Prion Proteins

Membrane glycosylphosphatidylinositol-anchored glycoproteins that may aggregate into rod-like structures. The prion protein (PRNP) gene is characterized by five TANDEM REPEAT SEQUENCES that encode a highly unstable protein region of five octapeptide repeats. Mutations in the repeat region and elsewhere in this gene are associated with CREUTZFELDT-JAKOB DISEASE; FATAL FAMILIAL INSOMNIA; GERSTMANN-STRAUSSLER DISEASE; Huntington disease-like 1, and KURU.

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Networked: 5416 relevant articles (33 outcomes, 331 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Collinge, John: 93 articles (10/2022 - 01/2002)
2.	Aguzzi, Adriano: 81 articles (11/2021 - 03/2002)
3.	Zerr, Inga: 68 articles (01/2022 - 01/2002)
4.	Kitamoto, Tetsuyuki: 67 articles (03/2022 - 06/2002)
5.	Ironside, James W: 60 articles (01/2021 - 01/2002)
6.	Yokoyama, Takashi: 57 articles (07/2019 - 05/2002)
7.	Gambetti, Pierluigi: 56 articles (10/2021 - 09/2002)
8.	Prusiner, Stanley B: 54 articles (05/2021 - 10/2000)
9.	Mohri, Shirou: 50 articles (10/2021 - 06/2002)
10.	Parchi, Piero: 49 articles (01/2022 - 04/2003)

Related Diseases

1.	Prion Diseases (Transmissible Spongiform Encephalopathies) 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. " 11/07/2017 - "In particular, it has been shown that the G127V mutation in this region of the human prion protein (huPrP) is protective against the spread of prion disease, but the mechanism of protection remains unknown. " 07/01/2014 - "Reversible symptoms and clearance of mutant prion protein in an inducible model of a genetic prion disease in Drosophila melanogaster." 07/01/2002 - "For definite histopathological diagnosis of a human prion disease, immunohistochemical detection of the prion protein deposits is the gold standard. " 09/01/1995 - "The delayed onset of CJD has complicated the analysis of inherited forms of the illness and led to the suggestion that mutations in the prion protein (PrP) gene are necessary but not sufficient for prion disease despite genetic linkage; indeed, an environmental factor such as a ubiquitous virus has been proposed as a second necessary factor. "
2.	Scrapie 12/01/2011 - "Genetic breeding programs aimed at enrichment of arginine-171 (171R) prion protein (PrP), the so-called ARR allele, in the sheep population have been demonstrated to be effective in reducing the occurrence of classical scrapie in the field. " 01/01/2021 - "Large-scale survey of prion protein genetic variability in scrapie disease-free goats from the United States." 07/01/2010 - "Models have been used to investigate the epidemiology of scrapie within individual flocks and at a regional level; to assess the efficacy of different control strategies, especially selective breeding programmes based on prion protein (PrP) genotype; to interpret the results of scrapie surveillance; and to inform the design of surveillance programmes. " 01/01/2020 - "To faithfully study scrapie strain variability in natural sheep isolates, transgenic mice expressing sheep cellular prion protein (PrPC) are used. " 01/01/2017 - "In our previous study, we demonstrated the propagation of mouse-passaged scrapie isolates with long incubation periods (L-type) derived from natural Japanese sheep scrapie cases in murine hypothalamic GT1-7 cells, along with disease-associated prion protein (PrPSc) accumulation. "
3.	Infections 03/13/2012 - "Alternatively, the differences between ID(50) and PMCAb(50) could be due to higher rate of clearance of infection-specific prion protein seeds in animals versus PMCAb reactions. " 11/01/2012 - "In this study, we show that mice overexpressing the cervid prion protein and susceptible to CWD (Tg(cerPrP)5037 mice) but lack CD21/35 expression completely resist clinical CWD upon peripheral infection. " 01/01/2007 - "It is proposed that physiological study of disrupted vagal function using techniques such as heart rate variability will indicate early, and ongoing, functional signs of infection even before levels of abnormal prion protein reach the thresholds currently used in tests for the presence of TSEs. " 01/28/2021 - "The conformational conversion of normal cellular prion protein (PrPC) into abnormal pathogenic prion protein (PrPSc) is critical for its infection and pathogenesis. " 01/01/2018 - "Prion protein protects mice from lethal infection with influenza A viruses."
4.	Creutzfeldt-Jakob Syndrome (Creutzfeldt-Jakob Disease) 01/15/2012 - "Creutzfeldt-Jakob disease with the M232R mutation in the prion protein gene in two cases showing different disease courses: a clinicopathological study." 03/01/2004 - "Immunocytochemical accumulation of prion protein (PrP) in lymphoid tissues is a feature of variant Creutzfeldt-Jakob disease (vCJD) that has been used both to aid in the diagnosis of patients and as a basis of large scale screening studies to assess the prevalence of preclinical disease in the UK. " 02/01/2004 - "A recently published study demonstrated the presence of pathological prion protein in sympathetic ganglia of patients suffering from variant Creutzfeldt-Jakob disease, suggesting that these mechanisms might apply to humans." 02/01/2004 - "Patient 1, a 53-year-old man with possible Creutzfeldt-Jakob disease, was enrolled in a research study that included sequencing of the prion protein gene. " 11/01/1993 - "Accumulation of abnormal prion protein in mice infected with Creutzfeldt-Jakob disease via intraperitoneal route: a sequential study."
5.	Bovine Spongiform Encephalopathy (Mad Cow Disease) 10/01/2014 - "The aim of this study was to determine the frequency of the 12-bp and 23-bp indel polymorphisms in the prion protein gene (PRNP) in cattle and to investigate the association between these frequencies and the occurrence of bovine spongiform encephalopathy (BSE). " 03/01/2014 - "Enhanced virulence of sheep-passaged bovine spongiform encephalopathy agent is revealed by decreased polymorphism barriers in prion protein conversion studies." 01/01/2014 - "The aim of this study was to investigate the presence of disease-associated prion protein (PrP(Sc)) in the skeletal muscle of cattle infected with classical bovine spongiform encephalopathy (C-BSE). " 06/01/2012 - "This study was conducted to investigate the presence of single nucleotide polymorphisms (SNPs) in the coding region of the bovine prion protein (PrP) gene among healthy and bovine spongiform encephalopathy (BSE-) affected cattle in Slovakia. " 10/28/2011 - "With the aim of finding new data on the events occurring in the Central Nervous System in animals affected with Bovine Spongiform Encephalopathy, a comprehensive genome wide gene expression study was conducted at different time points of the disease on mice genetically modified to model the bovine species brain in terms of cellular prion protein. "

Related Drugs and Biologics

1.	Protein Isoforms (Isoforms)
2.	Quinacrine (Mepacrine)
3.	Polysaccharides (Glycans)
4.	Autoantibodies
5.	Prions
6.	Proteins (Proteins, Gene)
7.	Codon (Codons)
8.	Peptide Hydrolases (Proteases)
9.	Peptides (Polypeptides)
10.	PrPSc Proteins

Related Therapies and Procedures

1.	Adjuvant Chemotherapy
2.	Therapeutics
3.	Immunotherapy
4.	Oral Administration
5.	Blood Transfusion (Blood Transfusions)