Major prion protein

PRNP
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	1E1G, 1E1J, 1E1P, 1E1S, 1E1U, 1E1W, 1FKC, 1FO7, 1H0L, 1HJM, 1HJN, 1I4M, 1OEH, 1OEI, 1QLX, 1QLZ, 1QM0, 1QM1, 1QM2, 1QM3, 2IV4, 2IV5, 2IV6, 2K1D, 2KUN, 2LBG, 2LEJ, 2LFT, 2LSB, 2LV1, 2OL9, 2W9E, 3HAF, 3HAK, 3HEQ, 3HER, 3HES, 3HJ5, 3HJX, 3MD4, 3MD5, 3NHC, 3NHD, 3NVF, 4DGI, 4E1H, 4E1I,%%s1E1G, 1E1J, 1E1P, 1E1S, 1E1U, 1E1W, 1FKC, 1FO7, 1H0L, 1HJM, 1HJN, 1I4M, 1OEH, 1OEI, 1QLX, 1QLZ, 1QM0, 1QM1, 1QM2, 1QM3, 2IV4, 2IV5, 2IV6, 2K1D, 2KUN, 2LBG, 2LEJ, 2LFT, 2LSB, 2LV1, 2M8T, 2W9E, 3HAF, 3HAK, 3HEQ, 3HER, 3HES, 3HJ5, 3HJX, 3MD4, 3MD5, 3NHC, 3NVF, 4DGI, 4E1H, 4E1I, 4KML, 4N9O
Identifiers
Aliases	PRNP, ASCR, AltPrP, CD230, CJD, GSS, KURU, PRIP, PrP, PrP27-30, PrP33-35C, PrPc, p27-30, prion protein
External IDs	OMIM: 176640 MGI: 97769 HomoloGene: 7904 GeneCards: PRNP
Gene location (Human)
Chr.	Chromosome 20 (human)
End	4,701,590 bp
Gene location (Mouse)
Chr.	Chromosome 2 (mouse)
End	131,780,349 bp
RNA expression pattern
	Top expressed in
	Brodmann area 23; ; Region I of hippocampus proper; ; retinal pigment epithelium; ; orbitofrontal cortex; ; endothelial cell; ; spinal ganglia; ; trigeminal ganglion; ; pons; ; germinal epithelium; ; cerebellar vermis;
	Top expressed in
	entorhinal cortex; ; Region I of hippocampus proper; ; amygdala; ; cingulate gyrus; ; subiculum; ; medial dorsal nucleus; ; lateral geniculate nucleus; ; primary motor cortex; ; medial geniculate nucleus; ; hippocampus proper;
	More reference expression data
	; ;
	More reference expression data
Gene ontology
Molecular function	chaperone binding; transmembrane transporter binding; microtubule binding; ATP-dependent protein binding; metal ion binding; tubulin binding; protein binding; identical protein binding; copper ion binding; lamin binding; amyloid-beta binding; protease binding; glycosaminoglycan binding; type 5 metabotropic glutamate receptor binding; type 8 metabotropic glutamate receptor binding; signaling receptor activity; protein-containing complex binding; cupric ion binding; cuprous ion binding;
Cellular component	mitochondrial outer membrane; membrane; mitochondrion; integral component of membrane; cytoplasm; Golgi apparatus; plasma membrane; cell surface; endoplasmic reticulum; membrane raft; anchored component of membrane; extracellular exosome; nucleus; extrinsic component of membrane; cytosol; postsynaptic density; inclusion body; dendrite; anchored component of external side of plasma membrane; nuclear membrane; intracellular membrane-bounded organelle; postsynapse;
Biological process	negative regulation of protein phosphorylation; negative regulation of interferon-gamma production; response to cadmium ion; negative regulation of calcineurin-NFAT signaling cascade; response to copper ion; negative regulation of apoptotic process; response to oxidative stress; negative regulation of DNA-binding transcription factor activity; negative regulation of T cell receptor signaling pathway; regulation of protein localization; negative regulation of activated T cell proliferation; learning or memory; cellular copper ion homeostasis; cellular response to copper ion; cell cycle; metabolism; negative regulation of interleukin-17 production; protein homooligomerization; negative regulation of interleukin-2 production; regulation of potassium ion transmembrane transport; long-term memory; positive regulation of cell death; negative regulation of protein processing; protein destabilization; activation of protein kinase activity; calcium-mediated signaling using intracellular calcium source; negative regulation of catalytic activity; positive regulation of neuron apoptotic process; regulation of peptidyl-tyrosine phosphorylation; positive regulation of peptidyl-tyrosine phosphorylation; positive regulation of protein tyrosine kinase activity; positive regulation of protein targeting to membrane; dendritic spine maintenance; negative regulation of long-term synaptic potentiation; regulation of glutamate receptor signaling pathway; positive regulation of neuron death; negative regulation of amyloid-beta formation; regulation of intracellular calcium activated chloride channel activity; negative regulation of dendritic spine maintenance; negative regulation of amyloid precursor protein catabolic process; positive regulation of protein localization to plasma membrane; response to amyloid-beta; cellular response to amyloid-beta; regulation of calcium ion import across plasma membrane; neuron projection maintenance;
	Sources:Amigo / QuickGO
Orthologs
	5621
	19122
	ENSG00000171867
	ENSMUSG00000079037
	P04156
	P04925
NM_183079; NM_000311; NM_001080121; NM_001080122; NM_001080123;
	NM_001271561
	NM_001278256; NM_011170
NP_000302; NP_001073590; NP_001073591; NP_001073592; NP_001258490;
	NP_898902; NP_000302.1; NP_001073590.1; NP_001073591.1; NP_001073592.1; NP_898902.1
	NP_001265185; NP_035300
	Wikidata
View/Edit Human	View/Edit Mouse

Major prion protein (PrP) is encoded in the human body by the PRNP gene also known as CD230 (cluster of differentiation 230). 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 PrP^C form, and the protease-resistant form designated PrP^Res such as the disease-causing PrP^Sc (scrapie) and an isoform located in mitochondria. The misfolded version PrP^Sc is associated with a variety of cognitive disorders and neurodegenerative diseases such as in animals: ovine scrapie, bovine spongiform encephalopathy (BSE, mad cow disease), feline spongiform encephalopathy, transmissible mink encephalopathy (TME), exotic ungulate encephalopathy, chronic wasting disease (CWD) which affects deer; and in humans: Creutzfeldt–Jakob disease (CJD), fatal familial insomnia (FFI), Gerstmann–Sträussler–Scheinker syndrome (GSS), kuru, and variant Creutzfeldt–Jakob disease (vCJD). Similarities exist between kuru, thought to be due to human ingestion of diseased individuals, and vCJD, thought to be due to human ingestion of BSE-tainted cattle products.

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