About: Prion Disease Pathway

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An Entity of Type : owl:Class, within Data Space : linked.opendata.cz associated with source document(s)

Attributes	Values
rdf:type	Class
rdfs:label	Prion Disease Pathway
rdfs:subClassOf	Neurodegenerative Disorders Pathway
Semantic_Type	Functional Concept
Preferred_Name	Prion Disease Pathway
NCI_META_CUI	CL329956
KEGG_ID	hsa05020
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.KEGG
Legacy_Concept_Name	Prion_Disease_Pathway
FULL_SYN	Prion DiseasePTKEGG Prion Disease PathwayPTNCI
DesignNote	This pathway originally was KEGG_ID hsa05060.
code	C38853
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