Prions

Small proteinaceous infectious particles which resist inactivation by procedures that modify NUCLEIC ACIDS and contain an abnormal isoform of a cellular protein which is a major and necessary component. The abnormal (scrapie) isoform is PrPSc (PRPSC PROTEINS) and the cellular isoform PrPC (PRPC PROTEINS). The primary amino acid sequence of the two isoforms is identical. Human diseases caused by prions include CREUTZFELDT-JAKOB SYNDROME; GERSTMANN-STRAUSSLER SYNDROME; and INSOMNIA, FATAL FAMILIAL.

Also Known As:

Networked: 4777 relevant articles (106 outcomes, 448 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Collinge, John: 103 articles (01/2022 - 10/2002)
2.	Aguzzi, Adriano: 96 articles (01/2022 - 10/2000)
3.	Prusiner, Stanley B: 92 articles (01/2022 - 10/2000)
4.	Legname, Giuseppe: 65 articles (06/2022 - 03/2002)
5.	Caughey, Byron: 58 articles (01/2022 - 05/2005)
6.	Soto, Claudio: 55 articles (01/2022 - 08/2002)
7.	Brandner, Sebastian: 50 articles (01/2022 - 03/2003)
8.	Andréoletti, Olivier: 49 articles (01/2022 - 10/2002)
9.	Telling, Glenn C: 46 articles (01/2022 - 05/2004)
10.	Castilla, Joaquín: 46 articles (12/2021 - 05/2004)

Related Diseases

1.	Prion Diseases (Transmissible Spongiform Encephalopathies) 12/01/2016 - "CEs were significantly effective in other prion disease animal models; however, the effects were less remarkable than those observed in the 263K prion-infected animals. " 01/01/2016 - "Prions, the etiological agents in transmissible spongiform encephalopathies, exhibit remarkable resistance to most methods of inactivation that are effective against conventional pathogens. " 11/17/2006 - "Therefore, PMCA offers great promise for the development of highly sensitive, specific, and early diagnosis of transmissible spongiform encephalopathy and to further understand the molecular basis of prion propagation." 01/01/2021 - "KEY MESSAGES: Msr1 expression is upregulated in prion-infected mouse brains at the terminal stage Msr1 deficiency does not affect prion disease progression Msr1 does not play a major role in prion clearance or prion pathogenesis Microglia-mediated phagocytosis and clearance of Aβ and prion may adopt distinct molecular pathways." 01/01/2022 - "Uniquely, the entire prion disease process can be faithfully modeled through transmission of human prions to non-human primates (NHPs), raising the question of whether NHP models should be used to assess therapeutic efficacy. "
2.	Infections 06/27/2012 - "Here, we report that mice expressing PrP deleted for a short, polybasic region at the N terminus (residues 23-31) display a dramatically reduced susceptibility to prion infection and accumulate greatly reduced levels of PrP(Sc). " 08/16/2001 - "In cells treated with the most potent antibody, Fab D18, prion replication is abolished and pre-existing PrPSc is rapidly cleared, suggesting that this antibody may cure established infection. " 12/01/2021 - "Canine D163-PrP polymorphic variant does not provide complete protection against prion infection in small ruminant PrP context." 02/10/2015 - "Immunohistochemistry (IHC) is currently considered the gold standard for diagnosis of a prion infection but may be insensitive to early or sub-clinical CWD that are important to understanding CWD transmission and ecology. " 01/01/2021 - "Analysis of synaptic markers in ME7 prion mice revealed a significant reduction of genes that are associated with early response in synaptic plasticity, extracellular matrix structure and post-synaptic density, but no further reduction following systemic infection. "
3.	Scrapie 11/01/1994 - "High prion and PrPSc levels but delayed onset of disease in scrapie-inoculated mice heterozygous for a disrupted PrP gene." 09/01/2019 - "In this study we assessed the efficacy of sterilization using the EU recommended autoclave procedure for prions (133°C, 3 Bar for 20 min) on the atypical or Nor98 (AS/Nor98) scrapie strain of sheep and goats. " 02/21/2007 - "The present study is the first report describing effective amplification of PrP(Sc) of the mouse-adapted scrapie prion and this improved PMCA technique will contribute to prion research that uses mice as experimental animals." 03/01/2011 - "Animal transmission studies offer the potential for 'gold standard' confirmatory testing but are limited by both transmission barrier effects and known effects of fixation on scrapie prion titre in experimental models. " 01/13/2016 - "Previous experiments carried out in a sheep scrapie model demonstrated that the transfusion of 200 μl of prion-infected whole blood has an apparent 100% efficacy for disease transmission. "
4.	Neurodegenerative Diseases (Neurodegenerative Disease) 10/01/2018 - "Therefore, interaction of PAs with PrPC and enhancement of α-cleavage represent mechanisms that can be beneficial for the treatment of prion and other neurodegenerative diseases." 01/01/2018 - "Through this approach we aimed to identify natural yeast-prion inhibitors that could be useful in the development of novel treatment strategies for neurodegenerative disorders. " 02/01/2017 - "In this review, we illustrate the utility of cellular biosensors to gain mechanistic insight into prion transmission and to study neurodegenerative diseases in a reductionist fashion." 01/01/2017 - "Although recent studies have shown the dysregulation of RhoA in a variety of neurodegenerative diseases, the role of RhoA in prion pathogenesis remains unclear. " 10/01/2016 - "[The Prion-like Mechanism in Neurodegenerative Diseases-Current Studies and Future Prospects]."
5.	Alzheimer Disease (Alzheimer's Disease) 01/01/2012 - "Continuous production of prions after infectious particles are eliminated: implications for Alzheimer's disease." 09/02/2015 - "Recent functional studies uncovered a key role of endoplasmic reticulum (ER) stress and the unfolded protein response (UPR) in the occurrence of synaptic dysfunction and neurodegeneration in Prion-related disorders and Alzheimer's disease. " 04/01/2023 - "These concepts are applicable to prion-like diseases such as Parkinson's and Alzheimer's disease, where evidence of strain diversity is beginning to emerge." 03/13/2023 - "Channelizing microglia to target extracellular Tau could be a good strategy to combat the prion-like transmission and seeding problem in Alzheimer's disease. " 01/01/2023 - "Aβ and Tau Prions Causing Alzheimer's Disease."

Related Drugs and Biologics

1.	Proteins (Proteins, Gene)
2.	Biomarkers (Surrogate Marker)
3.	Prions
4.	Protein Isoforms (Isoforms)
5.	Vaccines
6.	Amyloid (Amyloid Fibrils)
7.	Protein Aggregates
8.	Antibodies
9.	Prion Proteins
10.	PrPSc Proteins

Related Therapies and Procedures

1.	Therapeutics
2.	Injections
3.	Blood Transfusion (Blood Transfusions)
4.	Immunotherapy
5.	Intraperitoneal Injections