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Sudheer Babu Sangeetham, Krisztina Huszár, Petra Bencsura, Antal Nyeste, Éva Hunyadi-Gulyás, Elfrieda Fodor, Ervin Welker
Transmissible spongiform encephalopathies are centered on the conformational transition of the prion protein from a mainly helical, monomeric structure to a β-sheet rich ordered aggregate. Experiments indicate that the main infectious and toxic species in this process are however shorter oligomers, formation of which from the monomers is yet enigmatic. Here, we created 25 variants of the mouse prion protein site-specifically containing one genetically-incorporated para-benzoyl-phenylalanine (pBpa), a cross-linkable non-natural amino acid, in order to interrogate the interface of a prion protein-dimer, which might lie on the pathway of oligomerization...
May 17, 2018: Journal of Molecular Biology
Wen-Hao Zhao, Chang-Feng Chi, Yu-Qin Zhao, Bin Wang
Collagen is one of the most useful biomaterials and widely applied in functional food and cosmetics. However, some consumers have paid close attention to the safety of mammalian collagens because of the outbreaks of bovine spongiform encephalopathy (BSE), foot-and-mouth disease (FMD), and other prion diseases. Therefore, there is a strong demand for developing alternative sources of collagen, with one promising source being from the process by-products of commercial fisheries. In this report, acid-soluble collagen (ASC-SB) and pepsin-soluble collagen (PSC-SB) from swim bladders of miiuy croaker ( Miichthys miiuy ) were isolated with yields of 1...
May 12, 2018: Marine Drugs
Mengyu Lai, Hao Yao, Syed Zahid Ali Shah, Wei Wu, Di Wang, Ying Zhao, Lu Wang, Xiangmei Zhou, Deming Zhao, Lifeng Yang
Prion diseases are neurodegenerative disorders characterized by the accumulation of misfolded prion protein, spongiform changes in the brain, and brain inflammation as a result of the wide-spread activation of microglia. Autophagy is a highly conserved catabolic process for the clearance of cytoplasmic components, including protein aggregates and damaged organelles; this process also eliminates pathological PrPSc as it accumulates during prion infection. The NALP3 inflammasome is a multiprotein complex that is a component of the innate immune system and is responsible for the release of pro-inflammatory cytokines...
2018: Frontiers in Aging Neuroscience
Megan C Romano, Alan T Loynachan, Dave C Bolin, Uneeda K Bryant, Laura Kennedy, Mike S Filigenzi, Birgit Puschner, Robert H Poppenga, Cynthia L Gaskill
Use of the neurotoxic rodenticide bromethalin has steadily increased since 2011, resulting in an increased incidence of bromethalin intoxications in pets. Presumptive diagnosis of bromethalin toxicosis relies on history of possible rodenticide exposure coupled with compatible neurologic signs or sudden death, and postmortem examination findings that eliminate other causes of death. Diagnosis is confirmed by detecting the metabolite desmethylbromethalin (DMB) in tissues. In experimental models, spongiform change in white matter of the central nervous system (CNS) is the hallmark histologic feature of bromethalin poisoning...
April 1, 2018: Journal of Veterinary Diagnostic Investigation
Fei Wang, Xinhe Wang, Romany Abskharon, Jiyan Ma
Transmissible spongiform encephalopathies, also known as prion diseases, are a group of fatal neurodegenerative disorders affecting both humans and animals. The central pathogenic event in prion disease is the misfolding of normal prion protein (PrPC ) into the pathogenic conformer, PrPSc , which self-replicates by converting PrPC to more of itself. The biochemical hallmark of PrPSc is its C-terminal resistance to proteinase K (PK) digestion, which has been historically used to define PrPSc and is still the most widely used characteristic for prion detection...
April 24, 2018: Acta Neuropathologica Communications
Kazuhiro Tokuda, Byron Baron, Yasuhiro Kuramitsu, Takao Kitagawa, Nobuko Tokuda, Naoyuki Morishige, Masaaki Kobayashi, Kazuhiro Kimura, Kazuyuki Nakamura, Koh-Hei Sonoda
PURPOSE: Numerous fixative solutions are available but many are not amenable to the histomorphological preservation of retinae. The investigators specifically focused on retinal histological studies, which rather than 4% formaldehyde (FA), often use Davidson's fixative. However the latter has its limitations. The purpose of this study was to produce a new fixative which maintains retinae closer to the in vivo conditions. STUDY DESIGN: Experimental design. METHODS: Four fixative formulations (4% paraformaldehyde, Davidson's fixative, modified Davidson's fixative and an in-house fixative - TB-Fix) were tested on retinae and the outcomes on histomorphology and immunohistochemical staining for selected antigenic markers was compared...
April 24, 2018: Japanese Journal of Ophthalmology
Yevgeniya A Mironova, Jing-Ping Lin, Ashley Kalinski, Lucas Huffman, Guy M Lenk, Leif A Havton, Miriam H Meisler, Roman J Giger
The signaling lipid phosphatidylinositol 3,5-bisphosphate, PI(3,5)P2, functions in vesicular trafficking through the endo-lysosomal compartment. Cellular levels of PI(3,5)P2 are regulated by an enzyme complex comprised of the kinase PIKFYVE, the phosphatase FIG4, and the scaffold protein VAC14. Mutations of human FIG4 cause inherited disorders including Charcot-Marie-Tooth disease type 4J, polymicrogyria with epilepsy, and Yunis-Varón syndrome. Constitutive Fig4-/- mice exhibit intention tremor, spongiform degeneration of neural tissue, hypomyelination, and juvenile lethality...
April 24, 2018: Human Molecular Genetics
F Serra, S Dudas, J M Torres, R Anderson, A Oevermann, J C Espinosa, S Czub, T Seuberlich
Bovine spongiform encephalopathy (BSE) is caused by different prion strains that are discriminated by the molecular characteristics of the pathological prion protein. In 2011, Switzerland reported two presumptive cases of BSE in cattle with a prion protein phenotype different from previously described strains, and it was unclear whether these findings were related to a transmissible disease and have implications on animal and public health. In this study, brain tissues of these cases were inoculated into transgenic mice expressing the bovine prion protein (BoPrP-Tg110) and into cattle...
April 19, 2018: Transboundary and Emerging Diseases
Yong-Chan Kim, Byung-Hoon Jeong
Prion diseases are caused by structural changes in normal prion protein (PrPC ). The prion gene family includes four members: prion protein ( PRNP ), prion-like protein ( PRND ), shadow of PRNP ( SPRN ) and prion-related protein ( PRNT ). Genetic association studies of prion diseases and the other genes in the prion gene family, except for PRNT , have been performed in cattle. Our previous studies indicated that the distribution of PRNP promoter polymorphisms related with bovine spongiform encephalopathy susceptibility is significantly different in Hanwoo (Korean native cattle) and Holstein cattle...
April 17, 2018: Veterinary Record
Marcelo A Barria, Andrew Lee, Alison Je Green, Richard Knight, Mark W Head
Human prion diseases constitute a group of infectious and invariably fatal neurodegenerative disorders associated with misfolding of the prion protein. Variant Creutzfeldt-Jakob disease (vCJD) is a zoonotic prion disease linked to oral exposure to the infectious agent that causes bovine spongiform encephalopathy (BSE) in cattle. The most recent case of definite vCJD was heterozygous (MV) at polymorphic codon 129 of the prion protein gene PRNP while all of the previous 177 definite or probable vCJD cases who underwent genetic analysis were methionine homozygous (MM)...
April 2018: Journal of Pathology. Clinical Research
Baaissa Babelhadj, Michele Angelo Di Bari, Laura Pirisinu, Barbara Chiappini, Semir Bechir Suheil Gaouar, Geraldina Riccardi, Stefano Marcon, Umberto Agrimi, Romolo Nonno, Gabriele Vaccari
Prions cause fatal and transmissible neurodegenerative diseases, including Creutzfeldt-Jakob disease in humans, scrapie in small ruminants, and bovine spongiform encephalopathy (BSE). After the BSE epidemic, and the associated human infections, began in 1996 in the United Kingdom, general concerns have been raised about animal prions. We detected a prion disease in dromedary camels (Camelus dromedarius) in Algeria. Symptoms suggesting prion disease occurred in 3.1% of dromedaries brought for slaughter to Ouargla abattoir in 2015-2016...
June 17, 2018: Emerging Infectious Diseases
Ryo Honda
Transmissible spongiform encephalopathy (TSE) is associated with misfolding of prion protein (PrP) into an amyloid β-rich aggregate. Previous studies have indicated that PrP interacts with Alzheimer disease amyloid-β peptide (Aβ), but it remains elusive how this interaction impacts on the misfolding of PrP. This study presents the first in vitro evidence that Aβ induces PrP-amyloid formation at submicromolar concentrations. Interestingly, systematic mutagenesis of PrP revealed that Aβ requires no specific amino acid sequences in PrP, and induces the misfolding of other unrelated proteins (insulin and lysozyme) into amyloid fibrils in a manner analogous to PrP...
April 12, 2018: Angewandte Chemie
Thomas J Hagenaars, Marielle B Melchior, Jack J Windig, Alex Bossers, Aart Davidse, Fred G van Zijderveld
Scrapie is a transmissible spongiform encephalopathy in sheep and an example of a disease that may be controlled through breeding for disease resistance. Member states of the European Union have introduced strategies for breeding against scrapie based on the selection of genetically resistant breeding rams. An ambitious strategy adopted in The Netherlands consisted of selecting resistant rams for breeding throughout both breeding and production sectors. Mathematical modelling of the effect of a breeding program on the spreading capacity of scrapie in a national flock is needed for making assessments on how long a breeding strategy needs to be maintained to achieve disease control...
2018: PloS One
Nai-Zheng Ding, Xing-Ming Wang, Xiang-Wen Jiao, Ran Li, Chao Zeng, Shan-Ni Li, Hong-Shan Guo, Ze-You Wang, Zhu Huang, Cheng-Qiang He
Prion protein (PrP) is encoded by a single copy gene Prnp in many cell and tissue types. PrP is very famous for its infectious conformers (PrPSC) resulting in transmissible spongiform encephalopathies (TSEs). At present, physiological functions of its cellular isoform (PrPC) remain ambiguous. Although PrPC expression has been found in uterus, whether it functions in maternal-fetal dialogue during early pregnant is unknown. In this study, we examined PrPC mRNA and protein in the uterus of peri-implantation mice, and found that they were expressed with a spatiotemporal dynamic pattern...
March 20, 2018: Biology of Reproduction
Kirby M Donnelly, Margaret M P Pearce
Protein aggregation is a central feature of most neurodegenerative diseases, including Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD), and amyotrophic lateral sclerosis (ALS). Protein aggregates are closely associated with neuropathology in these diseases, although the exact mechanism by which aberrant protein aggregation disrupts normal cellular homeostasis is not known. Emerging data provide strong support for the hypothesis that pathogenic aggregates in AD, PD, HD, and ALS have many similarities to prions, which are protein-only infectious agents responsible for the transmissible spongiform encephalopathies...
March 12, 2018: Journal of Visualized Experiments: JoVE
Cosimo Durante, Giorgio Grani, Livia Lamartina, Sebastiano Filetti, Susan J Mandel, David S Cooper
Importance: Thyroid nodules are common, being detected in up to 65% of the general population. This is likely due to the increased use of diagnostic imaging for purposes unrelated to the thyroid. Most thyroid nodules are benign, clinically insignificant, and safely managed with a surveillance program. The main goal of initial and long-term follow-up is identification of the small subgroup of nodules that harbor a clinically significant cancer (≈10%), cause compressive symptoms (≈5%), or progress to functional disease (≈5%)...
March 6, 2018: JAMA: the Journal of the American Medical Association
Sean D Raj, Rohit Ram, David J Sabbag, Mark A Sultenfuss, Rebecca Matejowsky
OBJECTIVE: Thyroid nodules are prevalent in over half the general population. Several multidisciplinary societies have management recommendations. However, the majority of data to support these guidelines are derived from studies of predominantly younger and female populations. This study's aim was to evaluate characteristics of thyroid nodules in a largely older and male Veteran population and apply these findings prospectively to reduce unnecessary thyroid fine needle aspiration (FNA)...
January 6, 2018: Current Problems in Diagnostic Radiology
M U Cinar, D A Schneider, D F Waldron, K I O'Rourke, S N White
Scrapie is a transmissible spongiform encephalopathy of sheep and goats, and scrapie eradication programs in many parts of the world rely on strong genetic resistance to classical scrapie in sheep. However, the utility of putative resistance alleles in goats has been a focus of research because goats can transmit scrapie to sheep and may serve as a scrapie reservoir. Prior work showed that disease-free survival time was significantly extended in orally inoculated goats singly heterozygous for prion amino acid substitutions S146 or K222, but average durations were only around 3 years post-inoculation...
March 2018: Veterinary Journal
Frédéric Leroy, Malaika Brengman, Wouter Ryckbosch, Peter Scholliers
The debate on meat's role in health and disease is a rowdy and dissonant one. This study uses the health section of the online version of The Daily Mail as a case study to carry out a quantitative and qualitative reflection on the related discourses in mass media during the first fifteen years of the 21st century. This period ranged from the fall-out of the bovine spongiform encephalopathy (BSE) crisis and its associated food safety anxieties, over the Atkins diet-craze in 2003 and the avian flu episode in 2007, to the highly influential publication of the report on colon cancer by the International Agency for Research on Cancer (IARC) in 2015...
June 1, 2018: Appetite
Andreas E Steinhilber, Felix F Schmidt, Wael Naboulsi, Hannes Planatscher, Alicia Niedzwiecka, Jutta Zagon, Albert Braeuning, Alfonso Lampen, Thomas O Joos, Oliver Poetz
The ban of processed animal proteins (PAPs) in feed for farmed animals introduced in 2001 was one of the main EU measures to control the bovine spongiform encephalopathy (BSE) crisis. Currently, microscopy and polymerase chain reaction (PCR) are the official methods for the detection of illegal PAPs in feed. However, the progressive release of the feed ban, recently with the legalization of nonruminant PAPs for the use in aquaculture, requires the development of alternative methods to determine the species origin and the source (legal or not)...
March 20, 2018: Analytical Chemistry
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