prion disease
MONDO:0005429Mondo
Findings
No curated finding names prion disease yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
A transmissible disease that is caused by a protein that is able to induce abnormal folding of normal cellular proteins, leading to characteristic spongiform brain changes, which are associated with neuronal loss without an inflammatory response. Such disorders have typically long incubation periods, but are then generally rapidly progressive and are uniformly fatal.
Definition from the Mondo Disease Ontology (MONDO:0005429), read 2026-09-29. CC BY 4.0.
Where it sits
Other names
1 name
Resolves to: prion disease
- Also called
- spongiform encephalopathy