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Inclusion body disease

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https://www.readbyqxmd.com/read/28527044/endocytic-vesicle-rupture-is-a-conserved-mechanism-of-cellular-invasion-by-amyloid-proteins
#1
William P Flavin, Luc Bousset, Zachary C Green, Yaping Chu, Stratos Skarpathiotis, Michael J Chaney, Jeffrey H Kordower, Ronald Melki, Edward M Campbell
Numerous pathological amyloid proteins spread from cell to cell during neurodegenerative disease, facilitating the propagation of cellular pathology and disease progression. Understanding the mechanism by which disease-associated amyloid protein assemblies enter target cells and induce cellular dysfunction is, therefore, key to understanding the progressive nature of such neurodegenerative diseases. In this study, we utilized an imaging-based assay to monitor the ability of disease-associated amyloid assemblies to rupture intracellular vesicles following endocytosis...
May 19, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28523201/sarcoidosis-is-it-a-possible-trigger-of-inclusion-body-myositis
#2
Ali Zakaria, Issam Turk, Kenneth Leung, Ana Capatina-Rata, Waseem Farra
Sarcoidosis is a multisystem disorder of unknown etiology, characterized pathologically by the presence of nonnecrotizing granulomatous inflammation in affected organs. Although skeletal muscle is involved in 50-80 percent of individuals with sarcoidosis, symptomatic myopathy has been shown to be a rare manifestation of the disease. Inclusion body myositis (IBM) is a rare acquired idiopathic inflammatory myopathy with the insidious onset of asymmetric and distal muscle weakness that characteristically involves the quadriceps, tibialis anterior, and forearm flexors...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/28522732/unbiased-proteomics-of-early-lewy-body-formation-model-implicates-active-microtubule-affinity-regulating-kinases-marks-in-synucleinopathies
#3
Michael X Henderson, Charlotte Hiu-Yan Chung, Dawn M Riddle, Bin Zhang, Ronald J Gathagan, Steven H Seeholzer, John Q Trojanowski, Virginia M Y Lee
Parkinson's disease (PD) patients progressively accumulate intracytoplasmic inclusions formed by misfolded α-synuclein known as Lewy bodies (LBs). LBs also contain other proteins that may or may not be relevant in the disease process. To identify proteins involved early in LB formation, we performed proteomic analysis of insoluble proteins in a primary neuron culture model of α-synuclein pathology. We identified proteins previously found in authentic LBs in PD as well as several novel proteins, including the microtubule affinity-regulating kinase 1 (MARK1), one of the most enriched proteins in this model of LB formation...
May 17, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28520460/the-influence-of-body-mass-index-on-outcomes-after-hip-arthroscopic-surgery-with-capsular-plication-for-the-treatment-of-femoroacetabular-impingement
#4
Bryan M Saltzman, Benjamin D Kuhns, Bryce Basques, Timothy Leroux, Jennifer Alter, Richard C Mather, Michael J Salata, Shane J Nho
BACKGROUND: It remains unknown how variations in body mass index (BMI) influence outcomes after primary hip arthroscopic surgery with capsular plication for femoroacetabular impingement (FAI). PURPOSE: To evaluate the effect that abnormal BMI (namely, overweight, obese, morbidly obese, and underweight) versus normal weight has on patient-reported clinical outcomes more than 2 years postoperatively from primary hip arthroscopic surgery with capsular plication by a single surgeon...
May 1, 2017: American Journal of Sports Medicine
https://www.readbyqxmd.com/read/28515291/differential-disease-susceptibility-in-experimentally-reptarenavirus-infected-boa-constrictors-and-ball-pythons
#5
Mark D Stenglein, David Sanchez-Migallon Guzman, Valentina E Garcia, Marylee L Layton, Laura L Hoon-Hanks, Scott M Boback, M Kevin Keel, Tracy Drazenovich, Michelle G Hawkins, Joseph L DeRisi
Inclusion body disease (IBD) is an infectious disease originally described in captive snakes. It has traditionally been diagnosed by the presence of large eosinophilic cytoplasmic inclusions, and is associated with neurological, gastrointestinal and lymphoproliferative disorders. Previously, we identified and established a culture system for a novel lineage of arenaviruses isolated from boa constrictors diagnosed with IBD. Although ample circumstantial evidence suggested that these viruses, now known as reptarenaviruses, cause IBD, there has been no formal demonstration of disease causality since their discovery...
May 17, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28512218/ubiquitin-and-atp-dependent-unfoldase-activity-of-p97-vcp%C3%A2-nploc4%C3%A2-ufd1l-is-enhanced-by-a-mutation-that-causes-multisystem-proteinopathy
#6
Emily E Blythe, Kristine C Olson, Vincent Chau, Raymond J Deshaies
p97 is a "segregase" that plays a key role in numerous ubiquitin (Ub)-dependent pathways such as ER-associated degradation. It has been hypothesized that p97 extracts proteins from membranes or macromolecular complexes to enable their proteasomal degradation; however, the complex nature of p97 substrates has made it difficult to directly observe the fundamental basis for this activity. To address this issue, we developed a soluble p97 substrate-Ub-GFP modified with K48-linked ubiquitin chains-for in vitro p97 activity assays...
May 16, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28508875/early-phase-clinical-trials-of-anticancer-agents-in-children-and-adolescents-an-itcc-perspective
#7
REVIEW
Lucas Moreno, Andrew D J Pearson, Xavier Paoletti, Irene Jimenez, Birgit Geoerger, Pamela R Kearns, C Michel Zwaan, Francois Doz, Andre Baruchel, Josef Vormoor, Michela Casanova, Stefan M Pfister, Bruce Morland, Gilles Vassal
In the past decade, the landscape of drug development in oncology has evolved dramatically; however, this paradigm shift remains to be adopted in early phase clinical trial designs for studies of molecularly targeted agents and immunotherapeutic agents in paediatric malignancies. In drug development, prioritization of drugs on the basis of knowledge of tumour biology, molecular 'drivers' of disease and a drug's mechanism of action, and therapeutic unmet needs are key elements; these aspects are relevant to early phase paediatric trials, in which molecular profiling is strongly encouraged...
May 16, 2017: Nature Reviews. Clinical Oncology
https://www.readbyqxmd.com/read/28505979/a-systematic-review-and-meta-analysis-of%C3%A2-prevalence-studies-of-sporadic-inclusion-body-myositis
#8
Aoife Callan, Gorana Capkun, Vijayalakshmi Vasanthaprasad, Rita Freitas, Merrilee Needham
BACKGROUND: Sporadic Inclusion Body Myositis (sIBM) is a rare and slowly progressive debilitating muscle disease with symptoms generally developing≥50 years of age. OBJECTIVE: To conduct a systematic review and meta-analysis of the prevalence of sIBM literature, including a methodological quality assessment of the selected papers. METHODS: A systematic search of Medline, Embase, Cochrane Database of Systematic Reviews and major Myositis and Neurological conferences was conducted...
May 6, 2017: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/28501472/prokaryotic-expression-of-a-codon-optimized-capsid-gene-from-duck-circovirus-and-its-application-to-an-indirect-elisa
#9
Cui Yang, Yu Xu, Renyong Jia, Pengfei Li, Lei Zhang, Mingshu Wang, Dekang Zhu, Shun Chen, Mafeng Liu, Zhongqiong Yin, Anchun Cheng
Duck circovirus (DuCV), as a causative agent of long-term immunosuppressive disease, has caused heavy damage to waterfowl breeding worldwide. In this study, the full-length Cap (capsid) gene of DuCV was expressed in Escherichia coli (E. coli) for the first time by optimizing the codons in its nuclear localization signal (NLS) regions. The recombinant protein was expressed as inclusion bodies, and the quantification of purified Cap protein could reach 0.29mgml(-1). Moreover, an indirect ELISA method (Cap-ELISA) was established based on the recombinant Cap protein...
May 10, 2017: Journal of Virological Methods
https://www.readbyqxmd.com/read/28500967/a-systematic-review-on-potential-mechanisms-of-minocycline-in-kidney-diseases
#10
REVIEW
Hamed Haghi-Aminjan, Mohammad Hossein Asghari, Mohammad Hadi Goharbari, Mohammad Abdollahi
Kidney diseases need specialized health care and still are a reason of death. There is a large body of evidence that indicates minocycline possesses some cytoprotective effects beside of antibacterial properties. In this review, we aimed to explain cytoprotective mechanisms and kidney protection of minocycline. In order to find the effects of minocycline on kidney diseases a systematic literature search was performed, according to the guidelines proposed at the PRISMA statement in the electronic databases, including: PubMed, Scopus, and Web of Science up to August 2016, using the term 'minocycline' combined either by 'kidney' or 'renal' and published in English language...
February 8, 2017: Pharmacological Reports: PR
https://www.readbyqxmd.com/read/28499424/ultrastructural-deposits-appearing-as-zebra-bodies-in-renal-biopsy-fabry-disease-comparative-case-reports
#11
Precil Diego Miranda de Menezes Neves, Juliana Reis Machado, Fabiano Bichuette Custódio, Maria Luíza Gonçalves Dos Reis Monteiro, Shigueo Iwamoto, Marlene Freire, Marisa França Ferreira, Marlene Antônia Dos Reis
BACKGROUND: Fabry Disease (FD) is a genetic disorder caused by alpha-galactosidase A deficiency. Certain drugs, such as hydroxychloroquine, can produce renal deposits that mimic morphological findings seen in FD, characterizing a type of drug-induced renal phospholipidosis. CASE PRESENTATION: Case 1: A 28-year-old female patient with systemic lupus erythematosus who had been using hydroxychloroquine for 14 months presented subnephrotic proteinuria. Renal biopsy showed deposits compatible with FD...
May 12, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28495066/salvage-pulmonary-resection-after-stereotactic-body-radiotherapy-a-feasible-and-safe-option-for-local-failure-in-selected-patients
#12
Mara B Antonoff, Arlene M Correa, Boris Sepesi, Quynh-Nhu Nguyen, Garrett L Walsh, Stephen G Swisher, Ara A Vaporciyan, Reza J Mehran, Wayne L Hofstetter, David C Rice
OBJECTIVE: For inoperable patients with pulmonary malignancy, stereotactic body radiotherapy is a reasonable therapeutic option. Despite good early tumor control, local failure occurs in up to 10% of patients by 3 years. Because management of local recurrence after stereotactic body radiotherapy is unclear, we evaluated use of surgery as a salvage option. METHODS: A retrospective review was conducted of consecutive patients from a single institution who underwent salvage resection of primary and metastatic pulmonary malignancies previously treated with stereotactic body radiotherapy...
April 14, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28493327/burden-of-illness-and-healthcare-resource-use-in-us-patients-with-sporadic-inclusion-body-myositis
#13
Gorana Capkun, Aoife Callan, Haijun Tian, Zhongyuan Wei, Changgeng Zhao, Neetu Agashivala, Victoria Barghout
INTRODUCTION: We analyzed the burden of illness of sporadic Inclusion Body Myositis (sIBM) patients and the costs to the healthcare system. METHODS: A retrospective cohort analysis of 333 sIBM patients aged ≥50 years was performed using United States claims data. sIBM patients were matched in a 1:5 ratio to randomly selected individuals with ≥1 healthcare encounter within the year of index date. RESULTS: sIBM patients presented with higher rates of disease- and muscle-related conditions, such as myalgia, myositis, muscle weakness, dysphagia, pneumonia and falls...
May 10, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28490476/socioeconomic-status-in-childhood-and-c-reactive-protein-in-adulthood-a-systematic-review-and-meta-analysis
#14
REVIEW
Richard S Liu, Allison E Aiello, Fiona K Mensah, Constantine E Gasser, Kuna Rueb, Billie Cordell, Markus Juonala, Melissa Wake, David P Burgner
BACKGROUND: Inflammation plays a central role in cardiometabolic disease and may represent a mechanism linking low socioeconomic status (SES) in early life and adverse cardiometabolic health outcomes in later life. Accumulating evidence suggests an association between childhood SES and adult inflammation, but findings have been inconsistent. METHODS: We conducted a systematic review and meta-analysis of observational studies to quantify the association between childhood (age <18 years) SES and the inflammatory marker C reactive protein (CRP) in adulthood...
May 10, 2017: Journal of Epidemiology and Community Health
https://www.readbyqxmd.com/read/28489776/is-c-reactive-protein-a-marker-of-obstructive-sleep-apnea-a-meta-analysis
#15
Kun Li, Peng Wei, Yanwen Qin, Yongxiang Wei
BACKGROUND: Obstructive sleep apnea (OSA) is a common disease, distinguished by recurrent episodes of upper airway obstruction during sleep, with an inflammatory component. C-reactive protein (CRP) and high-sensitivity C-reactive protein (hs-CRP) are markers of systemic inflammation and may serve as biomarkers of OSA. METHODS: Scientific studies published from January 1, 2006, to January 1, 2016 were obtained via searches of PubMed, Embase, SCI, and China National Knowledge Internet (CNKI) using relevant terms...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28487381/improved-microrna-suppression-by-wpre-linked-tough-decoy-microrna-sponges
#16
Anne Kruse Hollensen, Rune Thomsen, Rasmus O Bak, Charlotte Christie Petersen, Eva Reumert Ermegaard, Lars Aagaard, Christian Kroun Damgaard, Jacob Giehm Mikkelsen
Our genes are posttranscriptionally regulated by microRNAs (miRNAs) inducing translational suppression and degradation of targeted mRNAs. Strategies to inhibit miRNAs in a spatiotemporal manner in a desired cell type or tissue, or at a desired developmental stage, can be crucial for understanding miRNA function and for pushing forward miRNA suppression as a feasible rationale for genetic treatment of disease. For such purposes, RNA polymerase II (RNApolII)-transcribed Tough Decoy (TuD) miRNA inhibitors are particularly attractive...
May 9, 2017: RNA
https://www.readbyqxmd.com/read/28486622/motivational-interviewing-by-general-practitioners-for-type-2-diabetes-patients-a-systematic-review
#17
Isaraporn Thepwongsa, Radhakrishnan Muthukumar, Pattapong Kessomboon
Background.: Motivational interviewing (MI) is an effective tool to help clinicians with facilitating behavioural changes in many diseases and conditions. However, different forms of MI are required in different health care settings and for different clinicians. Although general practitioners (GPs) play a major role in Type 2 diabetes management, the effects of MI delivered by GPs intended to change the behaviours of their Type 2 diabetes patients and GP outcomes, defined as GP knowledge, satisfaction and practice behaviours, have not been systematically reviewed...
May 9, 2017: Family Practice
https://www.readbyqxmd.com/read/28486373/medical-and-obstetric-complications-among-pregnant-women-with-liver-cirrhosis
#18
Anna Palatnik, Mary E Rinella
OBJECTIVE: To evaluate clinical characteristics and obstetric outcomes in pregnant women with liver cirrhosis. METHODS: This was a retrospective matched cohort study of women with liver cirrhosis between January 2005 and January 2016 in a university hospital. Women in a case group were matched to women in a control group according to year of delivery, age, body mass index, and parity in a 1:4 ratio. Bivariable and multivariable analyses were performed to compare the prevalence of the primary composite outcome, which included any one of the following: fetal or neonatal demise, placental abruption, preeclampsia, preterm delivery at less than 37 weeks of gestation, and small-for-gestational age neonate between women in the case group and those in the control group...
May 5, 2017: Obstetrics and Gynecology
https://www.readbyqxmd.com/read/28480235/do-positive-anaerobic-culture-results-affect-physicians-clinical-management-decisions
#19
Tanaya Bhowmick, Tilly A Varughese, Schweta Arakali, Susan E Boruchoff
BACKGROUND: Aerobic and anaerobic cultures from body fluids, abscesses, and wounds are ordered routinely. Prior studies have shown that the results of anaerobic blood cultures do not frequently lead to changes in patient management. METHODS: We performed a retrospective chart review to determine whether positive results of anaerobic tissue and fluid cultures (excluding blood) affect physicians' treatment approaches. Of 3234 anaerobic cultures, 174 unique patient admissions had positive cultures and met inclusion criteria...
2017: Open Forum Infectious Diseases
https://www.readbyqxmd.com/read/28476121/menopausal-hormone-therapy-a-systematic-review-of-cost-effectiveness-evaluations
#20
Louiza S Velentzis, Usha Salagame, Karen Canfell
BACKGROUND: Several evaluations of the cost-effectiveness (CE) of menopausal hormone therapy (MHT) have been reported. The aim of this study was to systematically and critically review economic evaluations of MHT since 2002, after the Women's Health Initiative (WHI) trial results on MHT were published. METHODS: The inclusion criteria for the review were: CE analyses of MHT versus no treatment, published from 2002-2016, in healthy women, which included both symptom relief outcomes and a range of longer term health outcomes (breast cancer, coronary heart disease, stroke, fractures and colorectal cancer)...
May 5, 2017: BMC Health Services Research
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