentrations of proteins labeled with catalytic nanoprobes...
September 18, 2017: Advanced Materials
https://www.readbyqxmd.com/read/28922483/molecular-basis-of-oral-squamous-cell-carcinoma-in-young-patients-is-it-any-different-from-older-patients
#11
REVIEW
Sara Ferreira Dos Santos Costa, Peter A Brennan, Ricardo Santiago Gomez, Eduardo Rodrigues Fregnani, Alan Roger Santos-Silva, Manoela Domingues Martins, Gilberto de Castro-Junior, Siavash Rahimi, Felipe Paiva Fonseca
Oral squamous cell carcinoma (OSCC) is the commonest subtype of oral cancer, mainly affecting older patients. It used to be a rare disease among individuals younger than 40 years, but recently increased incidences in this age group are being reported worldwide. The pathogenesis of OSCC affecting young patients remains controversial and the well known etiological factors for oral cancer, tobacco and alcohol use, are believed to play a minor role in the carcinogenesis of the neoplasm, suggesting that the etiology and the molecular basis of OSCC may differ between younger and older patients...
September 18, 2017: Journal of Oral Pathology & Medicine
https://www.readbyqxmd.com/read/28922472/mirna-506-promotes-primary-biliary-cholangitis-like-features-in-cholangiocytes-and-immune-activation
#12
Oihane Erice, Patricia Munoz-Garrido, Javier Vaquero, Maria J Perugorria, Maite G Fernandez-Barrena, Elena Saez, Alvaro Santos-Laso, Ander Arbelaiz, Raul Jimenez-Agüero, Joaquin Fernandez-Irigoyen, Enrique Santamaria, Verónica Torrano, Arkaitz Carracedo, Meenakshisundaram Ananthanarayanan, Marco Marzioni, Jesus Prieto, Ulrich Beuers, Ronald P Oude Elferink, Nicholas F LaRusso, Luis Bujanda, Jose J G Marin, Jesus M Banales
Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease associated with autoimmune phenomena targeting intrahepatic bile duct cells (cholangiocytes). Although PBC etiopathogenesis still remains obscure, development of anti-mitochondrial auto-antibodies against pyruvate dehydrogenase complex-E2 (PDC-E2) is a common feature. MicroRNA (miR) dysregulation occurs in liver and immune cells of PBC patients, but their functional relevance is largely unknown. We previously reported that miR-506 is overexpressed in PBC cholangiocytes and directly targets both Cl(-) /HCO3(-) anion exchanger 2 (AE2) and type III inositol 1,4,5-trisphosphate receptor (InsP3R3), leading to cholestasis...
September 18, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28922459/therapeutic-plasma-exchange-in-the-treatment-of-complicated-plasmodium-falciparum-malaria-a-case-report
#13
Tsong-Yih Ou, Cheng-Yen Chuang, Chun-Da Chen, Chung-Yi Cheng
Severe falciparum malaria is associated with multiple organ dysfunction and a high rate of fatal outcome. Malaria is a world-wide disease in tropical areas through the bites of vector mosquitoes. Parasitic protozoans introduced by the mosquito's saliva to the blood travel to the liver then mature and reproduce. In humans, malaria is caused by Plasmodium falciparum, P. malariae, P. ovale, P. vivax, and P. knowlesi, and P. falciparum causes most deaths. Typical malaria symptoms include fever, chills, fatigue, headache, nausea, and vomiting...
September 18, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28922436/genetic-risk-factors-for-autoimmune-thyroid-disease-might-affect-the-susceptibility-to-and-modulate-the-progression-of-primary-biliary-cholangitis
#14
Aleksander Kuś, Magdalena Arłukowicz-Grabowska, Konrad Szymański, Ewa Wunsch, Małgorzata Milkiewicz, Rafał Płoski, Zakera Shums, Gary L Norman, Piotr Milkiewicz, Tomasz Bednarczuk, Marcin Krawczyk
BACKGROUND AND AIMS: Patients with primary biliary cholangitis (PBC) frequently suffer from extrahepatic autoimmune conditions, of which autoimmune thyroid disease (AITD) is one of the most common. Previous studies identified several genetic variants increasing the odds of developing AITD. Here we investigate whether AITD-associated polymorphisms might also play a role in the development and clinical course of PBC and PBC associated with AITD (PBC-AITD). METHODS: To this end, we prospectively recruited 230 patients with PBC and 421 healthy controls...
September 2017: Journal of Gastrointestinal and Liver Diseases: JGLD
https://www.readbyqxmd.com/read/28922426/a-dna-vaccine-for-crimean-congo-hemorrhagic-fever-protects-against-disease-and-death-in-two-lethal-mouse-models
#15
Aura R Garrison, Charles J Shoemaker, Joseph W Golden, Collin J Fitzpatrick, John J Suschak, Michelle J Richards, Catherine V Badger, Carolyn M Six, Jacqueline D Martin, Drew Hannaman, Marko Zivcec, Eric Bergeron, Jeffrey W Koehler, Connie S Schmaljohn
Crimean-Congo hemorrhagic fever virus (CCHFV) is a tick-borne virus capable of causing a severe hemorrhagic fever disease in humans. There are currently no licensed vaccines to prevent CCHFV-associated disease. We developed a DNA vaccine expressing the M-segment glycoprotein precursor gene of CCHFV and assessed its immunogenicity and protective efficacy in two lethal mouse models of disease: type I interferon receptor knockout (IFNAR-/-) mice; and a novel transiently immune suppressed (IS) mouse model. Vaccination of mice by muscle electroporation of the M-segment DNA vaccine elicited strong antigen-specific humoral immune responses with neutralizing titers after three vaccinations in both IFNAR-/- and IS mouse models...
September 18, 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/28922425/hypoxia-inducible-factor-1-alpha-as-a-therapeutic-target-for-primary-effusion-lymphoma
#16
Prabha Shrestha, David A Davis, Ravindra P Veeranna, Robert F Carey, Coralie Viollet, Robert Yarchoan
Primary effusion lymphoma (PEL) is an aggressive B-cell lymphoma with poor prognosis caused by Kaposi's sarcoma-associated herpesvirus (KSHV). Previous studies have revealed that HIF-1α, which mediates much of the cellular response to hypoxia, plays an important role in life cycle of KSHV. KSHV infection promotes HIF-1α activity, and several KSHV genes are in turn activated by HIF-1α. In this study, we investigated the effects of knocking down HIF-1α in PELs. We observed that HIF-1α knockdown in each of two PEL lines leads to a reduction in both aerobic and anaerobic glycolysis as well as lipid biogenesis, indicating that HIF-1α is necessary for maintaining a metabolic state optimal for growth of PEL...
September 18, 2017: PLoS Pathogens
https://www.readbyqxmd.com/read/28922420/stable-c-and-n-isotope-analysis-of-hair-suggest-undernourishment-as-a-factor-in-the-death-of-a-mummified-girl-from-late-19th-century-san-francisco-ca
#17
Jelmer W Eerkens, Bryna Hull, Jena Goodman, Angela Evoy, Joshua D Kapp, Sidra Hussain, Richard E Green
The chance discovery of a 1.5-3.5 years old mummified girl presents a unique opportunity to further our understanding of health and disease among children in 19th Century San Francisco. This study focuses on carbon and nitrogen stable isotope signatures in serial samples of hair that cover the last 14 months of her life. Results suggest an initial omnivorous diet with little input from marine resources or C4 plants. Around six months before death δ15N starts a steady increase, with a noticeable acceleration just two months before she died...
2017: PloS One
https://www.readbyqxmd.com/read/28922404/eco-epidemiological-analysis-of-rickettsial-seropositivity-in-rural-areas-of-colombia-a-multilevel-approach
#18
Juan C Quintero V, Luis E Paternina T, Alexander Uribe Y, Carlos Muskus, Marylin Hidalgo, Juliana Gil, Astrid Vanessa Cienfuegos G, Lisardo Osorio Q, Carlos Rojas A
Rickettsiosis is a re-emergent infectious disease without epidemiological surveillance in Colombia. This disease is generally undiagnosed and several deadly outbreaks have been reported in the country in the last decade. The aim of this study is to analyze the eco-epidemiological aspects of rickettsial seropositivity in rural areas of Colombia where outbreaks of the disease were previously reported. A cross-sectional study, which included 597 people living in 246 households from nine hamlets in two municipalities of Colombia, was conducted from November 2015 to January 2016...
September 18, 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/28922375/elevated-igg4-serum-levels-in-patients-with-cystic-fibrosis
#19
Axelle Clerc, Quitterie Reynaud, Stéphane Durupt, Colette Chapuis-Cellier, Raphaële Nové-Josserand, Isabelle Durieu, Jean Christophe Lega
OBJECTIVE: Serum immunoglobulin (Ig) G4 elevation has been associated with several pathological conditions other than IgG4-related disease (IgG4-RD). In cystic fibrosis (CF), an elevation of specific IgG4 has been associated with colonization and infection by Pseudomonas aeruginosa. IgG4 elevation may be a marker of chronic infection or inflammatory stimulation. The aim of this study was to explore the prevalence of elevated IgG4 levels in CF and its correlation with the major clinical and microbiological features found in CF patients...
2017: PloS One
https://www.readbyqxmd.com/read/28922370/ileocolic-resection-is-associated-with-increased-susceptibility-to-injury-in-a-murine-model-of-colitis
#20
Troy Perry, Michael Laffin, Richard N Fedorak, Aducio Thiesen, Bryan Dicken, Karen L Madsen
Ileocolic resection (ICR) is the most common intestinal resection performed for Crohn's disease, with recurrences commonly occurring at the site of the anastomosis. This study used an animal model of ICR in wild-type mice to examine immunologic changes that developed around the surgical anastomosis and how these changes impacted gut responses to minor acute injury. ICR was performed in adult 129S1/SvlmJ mice and results compared with mice receiving sham or no surgery. Dextran sodium sulfate was given either on post-operative day 9 or day 24 to evaluate immune responses in the intestine both immediately following surgery and after a period of healing...
2017: PloS One
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