PRNP, prion protein, 5621

N. diseases: 426; N. variants: 47
Disease: Chronic wasting disease (CWD) ×
Source: ALL
Disease Score gda Association Type Type Original DB Sentence supporting the association PMID PMID Year
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 Biomarker disease BEFREE Cells reconstituted with cervid PrP and infected with CWD prions tested positive in prion conversion assay, whereas non-reconstituted cells were negative. 31371793 2019
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 Biomarker disease BEFREE The diagnosis of chronic wasting disease (CWD) relies on demonstration of the disease-associated misfolded CWD prion protein (PrPCWD) in brain or retropharyngeal lymph node tissue by immunodetection methods, e.g.ELISA and immunohistochemistry (IHC). 31461493 2019
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 Biomarker disease BEFREE However, addition of methionine at codon 208 together with the previously described rigid loop substitutions seems to hide a key in this species barrier, as it makes sheep recombinant prion protein highly susceptible to CWD-induced misfolding. 30592012 2019
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 Biomarker disease BEFREE In this work, we show that bank vole prion protein (PrP) is an excellent substrate for RT-QuIC reactions, enabling the detection of trace-amounts of CWD prions, regardless of prion strain and cervid species. 31819115 2019
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 GeneticVariation disease BEFREE These findings indicate that amino acid differences at PrP residue 226 dictate the selection and propagation of divergent strains in deer and elk with CWD. 31147460 2019
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 GeneticVariation disease BEFREE Specific nucleotide variations in the prion protein gene (PRNP) sequence have been associated with reduced susceptibility to CWD in white-tailed deer. 30041562 2018
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 GeneticVariation disease BEFREE We hypothesized that active vaccination with homologous and aggregation-prone recombinant prion protein (PrP) could overcome self-tolerance and induce autoantibody production against the cellular isoform of PrP (PrP<sup>C</sup>), which would be protective against CWD infection from peripheral routes. 30397182 2018
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 Biomarker disease BEFREE However, two studies on squirrel monkeys provided evidence that transmission of CWD prions resulting in prion disease is possible in these monkeys under experimental conditions and seven in vitro experiments provided evidence that CWD prions can convert human prion protein to a misfolded state. 28139079 2018
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 GeneticVariation disease BEFREE We found that assay sensitivity and specificity were indeed imperfect, and we were able to draw several conclusions pertinent to CWD biology from our analyses: (i) the shedding of prions in saliva increases with time postinoculation, but is common throughout the preclinical phase of disease; (ii) the shedding propensity is influenced neither by sex nor by prion protein genotype at codon 96; and (iii) the source of prion-containing inoculum used to infect deer affects the likelihood of prion shedding in saliva; oral inoculation of deer with CWD-positive saliva resulted in 2.77 times the likelihood of prion shedding in saliva compared to that from inoculation with CWD-positive brain. 29118163 2018
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 Biomarker disease BEFREE We used a cell-free seeded protein misfolding assay to determine whether CWD prions from elk, white-tailed deer, and reindeer in North America can convert the human prion protein to the disease-associated form. 30014840 2018
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 Biomarker disease BEFREE Misfolding of the normally folded prion protein of mammals (PrP<sup>C</sup>) into infectious fibrils causes a variety of diseases, from scrapie in sheep to chronic wasting disease (CWD) in cervids. 29310497 2018
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 GeneticVariation disease BEFREE We found that RT-QuIC identified significantly more CWD-positive animals than IHC using RAMALT tissues (121 vs. 86, respectively, out of 553 unique animals), and that longstanding disease presence was associated with an increasing frequency of less susceptible PRNP alleles. 29424295 2018
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 Biomarker disease BEFREE Here we report the successful amplification of disease-associated PrP in differentiated neurosphere cultures within 3 weeks after exposure to CWD prions from both white-tailed deer or elk. 28762865 2017
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 Biomarker disease BEFREE We report here on the ultrastructure of amyloid plaques in chronic wasting disease (CWD) transmitted to Tg20 transgenic mice overexpressing prion protein (PrP<sup>c</sup>). 29105545 2017
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 GeneticVariation disease BEFREE Although prior modeling work predicted that selection will continue, the potential for fitness costs of the 132L allele or new prion protein strains to arise suggest that it is prudent to assume balancing selection may prevent fixation of the 132L allele in populations with CWD. 29087314 2017
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 AlteredExpression disease BEFREE Here, we have specifically adapted the RT-QuIC technique to reveal PrP<sup>Sc</sup> seeding activity in feces of CWD infected cervids. 28994814 2017
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 GeneticVariation disease BEFREE Cervids with genotypes encoding for the prion protein (PRNP) that were considered to be more susceptible to CWD were more likely to excrete CWD prions (94 %) than cervids with genotypes considered to be less susceptible (64 %). 28708047 2017
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 GeneticVariation disease BEFREE Despite the lack of CWD reports in Brazil, the examined codons (95, 96, 116, 132, 225, and 226) of the PRNP gene showed potential CWD susceptibility in Brazilian deer. 28281927 2017
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 Biomarker disease BEFREE We hypothesized that it is possible to prevent peripheral CWD infection and CWD prion shedding by inducing auto-antibodies against the cellular prion protein (PrP<sup>C</sup>) by active vaccination. 28874781 2017
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 GeneticVariation disease BEFREE CWD susceptibility is modulated by species-specific polymorphisms in the prion protein gene (Prnp). 28350512 2017
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 GeneticVariation disease BEFREE We investigated the role of host-dependent factors on phenotypic diversity of chronic wasting disease (CWD) in different host species that express the same prion protein gene (Prnp). 26246570 2015
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 Biomarker disease BEFREE We show here, in stark contrast to this reported inhibitory effect, that quinacrine enhances deer and elk PrP(Sc) accumulation and promotes propagation of prions causing chronic wasting disease (CWD), a fatal, transmissible, neurodegenerative disorder of cervids of uncertain zoonotic potential. 24711410 2014
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 Biomarker disease BEFREE In this study, we inoculated two forms of atypical BSE (BASE and H-type BSE), a chronic wasting disease (CWD) isolate and seven isolates of atypical scrapie into gene-targeted transgenic (Tg) mice expressing the human prion protein (PrP). 22495232 2012
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 Biomarker disease BEFREE Our results show that cervid PrP(Sc) can induce the conversion of human PrP(C) but only after the CWD prion strain has been stabilized by successive passages in vitro or in vivo. 21209079 2011
CUI: C4700085
Disease: Chronic wasting disease (CWD)
Chronic wasting disease (CWD) 		0.100 AlteredExpression disease BEFREE To investigate the potential public health risks posed by CWD prions, we have investigated whether intracerebral inoculation of brain and spinal cord from CWD-infected mule deer transmits prion infection to transgenic mice overexpressing human prion protein with methionine or valine at polymorphic residue 129. 20610667 2010