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https://www.readbyqxmd.com/read/27925304/dura-mater-graft-associated-creutzfeldt-jakob-disease-with-30-year-incubation-period
#1
Masahiro Shijo, Hiroyuki Honda, Sachiko Koyama, Koji Ishitsuka, Koichiro Maeda, Junya Kuroda, Mitsugu Tanii, Takanari Kitazono, Toru Iwaki
Over 60% of all patients with dura mater graft-associated Creutzfeldt-Jakob disease (dCJD) have been diagnosed in Japan. The incubation period has ranged from 1 to 30 years and the age at onset from 15 to 80 years. Here, we report a 77-year-old male Japanese autopsied dCJD case with the longest incubation period so far in Japan. He received a cadaveric dural graft at the right cranial convexity following a craniotomy for meningioma at the age of 46. At 30 years post-dural graft placement, disorientation was observed as an initial symptom of dCJD...
December 7, 2016: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/27913587/suppressing-n-acetyl-l-aspartate-naa-synthesis-prevents-loss-of-neurons-in-a-murine-model-of-canavan-leukodystrophy
#2
Jiho Sohn, Peter Bannerman, Fuzheng Guo, Travis Burns, Laird Miers, Christopher Croteau, Naveen K Singhal, Jennifer A McDonough, David Pleasure
Canavan disease is a leukodystrophy caused by aspartoacylase (ASPA) deficiency. The lack of functional ASPA, an enzyme enriched in oligodendroglia that cleaves N-acetyl-L-aspartate (NAA) to acetate and L-aspartic acid, elevates brain NAA and causes "spongiform" vacuolation of superficial brain white matter and neighboring gray matter. In children with Canavan disease, neuroimaging shows early-onset dysmyelination and progressive brain atrophy. Neuron loss has been documented at autopsy in some cases. Prior studies have shown that mice homozygous for the Aspa nonsense mutation Nur7 also develop brain vacuolation...
December 2, 2016: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/27892798/transmissibility-of-gerstmann-str%C3%A3-ussler-scheinker-syndrome-in-rodent-models-new-insights-into-the-molecular-underpinnings-of-prion-infectivity
#3
Romolo Nonno, Michele Angelo Di Bari, Umberto Agrimi, Laura Pirisinu
Prion diseases, or transmissible spongiform encephalopathies, have revealed the bewildering phenomenon of transmissibility in neurodegenerative diseases. Hence, the experimental transmissibility of prion-like neurodegenerative diseases via template directed misfolding has become the focus of intense research. Gerstmann-Sträussler-Scheinker disease (GSS) is an inherited prion disease associated with mutations in the prion protein gene. However, with the exception of a few GSS cases with P102L mutation characterized by co-accumulation of protease-resistant PrP core (PrP(res)) of ∼21 kDa, attempts to transmit to rodents GSS associated to atypical misfolded prion protein with ∼8 kDa PrP(res) have been unsuccessful...
November 28, 2016: Prion
https://www.readbyqxmd.com/read/27885854/gossypibomas-a-surgeon-s-nightmare-patient-demographics-risk-factors-imaging-and-how-we-can-prevent-it
#4
Rishi Philip Mathew, Binston Thomas, Ram Shenoy Basti, Hadihally B Suresh
OBJECTIVES: Gossypibomas are cottinoid matrix left behind following surgery. Due to the legal issues associated with it, very few literature studies are available online, most of them being case reports. The purpose of our study was to identify the patient demographics, risk factors and imaging features. METHODS: Six surgically identified and histopathologically confirmed cases of gossypibomas recorded over a period 7 years from a single tertiary institution were retrospectively evaluated for patient demographics (sex, age, BMI), type of surgery, and duration from time of surgery to onset of symptoms...
November 25, 2016: British Journal of Radiology
https://www.readbyqxmd.com/read/27870787/can-nodular-hyperplasia-of-the-thyroid-gland-be-differentiated-from-follicular-adenoma-and-follicular-carcinoma-by-ultrasonography
#5
Sun Hye Jeong, Hyun Sook Hong, Eun Hye Lee
OBJECTIVES: The aim of this study was to evaluate the ultrasonographic features for differentiation of follicular thyroid lesions. METHODS: Ultrasonographic features of surgically confirmed 56 follicular adenoma (FA), 22 follicular carcinoma (FC), and 100 nodular hyperplasia (NH) were evaluated using univariable and multivariable multinomial logistic regression analyses, receiver operating characteristic analyses, and areas under the curve. RESULTS: Tumor diameter, margin, echotexture, cystic changes, calcification, hypoechoic rim, and vascularity were significant on univariable analysis...
December 2016: Ultrasound Quarterly
https://www.readbyqxmd.com/read/27852825/exosomes-in-the-pathology-of-neurodegenerative-diseases
#6
Jason Howitt, Andrew Francis Hill
More than thirty years ago two precipitate findings were discovered that challenged conventional thinking in biology. The first was the identification of a misfolded protein with transmissible properties associated with a group of neurodegenerative diseases known as transmissible spongiform encephalopathies. The second was the discovery of a new pathway used for the extracellular release of biomolecules, including extracellular vesicles call exosomes. Two decades later, convergence of these pathways was shown when exosomes were found to play a significant role in both the transmission and propagation of protein aggregates in disease...
November 16, 2016: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/27843909/melanoma-associated-spongiform-scleropathy-in-oculodermal-melanocytosis-with-primary-orbital-melanoma
#7
Roshni U Ranjit, Ilya M Leyngold, Curtis E Margo
PURPOSE: To describe spongiform scleropathy in a patient with oculodermal melanosis and without evidence of uveal melanoma. METHODS: Clinical-pathological correlation conducted in compliance with HIPPA (Health Insurance Privacy and Portability Act) regulations. RESULTS: Melanoma-associated spongiform scleropathy was an incidental finding in an 87-year-old woman with oculodermal melanocytosis treated for primary orbital melanoma. All previously reported cases of this scleropathy have been associated with uveal melanoma...
October 2016: Ocular Oncology and Pathology
https://www.readbyqxmd.com/read/27833227/creutzfeldt-jakob-disease-phenotype-and-course-our-experience-from-a-tertiary-center
#8
Sadanandavalli Retnaswami Chandra, Thomas Gregor Issac, Mariamma Philip, Veeranna Gadad
INTRODUCTION: Prion diseases are protein conformation disorders and neither caused by viroid or virus but is a transmissible particle labeled a prion by Pruisner. Normal prion protein becomes infectious by a different folding, but the triggers are not known. Based on the characteristic brain pathology, they are grouped under spongiform encephalopathy affecting both man and animals. Estimated prevalence is one per million. Creutzfeldt-Jakob disease (CJD) registry from National Institute and Neurosciences (NIMHANS), Bengaluru, reported 69 cases in 30 years...
September 2016: Indian Journal of Psychological Medicine
https://www.readbyqxmd.com/read/27825366/l-bse-experimentally-transmitted-to-sheep-presents-as-a-unique-disease-phenotype
#9
Marion M Simmons, Melanie J Chaplin, Timm Konold, Cristina Casalone, Katy E Beck, Leigh Thorne, Sharon Everitt, Tobias Floyd, Derek Clifford, John Spiropoulos
Apart from prion protein genotype, the factors determining the host range and susceptiblity for specific transmissible spongiform encephalopathy agents remain unclear. It is known that bovine atypical L-BSE can transmit to a range of species including primates and humanised transgenic mice. It is important, therefore, that there is as broad an understanding as possible of how such isolates might present in food animal species and how robust they are on inter- and intra-species transmission to inform surveillance sytems and risk assessments...
November 8, 2016: Veterinary Research
https://www.readbyqxmd.com/read/27822596/investigation-of-the-prion-protein-gene-prnp-polymorphisms-in-anatolian-murrah-and-crossbred-water-buffaloes-bubalus-bubalis
#10
Yalçın Yaman, Orhan Karadağ, Cemal Ün
Bovine spongiform encephalopathy (BSE) of the cattle is the outstanding disease among other transmissible spongiform encephalopathy (TSEs). It can be transmitted from the cattle to a human and causes a new variant of the Creutzfeldt-Jakob disease (CJD). It is known that prion protein coding gene (PRNP) plays a major role in the TSE susceptibility or resistance in some species. Recent researches demonstrated that the insertion (in) and deletion (del) polymorphisms within promoter and intron 1 region of the PRNP related to BSE susceptibility in cattle...
November 8, 2016: Tropical Animal Health and Production
https://www.readbyqxmd.com/read/27809706/oxidative-stress-and-mitochondrial-dysfunction-linked-neurodegenerative-disorders
#11
Md Torequl Islam
Reactive species play an important role in physiological functions. Overproduction of reactive species, notably reactive oxygen (ROS) and nitrogen (RNS) species along with the failure of balance by the body's antioxidant enzyme systems results in destruction of cellular structures, lipids, proteins, and genetic materials such as DNA and RNA. Moreover, the effects of reactive species on mitochondria and their metabolic processes eventually cause a rise in ROS/RNS levels, leading to oxidation of mitochondrial proteins, lipids, and DNA...
November 3, 2016: Neurological Research
https://www.readbyqxmd.com/read/27807855/atypical-scrapie-in-australia
#12
R W Cook, J Bingham, A S Besier, C L Bayley, M Hawes, P L Shearer, M Yamada, J Bergfeld, D T Williams, D J Middleton
BACKGROUND: Since its initial detection in Norway in 1998, atypical scrapie ('atypical/Nor98 scrapie') has been reported in sheep in the majority of European countries (including in regions free of classical scrapie) and in the Falkland Islands, the USA, Canada, New Zealand and Australia. CASE SERIES: The diagnosis in Australia of atypical scrapie in four Merino and one Merino-cross sheep showing clinical signs of neurological disease was based on the detection of grey matter neuropil vacuolation (spongiform change) in the brain (particularly in the molecular layer of the cerebellar cortex) and associated abnormal prion protein (PrP(Sc) ) deposition in both grey and white matter...
November 3, 2016: Australian Veterinary Journal
https://www.readbyqxmd.com/read/27803245/modulation-of-prion-polymerization-and-toxicity-by-rationally-designed-peptidomimetics
#13
Ankit Srivastava, Sakshi Sharma, Sandhya Sadanandan, Sakshi Gupta, Jasdeep Singh, Sarika Gupta, V Haridas, Bishwajit Kundu
Misfolding and aggregation of cellular prion protein (PrP(C)) is associated with a large array of neurological disorders commonly called the Transmissible spongiform encephalopathies (TSEs). Designing inhibitors against prions has remained a daunting task owing to limited information about mechanism(s) of their pathogenic self-assembly. Here, we explore the anti-prion properties of a combinatorial library of bispidine-based peptidomimetics (BPMs) that conjugate amino acids with hydrophobic and aromatic side chains...
November 1, 2016: Biochemical Journal
https://www.readbyqxmd.com/read/27782310/reflectance-confocal-microscopy-for-the-evaluation-of-sensitive-skin
#14
Y-F Ma, C Yuan, W-C Jiang, X-L Wang, P Humbert
BACKGROUND: Nowadays, the diagnosis for sensitive skin relies on subjective assessment or on the combination of subjective and objective evaluation. No quantitative evaluation is available. It could be expected that confocal microscopy imaging could be of interest to better define the condition. METHODS: Total 166 healthy female subjects were recruited in this study. Firstly, all subjects completed the sensitive questionnaire. Then, the cutaneous structures were measured by the reflectance confocal microscopy (RCM) on the face and fossa cubitalia...
October 26, 2016: Skin Research and Technology
https://www.readbyqxmd.com/read/27780019/regulatory-issues-associated-with-preharvest-food-safety-european-union-perspective
#15
Lis Alban
Free movement of safe and wholesome food is an essential aspect of any society. This article contains an updated description of the regulatory issues associated with preharvest food safety within the European Union. Salmonella, Campylobacter, Trichinella, antimicrobial resistance, and bovine spongiform encephalopathy are dealt with in detail. Moreover, Cysticercus bovis/Taenia saginata, Toxoplasma, Yersinia, verotoxigenic/shigatoxigenic Escherichia coli, Listeria, and foodborne viruses are briefly covered. The article describes how the focus in the European Union is changing to involve a supply chain view with a focus on cost-effectiveness...
October 2016: Microbiology Spectrum
https://www.readbyqxmd.com/read/27726110/reelin-expression-in-creutzfeldt-jakob-disease-and-experimental-models-of-transmissible-spongiform-encephalopathies
#16
Agata Mata, Laura Urrea, Silvia Vilches, Franc Llorens, Katrin Thüne, Juan-Carlos Espinosa, Olivier Andréoletti, Alejandro M Sevillano, Juan María Torres, Jesús Rodríguez Requena, Inga Zerr, Isidro Ferrer, Rosalina Gavín, José Antonio Del Río
Reelin is an extracellular glycoprotein involved in key cellular processes in developing and adult nervous system, including regulation of neuronal migration, synapse formation, and plasticity. Most of these roles are mediated by the intracellular phosphorylation of disabled-1 (Dab1), an intracellular adaptor molecule, in turn mediated by binding Reelin to its receptors. Altered expression and glycosylation patterns of Reelin in cerebrospinal and cortical extracts have been reported in Alzheimer's disease...
October 10, 2016: Molecular Neurobiology
https://www.readbyqxmd.com/read/27717881/n-acetylaspartate-supports-the-energetic-demands-of-developmental-myelination-via-oligodendroglial-aspartoacylase
#17
Jeremy S Francis, Ireneusz Wojtas, Vladimir Markov, Steven J Gray, Thomas J McCown, R Jude Samulski, Larissa T Bilaniuk, Dah-Jyuu Wang, Darryl C De Vivo, Christopher G Janson, Paola Leone
Breakdown of neuro-glial N-acetyl-aspartate (NAA) metabolism results in the failure of developmental myelination, manifest in the congenital pediatric leukodystrophy Canavan disease caused by mutations to the sole NAA catabolizing enzyme aspartoacylase. Canavan disease is a major point of focus for efforts to define NAA function, with available evidence suggesting NAA serves as an acetyl donor for fatty acid synthesis during myelination. Elevated NAA is a diagnostic hallmark of Canavan disease, which contrasts with a broad spectrum of alternative neurodegenerative contexts in which levels of NAA are inversely proportional to pathological progression...
December 2016: Neurobiology of Disease
https://www.readbyqxmd.com/read/27716411/genetic-histochemical-and-biochemical-studies-on-goat-tse-cases-from-cyprus
#18
Susanne Niedermeyer, Martin Eiden, Pavlos Toumazos, Penelope Papasavva-Stylianou, Ioannis Ioannou, Theodoros Sklaviadis, Cynthia Panagiotidis, Jan Langeveld, Alex Bossers, Thorsten Kuczius, Martin Kaatz, Martin H Groschup, Christine Fast
Scrapie and bovine spongiform encephalopathy (BSE) are transmissible spongiform encephalopathies (TSE's) affecting sheep and goats. Susceptibility of goats to scrapie is influenced by polymorphisms of the prion protein gene (PRNP) of the host. Five polymorphisms are associated with reduced susceptibility to TSE's. In the study presented here caprine samples from a scrapie eradication program on Cyprus were genotyped and further characterized using BioRad TeSeE rapid test, histological, immunohistochemical and biochemical methods...
October 6, 2016: Veterinary Research
https://www.readbyqxmd.com/read/27713096/regulation-of-mitofusin1-by-mahogunin-ring-finger-1-and-the-proteasome-modulates-mitochondrial-fusion
#19
Rukmini Mukherjee, Oishee Chakrabarti
Health and homoeostasis are maintained by a dynamic balance between mitochondrial fission and fusion. Mitochondrial fusion machinery is largely unknown in mammals. Only a few reports have illustrated the role of Fzo1 in mitochondrial fusion known in Saccharomyces cerevisiae. We demonstrate that the ubiquitin ligase Mahogunin Ring Finger-1 (MGRN1) interacts with and constitutively ubiquitinates the mammalian homolog, Mitofusin1 (Mfn1) via K63 linkages. In mice models, loss of Mgrn1 function leads to severe developmental defects and adult-onset spongiform neurodegeneration, similar to prion diseases...
December 2016: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27697033/recombinant-antibody-fragments-for-neurodegenerative-diseases
#20
Karen Manoutcharian, Roxanna Perez-Garmendia, Goar Gevorkian
Recombinant antibody fragments are promising alternatives to full-length immunoglobulins and offer important advantages compared with conventional monoclonal antibodies: extreme specificity, higher affinity, superior stability and solubility, reduced immunogenicity as well as easy and inexpensive large-scale production. Different antibody formats such as single-chain fragment variable (scFv), single-domain antibody fragments (VHHs or sdAbs), bispecific antibodies (bsAbs), intrabodies and nanobodies, are currently being studied in pre-clinical models of cancer as well as infectious and autoimmune diseases and many of them are being tested as therapeutics in clinical trials...
September 30, 2016: Current Neuropharmacology
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