Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Transgenic fatal familial insomnia mice indicate prion infectivity-independent mechanisms of pathogenesis and phenotypic expression of disease.

PLoS pathogens - 1 Apr 2015

Bouybayoune Ihssane, Mantovani Susanna, Del Gallo Federico, Bertani Ilaria, Restelli Elena, Comerio Liliana, Tapella Laura, Baracchi Francesca, Fernández-Borges Natalia, Mangieri Michela, Bisighini Cinzia, Beznoussenko Galina V, Paladini Alessandra, Balducci Claudia, Micotti Edoardo, Forloni Gianluigi, Castilla Joaquín, Fiordaliso Fabio, Tagliavini Fabrizio, Imeri Luca, Chiesa Roberto

Abstract excerpt

Fatal familial insomnia (FFI) and a genetic form of Creutzfeldt-Jakob disease (CJD178) are clinically different prion disorders linked to the D178N prion protein (PrP) mutation. The disease phenotype is determined by the 129 M/V polymorphism on the mutant allele, which is thought to influence D17...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Brain
Share to Electroencephalography

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Magnetic Resonance Imaging

Share to Maze Learning

Share to Mice, Transgenic

Share to Microscopy, Electron, Transmission

Share to Prion Proteins

Share to Prions

Source

Open in PubMed Open full text

Identifiers

PMID: 25880443
DOI: 10.1371/journal.ppat.1004796
PMCID: PMC4400166

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026. Prepared record publication-25880443-2026-09-04.2. Manifest publication-graph-cohort-2026-09-04.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 4 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Transgenic fatal familial insomnia mice indicate prion infectivity-independent mechanisms of pathogenesis and phenotypic expression of disease.PMID 25880443Read ↗

Transgenic mice recapitulate the phenotypic heterogeneity of genetic prion diseases without developing prion infectivity: Role of intracellular PrP retention in neurotoxicity.Semantic proximity Read ↗

Molecular pathology of fatal familial insomnia.Semantic proximity Read ↗

Mutant prion protein expression causes motor and memory deficits and abnormal sleep patterns in a transgenic mouse model.Semantic proximity Read ↗

Experimental Models of Inherited PrP Prion Diseases.Semantic proximity Read ↗

Profoundly different prion diseases in knock-in mice carrying single PrP codon substitutions associated with human diseases.Semantic proximity Read ↗

D178N prion protein mutation endows RML prions with new strain properties that do not mimic human genetic prion diseases.Semantic proximity Read ↗

Fatal Familial Insomnia: Clinical Aspects and Molecular Alterations.Semantic proximity Read ↗

A subtype of sporadic prion disease mimicking fatal familial insomnia.Semantic proximity Read ↗

Fatal familial insomnia and familial Creutzfeldt-Jakob disease: clinical, pathological and molecular features.Semantic proximity Read ↗

Genetic Creutzfeldt-Jakob disease and fatal familial insomnia: insights into phenotypic variability and disease pathogenesis.Semantic proximity Read ↗

Neurodegenerative illness in transgenic mice expressing a transmembrane form of the prion protein.Semantic proximity Read ↗

[The spectrum of prion pathology broadens: fatal familial insomnia].Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.