|1.||Alzheimer Disease (Alzheimer's Disease)
|2.||Neurodegenerative Diseases (Neurodegenerative Disease)
|3.||Parkinson Disease (Parkinson's Disease)
|1.||Collinge, John: 57 articles (10/2015 - 01/2003)|
|2.||Aguzzi, Adriano: 29 articles (12/2015 - 06/2002)|
|3.||Soto, Claudio: 27 articles (05/2015 - 08/2002)|
|4.||Gambetti, Pierluigi: 26 articles (10/2015 - 12/2002)|
|5.||Dong, Xiao-Ping: 25 articles (12/2015 - 08/2005)|
|6.||Brown, David R: 25 articles (09/2015 - 06/2002)|
|7.||Caughey, Byron: 25 articles (01/2015 - 03/2002)|
|8.||Prusiner, Stanley B: 22 articles (10/2015 - 03/2003)|
|9.||Carp, Richard I: 21 articles (01/2015 - 11/2002)|
|10.||Shi, Qi: 20 articles (12/2015 - 09/2006)|
04/01/2006 - "Among the most promising ones, antibodies have been shown to be protective against prion disease and heterocyclic small-molecule compounds have been proposed as antiprion lead compounds, initiating clinical trials."
01/01/2010 - "After passive transfer, anti-prion antibodies were shown to be very effective in curing peripheral but not central rodent prion disease, due to the fact that these anti-prion antibodies are relatively large molecules and cannot therefore cross the BBB. "
08/01/2010 - "Although there is currently no effective treatment for prion diseases, significant advances have been made in suppressing its progress, using antibodies that block the conversion of PrP(C) into PrP(Sc). "
08/01/2010 - "In order to be effective in treating individuals that have prion diseases, antibodies must be capable of arresting disease in its late stages. "
07/21/2000 - "The antibodies may help to explore the relationship of 219Glu/Lys polymorphism to the pathogenesis of human prion diseases."
11/02/2006 - "The most potent target compound 2a revealed an EC(50) value of 20 nM determined with a cell model of prion disease, thus substantially improving the antiprion efficacy of quinacrine."
11/29/2004 - "In a cell-based assay, quinacrine inhibits the conversion of normal host prion protein (PrPC) to PrPSc at a half-maximal concentration of 300 nM. While these data suggest that quinacrine may be beneficial in the treatment of prion disease, its penetration into brain tissue has not been extensively studied. "
11/29/2004 - "The documented extensive brain tissue penetration is encouraging suggesting quinacrine might be useful in the treatment of prion disease. "
11/29/2004 - "If quinacrine penetrates brain tissue in concentrations exceeding that demonstrated for in vitro inhibition of PrPSc, it may be useful in the treatment of prion disease. "
04/01/2009 - "Safety and efficacy of quinacrine in human prion disease (PRION-1 study): a patient-preference trial."
01/01/2015 - "We hypothesize that the presence of heterologous prion proteins from one species might therefore constitute an effective treatment for prion disease in another species. "
06/01/2015 - "A few studies showed that amplification methods can detect prions in either cerebrospinal fluid, olfactory epithelium, blood and/or urine in various human prion diseases. "
04/01/2015 - "In contrast to recent progress in studies of laboratory rodent-adapted prions, current understanding of the molecular basis of human prion diseases and, especially, their vast phenotypic diversity is very limited. "
01/01/2015 - "Previous studies established that prion disease with unique strain-specific phenotypes could be induced by in vitro-formed recombinant PrP (rPrP) fibrils with structures different from that of authentic prions, or PrP(Sc). "
07/01/2013 - "A study demonstrating an interaction between cytosolically-exposed prion protein and MGRN1 suggested that disruption of MGRN1 function may contribute to prion disease pathogenesis, but we recently showed that neither loss of MGRN1 nor MGRN1 overexpression influences the onset or progression of prion disease following intracerebral inoculation with Rocky Mountain Laboratory prions. "
|4.||Amyloid (Amyloid Fibrils)IBA
01/01/2015 - "This study illustrates that transmissible prion diseases with very similar disease phenotypes could be produced via two alternative procedures: direct inoculation of recombinant PrP amyloid fibrils or in vitro-produced atypical PrPres. "
11/01/2014 - "Patient phenotypes, unclassifiable as prion disease, may depend on the location of the mutation in the N-terminal domain, outside the amyloid core of pathologic prion protein, although further functional studies are required to determine whether and how this mutation exerts its pathogenic effect. "
03/01/2014 - "In several recent studies transmissible prion disease was induced in animals by inoculation with recombinant prion protein amyloid fibrils produced in vitro. "
07/01/2012 - "Recent studies demonstrated that authentic PrP (Sc) and transmissible prion disease could be generated in wild type animals by inoculation of recombinant prion protein amyloid fibrils, which are structurally different from PrP (Sc) and lack any detectable PrP (Sc) particles. "
12/07/2011 - "Recent studies have identified amyloid fibrils ranging from bacteria to humans that have a beneficial role, instead of being associated with a misfolded state that has been implicated in diseases such as Alzheimer's, Parkinson's and prion diseases. "
|5.||Proteins (Proteins, Gene)IBA
11/01/2011 - "This cellular response may offer new insights into the homeostatic mechanisms involved in detection and clearance of the misfolded proteins that drive prion disease pathogenesis."
10/01/2008 - "This study suggests that accumulated citrullinated proteins and abnormal activation of PAD2 may function in the pathogenesis of prion diseases and serve as potential therapeutic targets."
04/01/2015 - "Proteomics analyses for the global proteins in the brain tissues of different human prion diseases."
01/01/2015 - "Proteins most notably affected were those also reported to be citrullinated in other disorders, including prion disease and MS. In vivo findings were extended in an in vitro model of simulated TBI employing normal human astrocytes. "
01/01/2015 - "iTRAQ-based mass spectrometric (MS) analysis of the eluted products from the brain tissues of a normal healthy subject and patients with various prion diseases identified 1,509 SNO proteins with high confidence [false discovery rate (FDR) <1%]. "
04/01/2009 - "Together, these findings suggest that either loss of PrP copper-dependent function or loss of copper-mediated protection against PrP polymerization makes a significant contribution to early onset prion disease."
05/01/2013 - "Utilizing NMR and EPR spectroscopy to probe the role of copper in prion diseases."
08/21/2012 - "Copper influences the pathogenesis of prion disease, but whether it is beneficial or detrimental remains controversial. "
08/21/2012 - "Disruption of copper homeostasis due to a mutation of Atp7a delays the onset of prion disease."
08/07/2012 - "Since prion diseases present metal dyshomeostasis and increased oxidative stress, we described the copper-binding site located in the human C-terminal domain of PrP-HuPrP(90-231), both in the wild-type protein and in the protein carrying the pathological mutation Q212P. "
|7.||RNA (Ribonucleic Acid)IBA
06/03/1999 - "Future RNA screening conducted as illustrated may help to reveal a spectrum of genes relevant for the etiopathogenesis and/or diagnosis of prion disease."
10/01/2013 - "This article reviews the novel approach of using RNA-based drugs as a therapeutic opportunity for prion disease. "
10/01/2013 - "Small RNA drugs for prion disease: a new frontier."
01/01/2013 - "Our results demonstrate the therapeutic potential of an RNA aptamer as to prion diseases."
01/01/2013 - "Here, we show that an RNA aptamer comprising only 12 residues, r(GGAGGAGGAGGA) (R12), reduces the PrP(Sc) level in mouse neuronal cells persistently infected with the transmissible spongiform encephalopathy agent. "
05/01/2004 - "The therapeutic efficacy of direct drug infusion into the brain, the target organ of transmissible spongiform encephalopathies, was assessed in transgenic mice intracerebrally infected with 263K scrapie agent. "
01/30/2006 - "Correlative studies support lipid peroxidation is linked to PrP(res) propagation as an early primary pathogenic event in prion disease."
11/29/2005 - "These studies provide new insight into the mechanism of prion polymerization, solve a long-standing practical problem in producing PrP-res fibrils from full-length PrP, and may help in identifying new genetic and environmental factors that modulate prion disease."
12/15/2015 - "Transmission of first-passage tg60CWD-H95(+) isolates into tg33 mice, however, elicited two prion disease presentations consistent with a mixture of strains associated with different PrP-res glycotypes. "
11/02/2015 - "The latter state includes the toxic species (scrapie PrP, PrP(Sc)) knowledge of which would facilitate the development of drugs against prion diseases. "
01/01/2006 - "The generation of novel PrP-specific monoclonal antibodies (MAbs) has greatly improved diagnostic methodology and basic research on prion diseases as well. "
01/01/2009 - "The results of the present study not only provide an example of the successful production of Ab2 monoclonal antibodies based on a well planned strategy for selection, but should also provide a new experimental approach that is applicable to the field of prion diseases."
11/01/2008 - "Thus, in nontransgenic mice, PrP-specific monoclonal antibodies were produced, useful in studies and diagnostics of prion diseases."
06/15/2006 - "Recent in vivo studies indicate that prion replication can be inhibited by anti-PrP monoclonal antibodies that led to the indefinite delay in the development of prion disease. "
04/01/2003 - "In the present study, this approach was adopted using a panel of anti-PrP monoclonal antibodies (MAbs) in conventional sandwich enzyme-linked immunosorbent assay (ELISA) to investigate hamster and two distinct strains of mouse prion diseases. "
|10.||Protein Isoforms (Isoforms)IBA
10/01/2014 - "The cellular prion protein, PrP(C), whose aberrant isoforms are related to prion diseases of humans and animals, has a still obscure physiological function. "
01/01/2014 - "During pathogenesis of prion disease, the prion protein misfolds into β-sheet rich, protease-resistant isoforms. "
11/01/2013 - "Other conformational variants of PrP have also been proposed to contribute to neurotoxicity in prion diseases, including misfolded intermediates as well as cytosolic and transmembrane isoforms. "
11/01/2013 - "Prion diseases encompass a diverse group of neurodegenerative conditions characterized by the accumulation of misfolded prion protein (PrP) isoforms. "
06/01/2013 - "Prion diseases are associated with the conformational conversion of cellular prion protein (PrP(C)) to pathological β-sheet isoforms (PrP(Sc)), which is the infectious agent beyond comprehension. "
|1.||Blood Transfusion (Blood Transfusions)
01/01/2015 - "In addition, over the last 5 years, various experimental studies carried out in animal models have confirmed that leukocyte reduction provides a high, but not absolute, protection against transmission of prion disease by blood transfusion. "
08/01/2006 - "Our results warrant further studies on the role of platelet PrPc in the transmission of prion diseases by blood transfusion."
12/01/2003 - "The possible transmission of variant CJD (vCJD) through blood transfusion or use of plasma-derived products prompted this study comparing infectivity in murine models of vCJD and Gerstmann-Sträussler-Scheinker (GSS) disease, a non-vCJD form of transmissible spongiform encephalopathy (TSE). "
01/01/2011 - "These data suggest that all blood components are vectors for prion disease transmission, and highlight the importance of multiple control measures to minimise the risk of human to human transmission of vCJD by blood transfusion."
01/01/2011 - "All clinically-relevant blood components transmit prion disease following a single blood transfusion: a sheep model of vCJD."
04/01/2013 - "Progress in therapeutics for rare disorders like prion disease is impeded by the lack of validated outcome measures and a paucity of natural history data derived from prospective observational studies. "
02/24/2011 - "The studies described herein suggest 2-aminothiazoles as promising new leads in the search for effective therapeutics for prion diseases."
07/28/2015 - "Hence, understanding the molecular mechanism of formation of the misfolded oligomers of PrP is critical for developing an understanding about the prion diseases and for developing anti-prion therapeutics. "
03/01/2015 - "Our results show that pathologic mutations promote subdomain separation and suggest that stabilization of the native structure might be a viable strategy for the development of novel therapeutics for prion diseases."
01/01/2014 - "In this context, the analysis of gene expression alterations occurring in prion-infected animals represents a powerful tool that may contribute to unravel the molecular basis of prion diseases and therefore discover novel potential targets for diagnosis and therapeutics. "
01/01/2006 - "In addition to AD and its models, this type of therapy has primarily been assessed in prion disease with positive results, further supporting the potential of immunotherapy for a variety of protein-related diseases in which clearance of the pathogenic agent is likely to alleviate symptoms."
06/10/2014 - "However, the failure to respond to aggressive immunotherapy warns against VGKC-complex Abs being pathogenic, and their presence does not preclude the possibility of prion disease."
01/01/2014 - "Testing for the presence of this neuronal surface autoantibody in patients presenting with subacute encephalopathy is therefore crucial as it may both revoke the bleak diagnosis of prion disease and allow institution of potentially life-saving immunotherapy. "
07/01/2013 - "Collectively, these findings support the disease-specific approach for immunotherapy of prion diseases but also suggest that the concept of conformation-specific immunotherapy may be complicated in individuals who are genetically predisposed to PrP(C) misfolding. "
02/01/2013 - "Immunotherapy in prion disease."
|4.||Surgical Instruments (Clip)
05/01/2007 - "Iatrogenic transmission of transmissible spongiform encephalopathies (TSEs) has been demonstrated via surgical instruments and there is concern over the efficacy of conventional decontamination techniques used to reprocess reusable instruments. "
01/01/2008 - "The unusual resistance of agents causing transmissible spongiform encephalopathies (TSEs) to chemical or thermal inactivation requires special decontamination procedures in order to prevent accidental transmission of these pathogens by surgical instruments. "
10/01/2007 - "The development of prion decontamination procedures that are compatible with routine cleaning and sterilization of medical and surgical instruments may reduce the risk of the transmission of prion disease in general surgery."
12/01/2006 - "Prion diseases can resist traditional inactivation strategies and may be iatrogenically transmitted by surgical instruments through the human population. "
09/01/2005 - "IN RESPONSE TO RECENT REPORTS from several major US medical centers regarding possible exposure of surgical patients to transmissible spongiform encephalopathies (TSEs), one institution developed guidelines to care for surgical patients with the preoperative diagnosis of "rule out TSE." BASED ON CURRENT sterilization technology, using disposable surgical instruments may be the safest way to eliminate concerns of iatrogenic transmission of TSE and reduce the number of hospital staff members who could be exposed to the TSE contagion. "
01/01/1998 - "Use of brain grafts to study the pathogenesis of prion diseases."
01/01/2013 - "All cases with acquired prion diseases except for one case of variant CJD are dura mater graft-associated CJD (dCJD), and the number of dCJD in Japan is account for more than 60％ of all patients with dCJD all over the world. "
05/01/2010 - "Compared with patients in other countries, a relatively larger number of Japanese patients characteristically have dura mater graft-associated CJD and hereditary prion diseases. "
10/01/2009 - "One possible explanation for the accelerated deposition of alpha-syn in the graft is that the aggregation of alpha-syn from the host tissue to the graft is spread by a prion disease-like mechanism. "
11/01/2007 - "The 72 patients with environmentally acquired prion diseases included 71 with dural graft-associated CJD (dCJD) and one with variant CJD (vCJD). "