JSON

prion disease

^ http://purl.obolibrary.org/obo/MONDO_0005429

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. [ NCIT:C128346-modified ]

Term info

database cross reference
  • EFO:0004720 (MONDO:equivalentTo)
  • DOID:649 (MONDO:equivalentTo)
  • NCIT:C128346 (MONDO:equivalentTo)
  • ICD9:046.19 (MONDO:relatedTo)
  • SCTID:230284004 (MONDO:equivalentTo)
  • MESH:D017096 (MONDO:equivalentTo)
comment

Editor note: check relationship to spongiform encephalopathy NCIT:C27585

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.

exactMatch

http://purl.obolibrary.org/obo/DOID_649, http://purl.obolibrary.org/obo/NCIT_C128346, http://identifiers.org/snomedct/230284004, http://identifiers.org/mesh/D017096

has exact synonym

prion protein disease, spongiform encephalopathy, prion induced disorder, prion disease pathway

id

MONDO:0005429