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https://www.readbyqxmd.com/read/28102851/cellular-prion-protein-mediates-early-apoptotic-proteome-alternation-and-phospho-modification-in-human-neuroblastoma-cells
#1
Saima Zafar, Christina Behrens, Hassan Dihazi, Matthias Schmitz, Inga Zerr, Walter J Schulz-Schaeffer, Sanja Ramljak, Abdul R Asif
Anti-apoptotic properties of physiological and elevated levels of the cellular prion protein (PrP(c)) under stress conditions are well documented. Yet, detrimental effects of elevated PrP(c) levels under stress conditions, such as exposure to staurosporine (STS) have also been described. In the present study, we focused on discerning early apoptotic STS-induced proteome and phospho-proteome changes in SH-SY5Y human neuroblastoma cells stably transfected either with an empty or PRNP-containing vector, expressing physiological or supraphysiological levels of PrP(c), respectively...
January 19, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28102071/temperature-induced-misfolding-in-prion-protein-evidences-of-multiple-partially-disordered-states-stabilized-by-non-native-hydrogen-bonds
#2
Neharika G Chamachi, Suman Chakrabarty
The structural basis of misfolding pathways of a cellular Prion (PrP(C)) into the toxic scrapie form (PrP(SC)) and identification of possible intermediates (e.g. PrP(*)) still eludes us. In this work, we have used a cumulative ~65µs of Replica Exchange Molecular Dynamics simulation data to construct the conformational free energy landscapes and capture the structural and thermodynamic characteristics associated with various stages of the thermal denaturation process in human Prion protein. The temperature dependent free energy surfaces consist of multiple metastable states stabilized by non-native contacts and hydrogen bonds, thus rendering the protein prone towards misfolding...
January 19, 2017: Biochemistry
https://www.readbyqxmd.com/read/28099852/yeast-short-lived-actin-associated-protein-forms-a-metastable-prion-in-response-to-thermal-stress
#3
Tatiana A Chernova, Denis A Kiktev, Andrey V Romanyuk, John R Shanks, Oskar Laur, Moiez Ali, Abheek Ghosh, Dami Kim, Zhen Yang, Maggie Mang, Yury O Chernoff, Keith D Wilkinson
Self-perpetuating ordered protein aggregates (amyloids and prions) are associated with a variety of neurodegenerative disorders. Although environmental agents have been linked to certain amyloid diseases, the molecular basis of their action remains unclear. We have employed endogenous yeast prions as a model system to study environmental control of amyloid formation. A short-lived actin-associated yeast protein Lsb2 can trigger prion formation by other proteins in a mode regulated by the cytoskeleton and ubiquitin-dependent processes...
January 17, 2017: Cell Reports
https://www.readbyqxmd.com/read/28098532/oral-transmission-of-l-type-bovine-spongiform-encephalopathy-agent-among-cattle
#4
Hiroyuki Okada, Yoshifumi Iwamaru, Morikazu Imamura, Kohtaro Miyazawa, Yuichi Matsuura, Kentaro Masujin, Yuichi Murayama, Takashi Yokoyama
To determine oral transmissibility of the L-type bovine spongiform encephalopathy (BSE) prion, we orally inoculated 16 calves with brain homogenates of the agent. Only 1 animal, given a high dose, showed signs and died at 88 months. These results suggest low risk for oral transmission of the L-BSE agent among cattle.
February 2017: Emerging Infectious Diseases
https://www.readbyqxmd.com/read/28098160/a-zip6-zip10-heteromer-controls-ncam1-phosphorylation-and-integration-into-focal-adhesion-complexes-during-epithelial-to-mesenchymal-transition
#5
Dylan Brethour, Mohadeseh Mehrabian, Declan Williams, Xinzhu Wang, Farinaz Ghodrati, Sepehr Ehsani, Elizabeth A Rubie, James R Woodgett, Jean Sevalle, Zhengrui Xi, Ekaterina Rogaeva, Gerold Schmitt-Ulms
The prion protein (PrP) evolved from the subbranch of ZIP metal ion transporters comprising ZIPs 5, 6 and 10, raising the prospect that the study of these ZIPs may reveal insights relevant for understanding the function of PrP. Building on data which suggested PrP and ZIP6 are critical during epithelial-to-mesenchymal transition (EMT), we investigated ZIP6 in an EMT paradigm using ZIP6 knockout cells, mass spectrometry and bioinformatic methods. Reminiscent of PrP, ZIP6 levels are five-fold upregulated during EMT and the protein forms a complex with NCAM1...
January 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28096357/prion-replication-without-host-adaptation-during-interspecies-transmissions
#6
Jifeng Bian, Vadim Khaychuk, Rachel C Angers, Natalia Fernández-Borges, Enric Vidal, Crystal Meyerett-Reid, Sehun Kim, Carla L Calvi, Jason C Bartz, Edward A Hoover, Umberto Agrimi, Jürgen A Richt, Joaquín Castilla, Glenn C Telling
Adaptation of prions to new species is thought to reflect the capacity of the host-encoded cellular form of the prion protein (PrP(C)) to selectively propagate optimized prion conformations from larger ensembles generated in the species of origin. Here we describe an alternate replicative process, termed nonadaptive prion amplification (NAPA), in which dominant conformers bypass this requirement during particular interspecies transmissions. To model susceptibility of horses to prions, we produced transgenic (Tg) mice expressing cognate PrP(C) Although disease transmission to only a subset of infected TgEq indicated a significant barrier to EqPrP(C) conversion, the resulting horse prions unexpectedly failed to cause disease upon further passage to TgEq...
January 17, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28096267/toward-the-atomic-structure-of-prpsc
#7
Jose A Rodriguez, Lin Jiang, David S Eisenberg
In this review, we detail our current knowledge of PrP(Sc) structure on the basis of structural and computational studies. We discuss the progress toward an atomic resolution description of PrP(Sc) and results from the broader field of amyloid studies that may further inform our knowledge of this structure. Moreover, we summarize work that investigates the role of PrP(Sc) structure in its toxicity, transmissibility, and species specificity. We look forward to an atomic model of PrP(Sc), which is expected to bring diagnostics and/or therapeutics to the field of prion disease...
January 17, 2017: Cold Spring Harbor Perspectives in Biology
https://www.readbyqxmd.com/read/28096265/prion-properties-of-sod1-in-amyotrophic-lateral-sclerosis-and-potential-therapy
#8
Caroline Sibilla, Anne Bertolotti
Amyotrophic lateral sclerosis (ALS) is a devastating and rapidly progressive neurodegenerative disease caused by the deterioration of motor neurons. The first symptoms of ALS always begin at a focal but variable site and consistently spread to neighboring regions, suggesting that neurodegeneration in ALS is an orderly and propagating process. Like other neurodegenerative diseases, misfolding of a specific protein is central to ALS. SOD1, the major constituent of the protein deposits in some familial and sporadic forms of ALS, propagates its misfolded conformation like prions, providing a plausible molecular basis for the focality and spreading of muscle weakness in ALS...
January 17, 2017: Cold Spring Harbor Perspectives in Biology
https://www.readbyqxmd.com/read/28096246/chronic-traumatic-encephalopathy-is-latency-in-symptom-onset-explained-by-tau-propagation
#9
Joshua Kriegel, Zachary Papadopoulos, Ann C McKee
Chronic traumatic encephalopathy (CTE) is a neurodegenerative tauopathy associated with repetitive mild brain trauma. CTE, previously termed "dementia pugilistica," has been identified in American football, ice hockey, baseball, rugby and soccer players, boxers, wrestlers, and military personnel exposed to blast and other traumatic brain injuries. There is often a long latency period between an individual's exposure to repetitive brain trauma and the clinical symptoms of CTE. The pathology of CTE is characterized by a progression from isolated focal perivascular hyperphosphorylated tau lesions in the cerebral cortex to a widespread tauopathy that involves diffuse cortical and medial temporal lobe regions...
January 17, 2017: Cold Spring Harbor Perspectives in Medicine
https://www.readbyqxmd.com/read/28096245/prion-like-characteristics-of-polyglutamine-containing-proteins
#10
Margaret M P Pearce, Ron R Kopito
Transmissible spongiform encephalopathies are infectious neurodegenerative diseases caused by the conversion of prion protein (PrP) into a self-replicating conformation that spreads via templated conversion of natively folded PrP molecules within or between cells. Recent studies provide compelling evidence that prion-like behavior is a general property of most protein aggregates associated with neurodegenerative diseases. Many of these disorders are associated with spontaneous protein aggregation, but genetic mutations can increase the aggregation propensity of specific proteins, including expansion of polyglutamine (polyQ) tracts, which is causative of nine inherited neurodegenerative diseases...
January 17, 2017: Cold Spring Harbor Perspectives in Medicine
https://www.readbyqxmd.com/read/28096244/experimental-models-of-inherited-prp-prion-diseases
#11
Joel C Watts, Stanley B Prusiner
The inherited prion protein (PrP) prion disorders, which include familial Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker disease, and fatal familial insomnia, constitute ∼10%-15% of all PrP prion disease cases in humans. Attempts to generate animal models of these disorders using transgenic mice expressing mutant PrP have produced variable results. Although many lines of mice develop spontaneous signs of neurological illness with accompanying prion disease-specific neuropathological changes, others do not...
January 17, 2017: Cold Spring Harbor Perspectives in Medicine
https://www.readbyqxmd.com/read/28096242/developing-therapeutics-for-prp-prion-diseases
#12
Kurt Giles, Steven H Olson, Stanley B Prusiner
The prototypical PrP prion diseases are invariably fatal, and the search for agents to treat them spans more than 30 years, with limited success. However, in the last few years, the application of high-throughput screening, medicinal chemistry, and pharmacokinetic optimization has led to important advances. The PrP prion inoculation paradigm provides a robust assay for testing therapeutic efficacy, and a dozen compounds have been reported that lead to meaningful extension in survival of prion-infected mice...
January 17, 2017: Cold Spring Harbor Perspectives in Medicine
https://www.readbyqxmd.com/read/28095625/potentiation-of-biological-effects-of-mesenchymal-stem-cells-in-ischemic-conditions-by-melatonin-via-upregulation-of-cellular-prion-protein-expression
#13
Jun Hee Lee, Yong-Seok Han, Sang Hun Lee
Mesenchymal stem cells (MSCs) are promising candidates for stem cell-based therapy in ischemic diseases. However, ischemic injury induces pathophysiological conditions, such as oxidative stress and inflammation, which diminish therapeutic efficacy of MSC-based therapy by reducing survival and functionality of transplanted MSCs. To overcome this problem, we explored the effects of melatonin on the proliferation, resistance to oxidative stress, and immunomodulatory properties of MSCs. Treatment with melatonin enhanced MSC proliferation and self-renewal via upregulation of cellular prion protein (PrP(C) ) expression...
January 17, 2017: Journal of Pineal Research
https://www.readbyqxmd.com/read/28095474/anti-prion-screening-for-acridine-dextran-and-tannic-acid-using-real-time-quaking-induced-conversion-a-comparison-with-prpsc-infected-cell-screening
#14
Jae Wook Hyeon, Su Yeon Kim, Sol Moe Lee, Jeongmin Lee, Seong Soo A An, Myung Koo Lee, Yeong Seon Lee
Prion propagation is mediated by the structural alteration of normal prion protein (PrPC) to generate pathogenic prion protein (PrPSc). To date, compounds for the inhibition of prion propagation have mainly been screened using PrPSc-infected cells. Real time-quaking-induced conversion (RT-QuIC) is one alternative screening method. In this study, we assessed the propagation inhibition effects of known anti-prion compounds using RT-QuIC and compared the results with those from a PrPSc-infected cell assay. Compounds were applied to RT-QuIC reactions at 0 h or 22 h after prion propagation to determine whether they inhibited propagation or reduced amplified aggregates...
2017: PloS One
https://www.readbyqxmd.com/read/28091514/novel-strain-properties-distinguishing-sporadic-prion-diseases-sharing-prion-protein-genotype-and-prion-type
#15
Laura Cracco, Silvio Notari, Ignazio Cali, Man-Sun Sy, Shu G Chen, Mark L Cohen, Bernardino Ghetti, Brian S Appleby, Wen-Quan Zou, Byron Caughey, Jiri G Safar, Pierluigi Gambetti
In most human sporadic prion diseases the phenotype is consistently associated with specific pairings of the genotype at codon 129 of the prion protein gene and conformational properties of the scrapie PrP (PrP(Sc)) grossly identified types 1 and 2. This association suggests that the 129 genotype favours the selection of a distinct strain that in turn determines the phenotype. However, this mechanism cannot play a role in the phenotype determination of sporadic fatal insomnia (sFI) and a subtype of sporadic Creutzfeldt-Jakob disease (sCJD) identified as sCJDMM2, which share 129 MM genotype and PrP(Sc) type 2 but are associated with quite distinct phenotypes...
January 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28087950/amyloid-prions-in-fungi
#16
Sven J Saupe, Daniel F Jarosz, Heather L True
Prions are infectious protein polymers that have been found to cause fatal diseases in mammals. Prions have also been identified in fungi (yeast and filamentous fungi), where they behave as cytoplasmic non-Mendelian genetic elements. Fungal prions correspond in most cases to fibrillary β-sheet-rich protein aggregates termed amyloids. Fungal prion models and, in particular, yeast prions were instrumental in the description of fundamental aspects of prion structure and propagation. These models established the "protein-only" nature of prions, the physical basis of strain variation, and the role of a variety of chaperones in prion propagation and amyloid aggregate handling...
December 2016: Microbiology Spectrum
https://www.readbyqxmd.com/read/28081197/morphine-withdrawal-modifies-prion-protein-expression-in-rat-hippocampus
#17
Vincenzo Mattei, Stefano Martellucci, Francesca Santilli, Valeria Manganelli, Tina Garofalo, Niccolò Candelise, Alessandra Caruso, Maurizio Sorice, Sergio Scaccianoce, Roberta Misasi
The hippocampus is a vulnerable brain structure susceptible to damage during aging and chronic stress. Repeated exposure to opioids may alter the brain so that it functions normally when the drugs are present, thus, a prolonged withdrawal might lead to homeostatic changes headed for the restoration of the physiological state. Abuse of morphine may lead to Reacting Oxygen Species-induced neurodegeneration and apoptosis. It has been proposed that during morphine withdrawal, stress responses might be responsible, at least in part, for long-term changes of hippocampal plasticity...
2017: PloS One
https://www.readbyqxmd.com/read/28077650/melanin-or-melanin-like-substance-interacts-with-the-n-terminal-portion-of-prion-protein-and-inhibits-abnormal-prion-protein-formation-in-prion-infected-cells
#18
Taichi Hamanaka, Keiko Nishizawa, Yuji Sakasegawa, Ayumi Oguma, Kenta Teruya, Hiroshi Kurahashi, Hideyuki Hara, Suehiro Sakaguchi, Katsumi Doh-Ura
Prion diseases are progressive fatal neurodegenerative illnesses caused by the accumulation of transmissible abnormal prion protein (PrP). To find treatments for prion diseases, we searched for substances from natural resources that inhibit abnormal PrP formation in prion-infected cells. We found that high-molecular-weight components from insect cuticle extracts reduced abnormal PrP levels. The chemical nature of these components was consistent with that of melanin. In fact, synthetic melanin produced from tyrosine or 3-hydroxy-l-tyrosine inhibited abnormal PrP formation...
January 11, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28076789/reduced-insulin-igf-1-signaling-restores-the-dynamic-properties-of-key-stress-granule-proteins-during-aging
#19
Marie C Lechler, Emily D Crawford, Nicole Groh, Katja Widmaier, Raimund Jung, Janine Kirstein, Jonathan C Trinidad, Alma L Burlingame, Della C David
Low-complexity "prion-like" domains in key RNA-binding proteins (RBPs) mediate the reversible assembly of RNA granules. Individual RBPs harboring these domains have been linked to specific neurodegenerative diseases. Although their aggregation in neurodegeneration has been extensively characterized, it remains unknown how the process of aging disturbs RBP dynamics. We show that a wide variety of RNA granule components, including stress granule proteins, become highly insoluble with age in C. elegans and that reduced insulin/insulin-like growth factor 1 (IGF-1) daf-2 receptor signaling efficiently prevents their aggregation...
January 10, 2017: Cell Reports
https://www.readbyqxmd.com/read/28076019/viral-and-prion-infections-of-the-central-nervous-system-radiologic-pathologic-correlation-from-the-radiologic-pathology-archives
#20
Kelly K Koeller, Robert Y Shih
Viral infections of the central nervous system (CNS) range in clinical severity, with the most severe proving fatal within a matter of days. Some of the more than 100 different viruses known to affect the brain and spinal cord are neurotropic with a predilection for producing CNS infection. The host response to viral infection of the CNS is responsible for the pathophysiology and imaging findings seen in affected patients. Viral CNS infections can take the form of meningitis, encephalitis, encephalomyelitis, or, when involving the spinal cord and nerve roots, encephalomyeloradiculitis...
January 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
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