PRNP

PRNP

Identifiers Symbols PRNP; ASCR; AltPrP; CD230; CJD; GSS; KURU; PRIP; PrP; PrP27-30; PrP33-35C; PrPc; p27-30 External IDs OMIM: 176640 MGI: 97769 HomoloGene: 7904 ChEMBL: 4869 GeneCards: PRNP Gene

Gene Ontology
Molecular function	• copper ion binding • protein binding • microtubule binding • tubulin binding • identical protein binding • ATP-dependent protein binding • chaperone binding
Cellular component	• nucleus • nucleolus • cytoplasm • endoplasmic reticulum • Golgi apparatus • plasma membrane • extrinsic to membrane • anchored to membrane • intracellular membrane-bounded organelle • membrane raft
Biological process	• negative regulation of protein phosphorylation • cellular copper ion homeostasis • response to oxidative stress • cell cycle arrest • axon guidance • learning or memory • metabolic process • negative regulation of interferon-gamma production • negative regulation of interleukin-17 production • negative regulation of interleukin-2 production • negative regulation of apoptotic process • negative regulation of sequence-specific DNA binding transcription factor activity • negative regulation of activated T cell proliferation • response to cadmium ion • response to copper ion • negative regulation of T cell receptor signaling pathway • protein homooligomerization • negative regulation of calcineurin-NFAT signaling cascade
Sources: Amigo / QuickGO

RNA expression pattern More reference expression data Orthologs Species Human Mouse Entrez 5621 19122 Ensembl ENSG00000171867 ENSMUSG00000079037 UniProt F7VJQ1 P04925 RefSeq (mRNA) NM_000311 NM_011170 RefSeq (protein) NP_000302 NP_035300 Location (UCSC) Chr 20:
4.67 – 4.68 Mb Chr 2:
131.91 – 131.94 Mb PubMed search

Major prion protein (PrP, for prion protein or protease-resistant protein), also known as CD230 (cluster of differentiation 230), is the only known example of a prion protein. In humans, it is encoded by the PRNP gene (PRioN Protein).

Expression of the protein is most predominant in the nervous system but occurs in many other tissues throughout the body.

The protein can exist in multiple isoforms, the normal PrPC, the disease-causing PrPSc, and an isoform located in the mitochondria. The mis-folded version PrPSc is associated with a variety of cognitive deficiencies and neurodegenerative diseases such as Creutzfeldt-Jakob disease, bovine spongiform encephalopathy, Gerstmann-Sträussler-Scheinker syndrome, fatal familial insomnia, and kuru.