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Inclusion body disease

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https://www.readbyqxmd.com/read/28440890/fluorescence-and-autoradiographic-evaluation-of-tau-pet-ligand-pbb3-to-%C3%AE-synuclein-pathology
#1
Shunsuke Koga, Maiko Ono, Naruhiko Sahara, Makoto Higuchi, Dennis W Dickson
BACKGROUND: The tau PET ligand 2-((1E,3E)-4-(6-([(11) C]methylamino)pyridin-3-yl)buta-1,3-dienyl)benzo[d]thiazol-6-ol ([(11) C]PBB3) binds to a wide range of tau pathology; however, binding property of PBB3 to non-tau inclusions remains unknown. To clarify whether [(11) C]PBB3 binds to α-synuclein pathology, reactivity of PBB3 was assessed by in vitro fluorescence and autoradiographic labeling of brain sections from α-synucleinopathies patients. METHOD: Of 10 pure Lewy body disease and 120 multiple system atrophy (MSA) cases in the Mayo Clinic brain bank, we selected 3 Lewy body disease and 4 MSA cases with a range of α-synuclein severity based on the quantitative analysis of α-synuclein burden...
April 25, 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28440839/refugee-health-and-rehabilitation-challenges-and-response
#2
Fary Khan, Bhasker Amatya
BACKGROUND: The current global refugee crisis poses major challenges in providing effective healthcare to refugees, particularly for non-communicable diseases (NCDs) management and disability. This article provides an overview of refugee health and potential challenges from the rehabilitation perspective. METHODS: A literature search (both academic and grey literature) was conducted using medical and health science electronic databases and internet search engines (2001-2016)...
April 21, 2017: Journal of Rehabilitation Medicine
https://www.readbyqxmd.com/read/28439627/nigral-injection-of-a-proteasomal-inhibitor-lactacystin-induces-widespread-glial-cell-activation-and-shows-various-phenotypes-of-parkinson-s-disease-in-young-and-adult-mouse
#3
Mari H Savolainen, Katrina Albert, Mikko Airavaara, Timo T Myöhänen
Proteinaceous inclusions, called Lewy bodies, are used as a pathological hallmark for Parkinson's disease (PD). Lewy bodies contain insoluble α-synuclein (aSyn) and many other ubiquitinated proteins, suggesting a role for protein degradation system failure in the PD pathogenesis. Indeed, proteasomal dysfunction has been linked to PD but commonly used in vivo toxin models, such as 6-OHDA or MPTP, do not have a significant effect on the proteasomal system or protein aggregation. Therefore, we wanted to study the characteristics of a proteasomal inhibitor, lactacystin, as a PD model on young and adult mice...
April 24, 2017: Experimental Brain Research. Experimentelle Hirnforschung. Expérimentation Cérébrale
https://www.readbyqxmd.com/read/28439062/a-rare-case-of-necrotizing-myopathy-and-fibrinous-and-organizing-pneumonia-with-anti-ej-antisynthetase-syndrome-and-ssa-antibodies
#4
Muhammad Kashif, Divya Arya, Masooma Niazi, Misbahuddin Khaja
BACKGROUND Idiopathic inflammatory myopathies are autoimmune disorders that can involve the skin, joints, muscles, and lungs. The most common of these disorders are dermatomyositis, polymyositis, overlap syndrome, and inclusion body myositis. Necrotizing autoimmune myopathy is an idiopathic inflammatory myopathy that is rarely associated with Sjögren's syndrome. The most common lung findings associated with anti-EJ antisynthetase syndrome are nonspecific interstitial pneumonia and usual interstitial pneumonia; this condition is rarely associated with fibrinous and organizing pneumonia...
April 25, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28431575/heterogeneous-ribonuclear-protein-a3-hnrnp-a3-is-present-in-dipeptide-repeat-protein-containing-inclusions-in-frontotemporal-lobar-degeneration-and-motor-neurone-disease-associated-with-expansions-in-c9orf72-gene
#5
Yvonne S Davidson, Louis Flood, Andrew C Robinson, Yoshihiro Nihei, Kohji Mori, Sara Rollinson, Anna Richardson, Bridget C Benson, Matthew Jones, Julie S Snowden, Stuart Pickering-Brown, Christian Haass, Tammaryn Lashley, David M A Mann
Frontotemporal Lobar Degeneration (FTLD) encompasses certain related neurodegenerative disorders which alter behaviour, personality and language. Heterogeneous ribonuclear proteins (hnRNPs) maintain RNA metabolism and changes in their function may underpin the pathogenesis of FTLD. Immunostaining for hnRNP A1, A2/B1 and A3 was performed on sections of temporal cortex with hippocampus from 61 patients with FTLD, stratified by pathological hallmarks into FTLD-tau and FTLD-TDP type A, B and C subtypes, and by genetics into patients with C9orf72 expansions, MAPT or GRN mutations, or those without known mutation...
April 21, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28430289/evidence-from-spatial-pattern-analysis-for-the-anatomical-spread-of-%C3%AE-synuclein-pathology-in-parkinson-s-disease-dementia
#6
Richard A Armstrong
<i>The objective of this study was to determine whether there is evidence from quantitative morphometry and spatial pattern analysis to support the hypothesis of anatomical spread of -synuclein in Parkinson's disease dementia (PDD). Hence, clustering of -synuclein-immunoreactive Lewy bodies (LB), Lewy neurites (LN), and Lewy grains (LG) was studied in -synuclein-immunolabeled sections of cortical and limbic regions in 12 cases of PDD. The data suggested that: (1) LB, LN, and LG occurred in clusters which in 63% of regions were regularly distributed parallel to the tissue boundary, (2) in approximately 30% of cortical regions, the estimated cluster size of LB, LN, and LG was within the size range of cellular columns associated with the cortico-cortical pathways, (3) regularly distributed clusters were present in anatomically connected regions, and (4) the clustering pattern was similar to that of prion protein (PrPsc) deposits in Creutzfeldt-Jacob disease (CJD)...
2017: Folia Neuropathologica
https://www.readbyqxmd.com/read/28424665/ca-similichlamydia-in-epitheliocystis-co-infection-of-gilthead-seabream-gills-unique-morphological-features-of-a-deep-branching-chlamydial-family
#7
Helena M B Seth-Smith, Pantelis Katharios, Nancy Dourala, José M Mateos, Alexander G J Fehr, Lisbeth Nufer, Maja Ruetten, Maricruz Guevara Soto, Lloyd Vaughan
The Planctomycetes-Verrucomicrobia-Chlamydiae (PVC) bacterial superphylum constitutes a broad range of organisms with an intriguing array of ultrastructural morphologies, including intracellular membranes and compartments and their corresponding complex genomes encoding these forms. The phylum Chlamydiae are all obligate intracellular bacteria and, although much is already known of their genomes from various families and how these regulate the various morphological forms, we know remarkably little about what is likely the deepest rooting clade of this phylum, which has only been found to contain pathogens of marine and fresh water vertebrates...
2017: Frontiers in Microbiology
https://www.readbyqxmd.com/read/28424265/effects-of-altered-sialic-acid-biosynthesis-on-n-linked-glycan-branching-and-cell-surface-interactions
#8
Nam D Pham, Poh-Choo Pang, Soumya Krishnamurthy, Amberlyn M Wands, Paola Grassi, Anne Dell, Stuart M Haslam, Jennifer J Kohler
GNE myopathy is a rare muscle disorder associated with aging and is related to sporadic inclusion body myositis (sIBM), the most common acquired muscle disease of aging. While the cause of sIBM is unknown, GNE myopathy is associated with mutations in UDP-GlcNAc 2-epimerase/ManNAc kinase (GNE). GNE harbors two enzymatic activities required for biosynthesis of sialic acid in mammalian cells. Mutations to both GNE domains are linked to GNE myopathy. However, correlation between mutation-associated reductions in sialic acid production and disease severity is imperfect...
April 19, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28421482/cost-effectiveness-of-stereotactic-radiosurgery-and-stereotactic-body-radiation-therapy-a-critical-review
#9
REVIEW
Nataniel H Lester-Coll, David J Sher
PURPOSE OF REVIEW: This review aims to summarize and appraise published cost-effectiveness studies on stereotactic radiosurgery (SRS) and stereotactic body radiation therapy (SBRT). RECENT FINDINGS: We performed a Medline search of cost-effectiveness studies of SRS, SBRT, and other cancer treatment modalities such as surgery and systemic therapy from 2006 to 2016. We included studies that used both modeling and retrospective review techniques. We excluded studies of benign disease...
June 2017: Current Oncology Reports
https://www.readbyqxmd.com/read/28408155/arthroscopic-capsular-plication-and-labral-seal-restoration-in-borderline-hip-dysplasia-2-year-clinical-outcomes-in-55-cases
#10
Sivashankar Chandrasekaran, Nader Darwish, Timothy J Martin, Carlos Suarez-Ahedo, Parth Lodhia, Benjamin G Domb
PURPOSE: To report clinical outcomes in patients with borderline dysplasia undergoing an arthroscopic technique of labral seal restoration with minimal acetabular rim resection and capsular plication. METHODS: Patients younger than 40 years with a lateral center-edge angle of 18° greater and 25° or less and 2-year follow-up after undergoing an arthroscopic technique of labral seal restoration with minimal rim resection (≤2 mm) and capsular plication (3-5 sutures placed in an oblique orientation to create an imbrication and inferior shift) were included...
April 10, 2017: Arthroscopy: the Journal of Arthroscopic & related Surgery
https://www.readbyqxmd.com/read/28406788/improving-patient-centered-care-by-assessing-patient-preferences-for-multiple-sclerosis-disease-modifying-agents-a-stated-choice-experiment
#11
Caroline S Carlin, Lucas Higuera, Sarah Anderson
CONTEXT: Long-term adherence to pharmaceutical treatment for multiple sclerosis (MS) is poor. A focus on patient preferences when determining the patient's therapeutic plan may improve this experience. OBJECTIVE: To identify factors important to patients with MS when evaluating their options for pharmaceutical agents that deliver disease-modifying therapy. DESIGN: Stated-choice experiment to a sample of patients with MS from privately and publicly insured enrollees in a regional health plan...
2017: Permanente Journal
https://www.readbyqxmd.com/read/28396410/spinal-motor-neuron-protein-supersaturation-patterns-are-associated-with-inclusion-body-formation-in-als
#12
Prajwal Ciryam, Isabella A Lambert-Smith, Daniel M Bean, Rosie Freer, Fernando Cid, Gian Gaetano Tartaglia, Darren N Saunders, Mark R Wilson, Stephen G Oliver, Richard I Morimoto, Christopher M Dobson, Michele Vendruscolo, Giorgio Favrin, Justin J Yerbury
Amyotrophic lateral sclerosis (ALS) is a heterogeneous degenerative motor neuron disease linked to numerous genetic mutations in apparently unrelated proteins. These proteins, including SOD1, TDP-43, and FUS, are highly aggregation-prone and form a variety of intracellular inclusion bodies that are characteristic of different neuropathological subtypes of the disease. Contained within these inclusions are a variety of proteins that do not share obvious characteristics other than coaggregation. However, recent evidence from other neurodegenerative disorders suggests that disease-affected biochemical pathways can be characterized by the presence of proteins that are supersaturated, with cellular concentrations significantly greater than their solubilities...
April 10, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28396209/immunogenicity-and-protective-efficacy-of-virus-like-particles-and-recombinant-fiber-proteins-in-broiler-breeder-vaccination-against-fowl-adenovirus-fadv-8b
#13
Ashish Gupta, Khawaja Ashfaque Ahmed, Lisanework E Ayalew, Shelly Popowich, Shanika Kurukulasuriya, Kalhari Goonewardene, Thushari Gunawardana, Ruwani Karunarathna, Davor Ojkic, Suresh K Tikoo, Philip Willson, Susantha Gomis
Inclusion body hepatitis (IBH) is an economically important diseases in broiler chicken industry. Several serotypes of fowl adenovirus (FAdV) can cause IBH, among them, serotype FAdV-8b is associated with the majority of the IBH cases in Canada. Here, we evaluated FAdV-8b virus-like particles (VLPs) and recombinant FAdV-8b fiber proteins (expressed in E. coli) as potential broiler-breeder vaccines against IBH. For assessing the immunogenicity of vaccines, we investigated both humoral and cellular immunity. The humoral immune response was evaluated by determining total IgY and virus-neutralizing antibody in serum at 14, 28, 35 and 60days post-immunization (dpi)...
May 9, 2017: Vaccine
https://www.readbyqxmd.com/read/28396162/phase-i-study-of-clofarabine-and-2-gy-total-body-irradiation-as-a-non-myeloablative-preparative-regimen-for-hematopoietic-stem-cell-transplantation-hsct-in-pediatric-patients-with-hematologic-malignancies-a-therapeutic-advances-in-childhood-leukemia-tacl-consortium
#14
Sandeep Soni, Hisham Abdel-Azim, Meghann McManus, Eneida Nemecek, Richard Sposto, Ann Woolfrey, Haydar Frangoul
Clofarabine is a purine nucleoside analog with immunosuppressive and anti-leukemic activity and its inclusion in reduced intensity regimens could potentially improve outcomes. We performed a prospective Phase I study of clofarabine combined with 2 Gy total body irradiation (TBI) as a non-myeloablative (NMA) preparative regimen for allogeneic stem cell transplantation in pediatric patients, who were considered at high risk of mortality from standard myeloablative regimens. The main goal of the study was to delineate the maximum feasible dose (MFD) of clofarabine in combination with 2 Gy TBI...
April 7, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28395233/the-effect-of-obesity-on-pathological-complete-response-and-survival-in-breast-cancer-patients-receiving-uncapped-doses-of-neoadjuvant-anthracycline-taxane-based-chemotherapy
#15
Alex Farr, Myriam Stolz, Lukas Baumann, Zsuzsanna Bago-Horvath, Elisabeth Oppolzer, Georg Pfeiler, Michael Seifert, Christian F Singer
PURPOSE: The effect of obesity in breast cancer patients undergoing neoadjuvant chemotherapy (NAC) remains controversial. The aim of this study was to determine the obesity-related effect on pathological complete response (pCR) and survival in women receiving full uncapped doses of NAC. METHODS: We retrospectively analyzed the data of all consecutive women who underwent anthracycline-taxane-based NAC for primary breast cancer between 2005 and 2015 at the Department of Obstetrics and Gynecology, Medical University of Vienna...
April 7, 2017: Breast: Official Journal of the European Society of Mastology
https://www.readbyqxmd.com/read/28389692/familial-early-onset-paget-s-disease-of-bone-associated-with-a-novel-hnrnpa2b1-mutation
#16
Xuan Qi, Qianqian Pang, Jiawei Wang, Zhen Zhao, Ou Wang, Lijun Xu, Jiangfeng Mao, Yan Jiang, Mei Li, Xiaoping Xing, Wei Yu, Asan, Weibo Xia
Paget disease of bone (PDB) is a common metabolic bone disease characterized by increased bone resorption and disorganized bone formation which affect single or multiple sites of bones. Although the exact cause of PDB is still controversial, genetic factors are considered to play an important role in PDB. Several genes involved in the differentiation or function of osteoclast were shown to be associated with PDB or related syndrome such as SQSTM1, TNFRSF11A, TNFRSF11B, and ZNF687. Multisystem proteinopathy (MSP), a newly proposed syndrome including inclusion body myopathy (IBM), PDB, frontotemporal dementia (FTD), and amyotrophic lateral sclerosis (ALS), is mainly caused by mutation in VCP gene...
April 7, 2017: Calcified Tissue International
https://www.readbyqxmd.com/read/28388919/scope-and-quality-of-cochrane-reviews-of-nutrition-interventions-a-cross-sectional-study
#17
Celeste E Naude, Solange Durao, Abigail Harper, Jimmy Volmink
BACKGROUND: All countries face significant challenges from complex manifestations of malnutrition, which affects one in three people globally. Systematic reviews provide ready-to-use syntheses of quality-appraised evidence to inform decision-making for actions. To enhance the utility and quality of future Cochrane nutrition evidence, we described the scope and quality of all nutrition systematic reviews in the Cochrane Database of Systematic Reviews (CDSR). METHODS: We screened all active CDSR records (31 July 2015) to identify reviews and protocols using pre-specified eligibility criteria and definitions...
April 7, 2017: Nutrition Journal
https://www.readbyqxmd.com/read/28386933/novel-conformation-selective-alpha-synuclein-antibodies-raised-against-different-in-vitro-fibril-forms-show-distinct-patterns-of-lewy-pathology-in-parkinson-s-disease
#18
Dustin J Covell, John L Robinson, Rizwan S Akhtar, Murray Grossman, Daniel Weintraub, Hannah M Bucklin, Rose M Pitkin, Dawn Riddle, Ahmed Yousef, John Q Trojanowski, Virginia M-Y Lee
AIMS: The aim of this study was to test the hypothesis that different conformations of misfolded α-synuclein (α-syn) are present in Parkinson's disease (PD) brain. METHODS: Using two previously characterized conformations of α-syn fibrils, we generated new conformation-selective, α-syn monoclonal antibodies (mAbs). We then interrogated multiple brain regions in a well-characterized autopsy cohort of PD patients (n = 49) with these mAbs, Syn7015 and Syn9029. RESULTS: Syn7015 detects Lewy bodies (LBs) and Lewy neurites (LNs) formed by pathological α-syn in all brain regions tested, and is particularly sensitive to LNs and small Lewy dots, inclusions believed to form early in the disease...
April 7, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28384112/inflammatory-myopathies-with-cutaneous-involvement-from-diagnosis-to-therapy
#19
Lyubomir A Dourmishev
The group of idiopathic inflammatory myopathies (IIM) include various disorders of skeletal muscles with or without skin involvement. The most common types are dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) and necrotizing autoimmune myopathy (NAM). Dermatomyositis subdivides into various clinical forms such as juvenile, amyopathic or paraneoplastic dermatomyositis, scleromyositis, overlap or anti-synthetase syndromes, etc. Recently, numerous new antibodies defining the characteristic clinical phenotype have been described as anti-MDA5 antibodies associated with interstitial lung disease and amyopathic dermatomyositis or anti-TIF1γ antibodies as markers for paraneoplastic dermatomyositis...
March 1, 2017: Folia Medica
https://www.readbyqxmd.com/read/28384060/genetic-characterization-of-h13-and-h16-influenza-a-viruses-in-gulls-larus-spp-with-clinically-severe-disease-and-concurrent-circovirus-infection
#20
Erika Lindh, Christine Ek-Kommonen, Marja Isomursu, Jukka Alasaari, Antti Vaheri, Olli Vapalahti, Anita Huovilainen
Influenza A viruses (IAVs) of the subtypes H13 and H16 are primarily found in gulls ( Larus spp., order Charadriiformes). Although the gull-adapted subtypes replicate efficiently during infection, gulls usually remain apparently healthy during infection. Avian influenza virus isolates are generally separated into two distinct populations, North American and Eurasian, because of the limited gene flow between the continents. Reassortment between these lineages does occur occasionally; however, direct intercontinental transmission of all eight gene segments is rare...
April 6, 2017: Journal of Wildlife Diseases
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