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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
Connie Luk, Samantha Jones, Claire Thomas, Nick C Fox, Tze H Mok, Simon Mead, John Collinge, Graham S Jackson
Importance: Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder associated with the accumulation of infectious abnormal prion protein through a mechanism of templated misfolding. A recent report has described the detection of abnormal prion protein in the urine of patients with variant CJD (vCJD) using protein misfolding by cyclic amplification, which was apparently absent in the more common sporadic form of CJD (sCJD). A noninvasive diagnostic test could improve early diagnosis of sCJD and, by screening donations, mitigate the potential risks of prion transmission through human urine-derived pharmaceuticals...
October 3, 2016: JAMA Neurology
Qi Shi, Wei Zhou, Cao Chen, Chen Gao, Kang Xiao, Jing Wang, Bao-Yun Zhang, Yuan Wang, Feng Zhang, Xiao-Ping Dong
The surveillance of CJD or human prion diseases (PrDs) has been conducted for ten years in China. To evaluate the quality of China CJD surveillance system, the collections of the clinical and epidemiological information, the sampling, the clinical examinations and laboratory tests and follow-up survey were separately analyzed based on the data from 2010 to 2015. The obtaining rates of clinical-information table, epidemiological-information table, sample inspection sheet and medical record of the referring patients from reporting units to the center of CJD surveillance maintained or reached at very high levels, being close to 100% in the past three years...
October 3, 2016: Prion
Emilie Jaumain, Isabelle Quadrio, Laetitia Herzog, Fabienne Reine, Human Rezaei, Olivier Andréoletti, Hubert Laude, Armand Perret-Liaudet, Stéphane Haïk, Vincent Béringue
: Prions are proteinaceous pathogens responsible for subacute spongiform encephalopathies in animals and humans. The prions responsible for bovine spongiform encephalopathy (BSE) are zoonotic agents, causing variant Creutzfeldt-Jakob disease (CJD) in humans. The transfer of prions between species is limited by a species barrier, which is thought to reflect structural incompatibilities between host cellular prion protein (PrP(C)) and the infecting pathological PrP assemblies (PrP(Sc)) constituting the prion...
September 28, 2016: Journal of Virology
Silvia Koscova, Dana Zakova Slivarichova, Ivana Tomeckova, Katarina Melicherova, Martin Stelzer, Alzbeta Janakova, Dana Kosorinova, Girma Belay, Eva Mitrova
Creutzfeldt-Jakob disease is a rare, but rapidly progressive, up to now untreatable and fatal neurodegenerative disorder. Clinical diagnosis of Creutzfeldt-Jakob disease (CJD) is difficult; however, it can be facilitated by suitable biomarkers. Aim of the present study is to compare levels of cerebrospinal fluid biomarkers (total tau protein, phosphorylated-tau protein, protein 14-3-3 and amyloid beta) in Slovak population of CJD suspect cases, retrospectively in over a 10-year period. One thousand three hundred sixty-four CSF samples from patients with suspect CJD, forming a homogenous group in terms of geographical as well as of equal transport conditions, storage and laboratory processing, were analysed...
September 24, 2016: Molecular Neurobiology
Oriol Grau-Rivera, Anna Calvo, Núria Bargalló, Gemma C Monté, Carlos Nos, Albert Lladó, José Luis Molinuevo, Ellen Gelpi, Raquel Sánchez-Valle
BACKGROUND: Quantitative neuroimaging might unveil abnormalities in prion diseases that are not perceivable at visual inspection. On the other hand, scarce studies have quantified volumetric changes in prion diseases. OBJECTIVES: We aim to characterize volumetric and diffusion tensor imaging (DTI) changes in patients with prion diseases who presented with either Creutzfeldt-Jakob disease (CJD) or fatal insomnia (FI) phenotype. METHODS: Twenty patients with prion diseases- 15 with CJD and 5 with fatal insomnia (FI)- and 40 healthy controls were examined with a 3-Tesla magnetic resonance imaging scanner...
September 21, 2016: Journal of Alzheimer's Disease: JAD
Gabor G Kovacs
Recent studies on iatrogenic Creutzfeldt-Jakob disease (CJD) raised concerns that one of the hallmark lesions of Alzheimer disease (AD), amyloid-β (Aβ), may be transmitted from human-to-human. The neuropathology of AD-related lesions is complex. Therefore, many aspects need to be considered in deciding on this issue. Observations of recent studies can be summarized as follows: 1) The frequency of iatrogenic CJD cases with parencyhmal and vascular Aβ deposits is statistically higher than expected; 2) The morphology and distribution of Aβ deposition may show distinct features; 3) The pituitary and the dura mater themselves may serve as potential sources of Aβ seeds; 4) Cadaveric dura mater from two examined cases shows Aβ deposition; and 5) There is a lack of evidence that the clinical phenotype of AD appears following the application of cadaveric pituitary hormone or dura mater transplantation...
September 20, 2016: Prion
Maria Gabriella Vita, Dorina Tiple, Alessandra Bizzarro, Anna Ladogana, Elisa Colaizzo, Sabina Capellari, Marcello Rossi, Piero Parchi, Carlo Masullo, Maurizio Pocchiari
We report a case of rapidly evolving neurological disease in a patient with neuropathological lesions of Creutzfeldt-Jakob disease (CJD), Lewy body dementia (LBD), chronic subcortical vascular encephalopathy and meningothelial meningioma. The coexistence of severe multiple pathologies in a single patient strengthens the need to perform accurate clinical differential diagnoses in rapidly progressive dementias.
September 16, 2016: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Oren S Cohen, Joab Chapman, Amos D Korczyn, Oliver L Siaw, Naama Warman-Alaluf, Zeev Nitsan, Shmuel Appel, Esther Kahana, Hanna Rosenmann, Chen Hoffmann
The use of diffusion MRI improved the accuracy of diagnosis in Creutzfeldt-Jakob disease (CJD) and expanded our knowledge of the changes occurring in the brain during the disease. The aim of this study was to test whether in patients with E200K familial CJD (fCJD) the clinical severity correlates with the disease burden as reflected by the extent of cortical involvement in DWI MRI. Consecutive fCJD patients were examined by a neurologist who performed several tests including the CJD neurological scale (CJD-NS), MiniMental status examination (MMSE), Frontal Assessment Battery (FAB), NIH Stroke Scale (NIHSS), and the expanded disability status scale (EDSS)...
September 13, 2016: Journal of Neural Transmission
Ali Al Balushi, Marshall W Meeks, Ghazala Hayat, Jafar Kafaie
BACKGROUND: Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive neurodegenerative disease that almost always results in death in under a year from onset of symptoms. Here, we report four cases of CJD with different clinical presentations diagnosed at our institution over a 2-year period. CASES: The first patient is an 82-year-old woman who presented with depression, cognitive decline, and word-finding difficulty over 4 weeks. The patient deteriorated neurologically to akinetic mutism and death within 6 weeks of presentation...
2016: Frontiers in Neurology
Roger A Moore, Young Pyo Choi, Mark W Head, James W Ironside, Robert Faris, Diane L Ritchie, Gianluigi Zanusso, Suzette Alise Priola
Aggregated and protease-resistant mammalian prion protein (PrPSc) is the primary protein component of infectious prions. Enriched PrPSc preparations are often used to study the mechanisms underlying prion disease. However, most enrichment procedures are relatively non-specific and tend to yield significant amounts of non-PrPSc components, including various proteins that could confound functional and structural studies. It is thus important to identify these proteins and assess their potential relevance to prion pathogenesis...
September 5, 2016: Journal of Proteome Research
André Karch, Franc Llorens, Matthias Schmitz, Amandeep Singh Arora, Saima Zafar, Peter Lange, Christian Schmidt, Inga Zerr
BACKGROUND: Cerebrospinal fluid (CSF) biomarkers are routinely used for the differential diagnosis of rapidly progressive dementia, but are also affected by patients' characteristics. OBJECTIVE: To assess if stratification by age, sex, and genetic risk factors improves the accuracy of cerebrospinal fluid (CSF) biomarkers in patients with rapidly progressive dementia. METHODS: 1,538 individuals with sporadic Creutzfeldt-Jakob disease (CJD), 173 with classic Alzheimer's disease (cAD), 37 with rapidly progressive Alzheimer's disease (rpAD), and 589 without signs of dementia were included in this retrospective diagnostic study...
October 18, 2016: Journal of Alzheimer's Disease: JAD
Chunyu Wang, Deming Zhao, Syed Zahid Ali Shah, Wei Yang, Chaosi Li, Lifeng Yang
Sporadic Creutzfeldt-Jakob disease (sCJD) is the most frequent fatal human prion disease with a rapid progression and unknown mechanism. The synaptic vesicle (SV) cycle pathway has been a hot research field associated with many neurodegenerative diseases that affect synaptic function and thus may affect pathogenesis of the disorder. Here, we used the iTRAQ-based proteomic method and a KEGG pathway enrichment analysis to meticulously analyze all pathways involved in sCJD disease. In total, 1670 proteins were validated in pooled cerebrospinal fluid (CSF) from 20 patients with sCJD compared with that from 13 patients without CJD...
August 25, 2016: Molecular Neurobiology
P Chandrasekaran, R Rajasekaran
The development of fatal transmissible spongiform encephalopathies (TSE) is associated with the conformational conversion of the normal cellular prion protein, PrP(C), into its pathogenic isoform, PrP(Sc). The present study revealed the structural consequences that induce the conversion of PrP(C)→ PrP(Sc) upon mutation V210I linked with genetic Creutzfeldt-Jakob disease (CJD) using the classical molecular dynamics (MD) approach. Similar to the experimental results, the mutant showed biased disruption in the local folding of α2 and the complete distortion of α3...
October 20, 2016: Molecular BioSystems
Patrick Oeckl, Fabian Metzger, Magdalena Nagl, Christine A F von Arnim, Steffen Halbgebauer, Petra Steinacker, Albert C Ludolph, Markus Otto
α-Synuclein (αSyn) is a major constituent of proteinaceous aggregates in neurodegenerative diseases such as Parkinson's disease (PD) and a potential biomarker candidate for diagnosis and treatment effects. However, studies about αSyn in cerebrospinal fluid (CSF) in diseases are inconsistent and mainly based on immunological assays. Quantitative information about β-synuclein (βSyn) and γ-synuclein (γSyn) in CSF is not available.Here, we present an alternative method for the simultaneous quantification of αSyn, βSyn and γSyn in CSF by multiple reaction monitoring (MRM) with a high sequence coverage (70%) of αSyn to validate previous, ELISA-based results and characterize synucleins in CSF in more detail...
October 2016: Molecular & Cellular Proteomics: MCP
J Janoutová, L Siváková, L Máslová, A Hozák, K Vařechová, V Janout
AIM: The analysis of the available reported data and epidemiological investigation data on the cases of Creutzfeld-Jakob disease (CJD) that emerged in the Nový Jičín district in 2001-2011, with a focus on the familial form of the disease. MATERIAL AND METHODS: Data from the Regional Public Health Service of the Moravian-Silesian Region, local Public Health Centre Nový Jičín, were used for the analysis. RESULTS: This is a retrospective report of 10 cases of familial Creutzfeld-Jakob disease (CJD) in the Nový Jičín district in 2001-2011, based on the data from the public health service...
2016: Epidemiologie, Mikrobiologie, Imunologie
Brent Race, Katie Phillips, Allison Kraus, Bruce Chesebro
Tauopathies are a family of neurodegenerative diseases in which fibrils of human hyperphosphorylated tau (P-tau) are believed to cause neuropathology. In Alzheimer disease, P-tau associates with A-beta amyloid and contributes to disease pathogenesis. In familial human prion diseases and variant CJD, P-tau often co-associates with prion protein amyloid, and might also accelerate disease progression. To test this latter possibility, here we compared progression of amyloid prion disease in vivo after scrapie infection of mice with and without expression of human tau...
July 3, 2016: Prion
A Dulamea, E Solomon
OBJECTIVE: Sporadic Creutzfeldt-Jakob disease (CJD) is a human prion disease, rapidly progressive and fatal, characterized by spongiform encephalopathy. The characteristic triad of signs - rapidly progressive dementia, myoclonus and periodic sharp wave complexes (PSWC) on electroencephalography (EEG) - usually appear in the late stages of the disease. The clinical diagnosis of CJD ante-mortem involves the exclusion of the rapidly progressive non-prionic dementias, the definitive diagnosis requiring brain tissue confirmation...
April 2016: Journal of Medicine and Life
Joel C Watts, Kurt Giles, Daniel J Saltzberg, Brittany N Dugger, Smita Patel, Abby Oehler, Sumita Bhardwaj, Andrej Sali, Stanley B Prusiner
: The biochemical and neuropathological properties of bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) prions are faithfully maintained upon transmission to guinea pigs. However, primary and secondary transmissions of BSE and vCJD in guinea pigs result in long incubation periods of ∼450 and ∼350 days, respectively. To determine if the incubation periods of BSE and vCJD prions could be shortened, we generated transgenic (Tg) mice expressing guinea pig prion protein (GPPrP)...
November 1, 2016: Journal of Virology
Francesco Carlo Morabito, Maurizio Campolo, Nadia Mammone, Mario Versaci, Silvana Franceschetti, Fabrizio Tagliavini, Vito Sofia, Daniela Fatuzzo, Antonio Gambardella, Angelo Labate, Laura Mumoli, Giovanbattista Gaspare Tripodi, Sara Gasparini, Vittoria Cianci, Chiara Sueri, Edoardo Ferlazzo, Umberto Aguglia
A novel technique of quantitative EEG for differentiating patients with early-stage Creutzfeldt-Jakob disease (CJD) from other forms of rapidly progressive dementia (RPD) is proposed. The discrimination is based on the extraction of suitable features from the time-frequency representation of the EEG signals through continuous wavelet transform (CWT). An average measure of complexity of the EEG signal obtained by permutation entropy (PE) is also included. The dimensionality of the feature space is reduced through a multilayer processing system based on the recently emerged deep learning (DL) concept...
May 3, 2016: International Journal of Neural Systems
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