National Cancer Institute Thesaurus

Last uploaded: February 23, 2024
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Id http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C38853
http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C38853
Preferred Name

Prion Disease Pathway
Synonyms
Prion Disease Pathway
Prion Disease
Type http://www.w3.org/2002/07/owl#Class

All Properties

label
Prion Disease Pathway
Legacy Concept Name
Prion_Disease_Pathway
DesignNote
This pathway originally was KEGG_ID hsa05060.
Preferred_Name
Prion Disease Pathway
Semantic_Type
Functional Concept
prefixIRI
Thesaurus:C38853
UMLS_CUI
C3536911
code
C38853
subClassOf
type
KEGG_ID
hsa05020
FULL_SYN
Prion Disease Pathway
Prion Disease
ALT_DEFINITION
Prion diseases, also termed transmissible spongiform encephalopathies (TSEs), are a group of fatal neurodegenerative diseases that affect humans and a number of other animal species. The etiology of these diseases is thought to be associated with the conversion of a normal protein, PrPC, into an infectious, pathogenic form, PrPSc. The conversion is induced by prion infections (for example, variant Creutzfeldt-Jakob disease (vCJD), iatrogenic CJD, Kuru), mutations (familial CJD, Gerstmann-Straussler-Scheinker syndrome, fatal familial insomnia (FFI)) or unknown factors (sporadic CJD (sCJD)), and is thought to occur after PrPC has reached the plasma membrane or is re-internalized for degradation. The PrPSc form shows greater protease resistance than PrPC and accumulates in affected individuals, often in the form of extracellular plaques. Pathways that may lead to neuronal death comprise oxidative stress, regulated activation of complement, ubiquitin-proteasome and endosomal-lysosomal systems, synaptic alterations and dendritic atrophy, corticosteroid response, and endoplasmic reticulum stress. In addition, the conformational transition could lead to the lost of a beneficial activity of the natively folded protein, PrPC.
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