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Record W4255808419 · doi:10.15273/pnsis.v43i1.3631

The Prion

2005· article· en· W4255808419 on OpenAlexaffvenue
Paul M. Gibbons

Bibliographic record

VenueProceedings of the Nova Scotian Institute of Science · 2005
Typearticle
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicPrion Diseases and Protein Misfolding
Canadian institutionsDalhousie University
Fundersnot available
KeywordsMutantBiologyImmune systemPrion proteinVirologyMicrogliaScrapieImmunologyGeneticsInflammationMedicinePathologyGeneDisease

Abstract

fetched live from OpenAlex

Transmissible spongifonn encephalopathies (TSE) have been documented in livestock for centuries but the nature of the putative causative agent as a contagious, mutant form of a host-encoded protein is a very recent discovery whose nuances remain unclear. In its normal conformation, the Prion is believed to be a short-lived uptake protein ubiquitous in nervous tissues.In contrast ,the mutant Prion usually has an identical primary structure , but has a radically different tertiary and quaternary structure that confers on it unusual stability and resistance to the normal post-translational reactions. Most importantly, the mutant protein binds to the normal Prion protein and alters its conformation to the mutant form. Transmission of TSE from host to host has been observed to occur primarily through ingestion of infected tissue and introduction of the mutant Prion to nervous tissue in the mouth, such as the cranial nerves serving the tongue. It is believed that the mutant Prion is transported within the parenchyma via highly motile microglia. The latent damage from eventual accumulation of mutant Prion is the result of the host's immune response to the protein that involves inflammatory TNF-alpha and IL-1 alpha and beta, among others. Clinical symptoms, however , presented well after the host's immune response resulted in spongiform changes to nervous tissue. Fortunately, there currently exists promising research that seeking to explain natural immunity to TSE and apply it to unaffected individuals.Les encaphalopathies spongiformes transmissibles (EST) sont signalees chez le betail depuis des siecles, mais la nature de l'agent causal presume, une forme mutante contagieuse de proteine encodee par l'hote, est une decouverte tres recente qui reste mysterieuse sous bien des rapports. Dans sa conformation normale, le prion serait une proteine messagere dont la vie est courte et qui serait tres repandue dans les tissus nerveux. Par centre, si le prion mutant a une structure primaire identique, sa structure tertiaire et sa structure quatemaire sont completement differentes et lui conferent une stabilite et une resistance inhabituelles aux reactions post-traductionnelles normales . Et cequi est plus important, la proteine prion mutante se lie a la proteine normale et modifie sa conformation pour la transformer en proteine mutante. On a observe que la transmission des EST d'un h6te a l'autre s'effectue principalement par l'ingestion de tissus infectes et l'introduction du prion mutant dans les tissus nerveux par voie orale, notamment par les nerfs craniens au niveau de la langue. On croit que le prion est transporte dans le parenchyme par des microglies tres mobiles. Les dommages latents d'une accumulation eventuelle du prion resultent de la reaction immunitaire de l'hote envers la proteine qui fait intervenir le TNF-alpha et les IL-1 alpha et beta, entre autres . Les symptomes cliniques, toutefois , qui se presentent longtemps apres la reaction immunitaire de l'hote, consistent en des modifications spongiformes au niveau des tissus nerveux. Heureusement, des recherches prometteuses tentent d'expliquer l'immunite naturelle envers les EST et de l'appliquer aux sujets non atteints.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame machine prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.

metaresearch head score (Codex)0.001
metaresearch head score (Gemma)0.002
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Not applicable · Consensus signal: Not applicable
GenreCandidate signal: Empirical · Consensus signal: none
Teacher disagreement score0.999
Threshold uncertainty score0.000

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0010.002
Meta-epidemiology (narrow)0.0020.000
Meta-epidemiology (broad)0.0010.001
Bibliometrics0.0020.003
Science and technology studies0.0020.002
Scholarly communication0.0070.003
Open science0.0010.003
Research integrity0.0030.003
Insufficient payload (model declined to judge)0.0720.109

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.011
GPT teacher head0.254
Teacher spread0.243 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designNot applicable
Domainnot available
GenreEmpirical

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

Quick stats

Citations0
Published2005
Admission routes2
Has abstractyes

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Same venueProceedings of the Nova Scotian Institute of ScienceSame topicPrion Diseases and Protein MisfoldingFrench-language works237,207