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Prion Related Diseases

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The cellular prion protein promotes neuronal regeneration after acute nasotoxic injury.

Lindsay E Parrie, Jenna A E Crowell, Julie A Moreno, Stephanie S Suinn, Glenn C Telling, Richard A Bessen,

Adult neurogenesis, analogous to early development, is comprised of several, often concomitant, processes including proliferation, differentiation, and formation of synaptic connections. However, due to continual, asynchronous turn-over, newly-born adult olfactory sensory neurons (OSNs) must integrate into existing circuitry. Additionally, OSNs express high levels of cellular prion protein (PrPC), particularly in ... Read more >>

Prion (Prion)
[2020, 14(1):31-41]

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Predicting the spread-risk potential of chronic wasting disease to sympatric ungulate species.

Catherine I Cullingham, Rhiannon M Peery, Anh Dao, Debbie I McKenzie, David W Coltman,

Wildlife disease incidence is increasing, resulting in negative impacts on the economy, biodiversity, and potentially human health. Chronic wasting disease (CWD) is a fatal, transmissible spongiform encephalopathy of cervids (wild and captive) which continues to spread geographically resulting in exposure to potential new host species. The disease agent (PrPCWD) is ... Read more >>

Prion (Prion)
[2020, 14(1):56-66]

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Cross-validation of the RT-QuIC assay for the antemortem detection of chronic wasting disease in elk.

N J Haley, R Donner, D M Henderson, J Tennant, E A Hoover, M Manca, B Caughey, N Kondru, S Manne, A Kanthasamay, S Hannaoui, S C Chang, S Gilch, S Smiley, G Mitchell, A D Lehmkuhl, B V Thomsen,

Chronic wasting disease is a progressively fatal, horizontally transmissible prion disease affecting several members of the cervid species. Conventional diagnosis relies on ELISA or IHC evaluation using tissues collected post-mortem; however, recent research has focused on newly developed amplification techniques using samples collected antemortem. The present study sought to cross-validate ... Read more >>

Prion (Prion)
[2020, 14(1):47-55]

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Chronic wasting disease associated with prion protein gene (PRNP) variation in Norwegian wild reindeer (Rangifer tarandus).

Mariella E Güere, Jørn Våge, Helene Tharaldsen, Sylvie L Benestad, Turid Vikøren, Knut Madslien, Petter Hopp, Christer M Rolandsen, Knut H Røed, Michael A Tranulis,

The emergence of CWD in Europe in 2016 and the first natural infection in wild reindeer warranted disease management. This led to the testing of 2424 hunted or culled reindeer during 2016-2018, from the infected subpopulation in the Nordfjella mountain range in Southern Norway. To identify any association between PRNP ... Read more >>

Prion (Prion)
[2020, 14(1):1-10]

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Carbonic anhydrase IX as a novel candidate in liquid biopsy.

Ozen Ozensoy Guler, Claudiu T Supuran, Clemente Capasso,

Among the diagnostic techniques for the identification of tumour biomarkers, the liquid biopsy is considered one that offers future research on precision diagnosis and treatment of tumours in a non-invasive manner. The approach consists of isolating tumor-derived components, such as circulating tumour cells (CTC), tumour cell-free DNA (ctDNA), and extracellular ... Read more >>

J Enzyme Inhib Med Chem (Journal of enzyme inhibition and medicinal chemistry)
[2020, 35(1):255-260]

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Cerebrospinal fluid neurogranin as a new player in prion disease diagnosis and prognosis.

Anna Villar-Pique, Inga Zerr, Franc Llorens,

Neural Regen Res (Neural regeneration research)
[2020, 15(5):861-862]

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Prion protein lowering is a disease-modifying therapy across prion strains, disease stages, and endpoints

Eric Vallabh Minikel, Hien Zhao, Jason Le, Jill O'Moore, Rose Pitstick, Samantha Graffam, George Carlson, Jasna Kriz, Jae Beom Kim, Jiyan Ma, Holger Wille, Judd Aiken, Deborah McKenzie, Katsumi Doh-ura, Matthew Beck, Rhonda O'Keefe, Jacqueline Stathopoulos, Tyler Caron, Stuart Schreiber, Jeffrey Carroll, Holly Kordasiewicz, Deborah Cabin, Sonia Vallabh,

Lowering of prion protein (PrP) expression in the brain is a genetically validated therapeutic hypothesis in prion disease. We recently showed that antisense oligonucleotide (ASO)-mediated PrP suppression extends survival and delays disease onset in intracerebrally prion-infected mice in both prophylactic and delayed dosing paradigms. Here, we examine the efficacy of ... Read more >>

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CSF Ubiquitin Levels Are Higher in Alzheimer's Disease than in Frontotemporal Dementia and Reflect the Molecular Subtype in Prion Disease.

Samir Abu-Rumeileh, Patrick Oeckl, Simone Baiardi, Steffen Halbgebauer, Petra Steinacker, Sabina Capellari, Markus Otto, Piero Parchi,

Disturbances in the ubiquitin-proteasome system seem to play a role in neurodegenerative dementias (NDs). Previous studies documented an increase of cerebrospinal fluid (CSF) free monoubiquitin in Alzheimer's disease (AD) and Creutzfeldt-Jakob disease (CJD). However, to date, no study explored this biomarker across the heterogeneous spectrum of prion disease. Using a ... Read more >>

Biomolecules (Biomolecules)
[2020, 10(4):]

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Transcriptomic Analysis Reveals Abnormal Expression of Prion Disease Gene Pathway in Brains from Patients with Autism Spectrum Disorders.

Salvo Danilo Lombardo, Giuseppe Battaglia, Maria Cristina Petralia, Katia Mangano, Maria Sofia Basile, Valeria Bruno, Paolo Fagone, Rita Bella, Ferdinando Nicoletti, Eugenio Cavalli,

The role of infections in the pathogenesis of autism spectrum disorder (ASD) is still controversial. In this study, we aimed to evaluate markers of infections and immune activation in ASD by performing a meta-analysis of publicly available whole-genome transcriptomic datasets of brain samples from autistic patients and otherwise normal people. ... Read more >>

Brain Sci (Brain sciences)
[2020, 10(4):]

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DMSO-mediated curing of several yeast prion variants involves Hsp104 expression and protein solubilization, and is decreased in several autophagy related gene (atg) mutants.

Jane E Dorweiler, Joanna O Obaoye, Mitch J Oddo, Francesca M Shilati, Grace M Scheidemantle, Thomas J Coleman, Jacob A Reilly, Gregory R Smith, Anita L Manogaran,

Chaperones and autophagy are components of the protein quality control system that contribute to the management of proteins that are misfolded and aggregated. Here, we use yeast prions, which are self-perpetuating aggregating proteins, as a means to understand how these protein quality control systems influence aggregate loss. Chaperones, such as ... Read more >>

PLoS ONE (PloS one)
[2020, 15(3):e0229796]

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Five Novel PRNP Gene Polymorphisms and Their Potential Effect on Scrapie Susceptibility in Three Native Ethiopian Sheep Breeds 

Cemal Un, Eden Teferedegn, Yalcin Yaman,

<title>Abstract</title> <p> <bold>Background: </bold>Classical scrapie susceptibility in sheep has been linked to three polymorphisms at codon 136, 154, and 171 in the prion protein gene (<italic>PRNP)</italic> whereas atypical scrapie susceptibility is related to polymorphisms at position 141. Many other variants over the length of the <italic>PRNP</italic> have been ... Read more >>

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Protein assembly systems in natural and synthetic biology.

Giulio Chiesa, Szilvia Kiriakov, Ahmad S Khalil,

The traditional view of protein aggregation as being strictly disease-related has been challenged by many examples of cellular aggregates that regulate beneficial biological functions. When coupled with the emerging view that many regulatory proteins undergo phase separation to form dynamic cellular compartments, it has become clear that supramolecular assembly plays ... Read more >>

BMC Biol. (BMC biology)
[2020, 18(1):35]

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A probable role of copper in the comorbidity in Wilson's and Creutzfeldt-Jakob's Diseases: a case report.

Effrosyni Koutsouraki, Dimitrios Michmizos, Olga Patsi, John Tzartos, Martha Spilioti, Marianthi Arnaoutoglou, Magda Tsolaki,

BACKGROUND:To the best of our knowledgedd, there is currently no case in the literature reporting the comorbidity of Wilson's and Creutzfeldt-Jakob disease (CJD), linked through copper. CASE PRESENTATION:A 44-year-old male with a history of inherited Wilson's disease (hepatolenticular degeneration), which manifested as mild liver injury and psychiatric symptoms, was admitted ... Read more >>

Virol. J. (Virology journal)
[2020, 17(1):35]

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Prion protein-mediated calcium level alteration governs neuronal cell damage through AMPK-autophagy flux

Sang-Youel Park, Ji-Hong Moon,

<title>Abstract</title> <p> Background The distinctive molecular structure of the prion protein, PrPsc, is established only in mammals with infectious prion diseases. Prion protein characterizes either the transmissible pathogen itself or a primary constituent of the disease. Our report suggested that prion-mediated neuronal cell death is triggered by the ... Read more >>

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Development and structural determination of an anti-PrPC aptamer that blocks pathological conformational conversion of prion protein.

Tsukasa Mashima, Joon-Hwa Lee, Yuji O Kamatari, Tomohiko Hayashi, Takashi Nagata, Fumiko Nishikawa, Satoshi Nishikawa, Masahiro Kinoshita, Kazuo Kuwata, Masato Katahira,

Prion diseases comprise a fatal neuropathy caused by the conversion of prion protein from a cellular (PrPC) to a pathological (PrPSc) isoform. Previously, we obtained an RNA aptamer, r(GGAGGAGGAGGA) (R12), that folds into a unique G-quadruplex. The R12 homodimer binds to a PrPC molecule, inhibiting PrPC-to-PrPSc conversion. Here, we developed ... Read more >>

Sci Rep (Scientific reports)
[2020, 10(1):4934]

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Combination of copper ions and nucleotide generates aggregates from prion protein fragments in the N-terminal domain.

Noriyuki Shiraishi, Yoshiaki Hirano,

BACKGROUND:It has been previously found that PrP23-98, which contains four highly conserved octarepeats (residues 60-91) and one partial repeat (residues 92-96), polymerizes into amyloid-like and proteinase K-resistant spherical aggregates in the presence of NADPH plus copper ions. OBJECTIVE:We aimed to determine the requirements for the formation of these aggregates. METHODS:In ... Read more >>

Protein Pept. Lett. (Protein and peptide letters)
[2020, :]

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CSF SerpinA1 in Creutzfeldt-Jakob disease and frontotemporal lobar degeneration.

Samir Abu-Rumeileh, Steffen Halbgebauer, Petra Steinacker, Sarah Anderl-Straub, Barbara Polischi, Albert C Ludolph, Sabina Capellari, Piero Parchi, Markus Otto,

OBJECTIVE:SerpinA1 (alpha-1 antitrypsin) is an acute inflammatory protein, which seems to play a role in neurodegeneration and neuroinflammation. In Alzheimer's disease and synucleinopathies, SerpinA1 is overexpressed in the brain and the cerebrospinal fluid (CSF) showing abnormal patterns of its charge isoforms. To date, no comprehensive studies explored SerpinA1 CSF isoforms in ... Read more >>

Ann Clin Transl Neurol (Annals of clinical and translational neurology)
[2020, 7(2):191-199]

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Exosomes as mediators of neuron-glia communication in neuroinflammation.

María Pascual, Francesc Ibáñez, Consuelo Guerri,

In recent years, a type of extracellular vesicles named exosomes has emerged that play an important role in intercellular communication under physiological and pathological conditions. These nanovesicles (30-150 nm) contain proteins, RNAs and lipids, and their internalization by bystander cells could alter their normal functions. This review focuses on recent ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(5):796-801]

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Unraveling the Molecular Mechanism of Prion H2 C-Terminus Misfolding by Metadynamics Simulations.

Zerong Xu, Hongli Liu, Shuo Wang, Qianqian Zhang, Xiaojun Yao, Shuangyan Zhou, Huanxiang Liu,

Conformational transition from the normal cellular form of prion protein (PrPC) to the pathogenic "scrapie" form (PrPSc) is considered to be a key event in the occurrence of prion disease. Additionally, the H2 C-terminus is widely considered to be a vital site for PrP conformational transition, which can be used ... Read more >>

ACS Chem Neurosci (ACS chemical neuroscience)
[2020, 11(5):772-782]

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Diagnostic Accuracy of Prion Disease Biomarkers in Iatrogenic Creutzfeldt-Jakob Disease.

Franc Llorens, Anna Villar-Piqué, Peter Hermann, Matthias Schmitz, Olga Calero, Christiane Stehmann, Shannon Sarros, Fabio Moda, Isidre Ferrer, Anna Poleggi, Maurizio Pocchiari, Marcella Catania, Sigrid Klotz, Carl O'Regan, Francesca Brett, Josephine Heffernan, Anna Ladogana, Steven J Collins, Miguel Calero, Gabor G Kovacs, Inga Zerr,

Human prion diseases are classified into sporadic, genetic, and acquired forms. Within this last group, iatrogenic Creutzfeldt-Jakob disease (iCJD) is caused by human-to-human transmission through surgical and medical procedures. After reaching an incidence peak in the 1990s, it is believed that the iCJD historical period is probably coming to an ... Read more >>

Biomolecules (Biomolecules)
[2020, 10(2):]

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Core transcriptional regulatory circuits in prion diseases.

Taek-Kyun Kim, Inyoul Lee, Ji-Hoon Cho, Brenda Canine, Andrew Keller, Nathan D Price, Daehee Hwang, George Carlson, Leroy Hood,

Complex diseases involve dynamic perturbations of pathophysiological processes during disease progression. Transcriptional programs underlying such perturbations are unknown in many diseases. Here, we present core transcriptional regulatory circuits underlying early and late perturbations in prion disease. We first identified cellular processes perturbed early and late using time-course gene expression data ... Read more >>

Mol Brain (Molecular brain)
[2020, 13(1):10]

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3-Nitrotyrosine: a versatile oxidative stress biomarker for major neurodegenerative diseases.

Maria Bandookwala, Pinaki Sengupta,

Reactive oxygen species are generated as a by-product of routine biochemical reactions. However, dysfunction of the antioxidant system or mutations in gene function may result in the elevated production of the pro-oxidant species. Modified endogenous molecules due to chemical interactions with increased levels of reactive oxygen and nitrogen species in ... Read more >>

Int. J. Neurosci. (The International journal of neuroscience)
[2020, :1-16]

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Goats naturally devoid of PrPC are resistant to scrapie.

Øyvind Salvesen, Arild Espenes, Malin R Reiten, Tram T Vuong, Giulia Malachin, Linh Tran, Olivier Andréoletti, Ingrid Olsaker, Sylvie L Benestad, Michael A Tranulis, Cecilie Ersdal,

Prion diseases are progressive and fatal, neurodegenerative disorders described in humans and animals. According to the "protein-only" hypothesis, the normal host-encoded prion protein (PrPC) is converted into a pathological and infectious form (PrPSc) in these diseases. Transgenic knockout models have shown that PrPC is a prerequisite for the development of ... Read more >>

Vet. Res. (Veterinary research)
[2020, 51(1):1]

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The PERK-Dependent Molecular Mechanisms as a Novel Therapeutic Target for Neurodegenerative Diseases.

Wioletta Rozpędek-Kamińska, Natalia Siwecka, Adam Wawrzynkiewicz, Radosław Wojtczak, Dariusz Pytel, J Alan Diehl, Ireneusz Majsterek,

Higher prevalence of neurodegenerative diseases is strictly connected with progressive aging of the world population. Interestingly, a broad range of age-related, neurodegenerative diseases is characterized by a common pathological mechanism-accumulation of misfolded and unfolded proteins within the cells. Under certain circumstances, such protein aggregates may evoke endoplasmic reticulum (ER) stress ... Read more >>

Int J Mol Sci (International journal of molecular sciences)
[2020, 21(6):]

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Co-existence of PrPD types 1 and 2 in sporadic Creutzfeldt-Jakob disease of the VV subgroup: phenotypic and prion protein characteristics.

Ignazio Cali, Gianfranco Puoti, Jason Smucny, Paul Michael Curtiss, Laura Cracco, Tetsuyuki Kitamoto, Rossana Occhipinti, Mark Lloyd Cohen, Brian Stephen Appleby, Pierluigi Gambetti,

We report a detailed study of a cohort of sporadic Creutzfeldt-Jakob disease (sCJD) VV1-2 type-mixed cases (valine homozygosity at codon 129 of the prion protein, PrP, gene harboring disease-related PrP, PrPD, types 1 and 2). Overall, sCJDVV1-2 subjects showed mixed clinical and histopathological features, which often correlated with the relative ... Read more >>

Sci Rep (Scientific reports)
[2020, 10(1):1503]

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