Names & Taxonomy

Uniprot ID:
P04156
Entry Name:
PRIO_HUMAN
Status:
reviewed
Protein Names:
Major prion protein (PrP) (ASCR) (PrP27-30) (PrP33-35C) (CD antigen CD230)
Gene Names:
PRNP ALTPRP PRIP PRP
Gene Names Primary:
PRNP
Organism:
Homo sapiens (Human)

Structure

Length:
253
Sequence:
MANLGCWMLVLFVATWSDLGLCKKRPKPGGWNTGGSRYPGQGSPGGNRYPPQGGGGWGQPHGGGWGQPHGGGWGQPHGGGWGQPHGGGWGQGGGTHSQWNKPSKPKTNMKHMAGAAAAGAVVGGLGGYMLGSAMSRPIIHFGSDYEDRYYRENMHRYPNQVYYRPMDEYSNQNNFVHDCVNITIKQHTVTTTTKGENFTETDVKMMERVVEQMCITQYERESQAYYQRGSSMVLFSSPPVILLISFLIFLIVG
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Cell membrane; Lipid-anchor, GPI-anchor

Function

Function:
Its primary physiological function is unclear. Has cytoprotective activity against internal or environmental stresses. May play a role in neuronal development and synaptic plasticity. May be required for neuronal myelin sheath maintenance. May play a role in iron uptake and iron homeostasis. Soluble oligomers are toxic to cultured neuroblastoma cells and induce apoptosis (in vitro) (PubMed:12732622, PubMed:19936054, PubMed:20564047). Association with GPC1 (via its heparan sulfate chains) targets PRNP to lipid rafts. Also provides Cu(2+) or ZN(2+) for the ascorbate-mediated GPC1 deaminase degradation of its heparan sulfate side chains (By similarity).
Cross Reference Drug Bank:
DB00759
Gene Ontology Go:
anchored component of membrane
cell surface
cytoplasm
endoplasmic reticulum
extracellular exosome
extrinsic component of membrane
Golgi apparatus
membrane raft
nucleus
plasma membrane
copper ion binding
identical protein binding
microtubule binding
tubulin binding
axon guidance
cell cycle arrest
cellular copper ion homeostasis
cellular response to copper ion
cellular response to drug
learning or memory
metabolic process
negative regulation of activated T cell proliferation
negative regulation of apoptotic process
negative regulation of calcineurin-NFAT signaling cascade
negative regulation of interferon-gamma production
negative regulation of interleukin-17 production
negative regulation of interleukin-2 production
negative regulation of protein phosphorylation
negative regulation of sequence-specific DNA binding transcription factor activity
negative regulation of T cell receptor signaling pathway
protein homooligomerization
regulation of potassium ion transmembrane transport
regulation of protein localization
response to cadmium ion
response to oxidative stress
Gene Ontology Biological Process:
axon guidance
cell cycle arrest
cellular copper ion homeostasis
cellular response to copper ion
cellular response to drug
learning or memory
metabolic process
negative regulation of activated T cell proliferation
negative regulation of apoptotic process
negative regulation of calcineurin-NFAT signaling cascade
negative regulation of interferon-gamma production
negative regulation of interleukin-17 production
negative regulation of interleukin-2 production
negative regulation of protein phosphorylation
negative regulation of sequence-specific DNA binding transcription factor activity
negative regulation of T cell receptor signaling pathway
protein homooligomerization
regulation of potassium ion transmembrane transport
regulation of protein localization
response to cadmium ion
response to oxidative stress
Gene Ontology Molecular Function:
copper ion binding
identical protein binding
microtubule binding
tubulin binding
Gene Ontology Cellular Component:
anchored component of membrane
cell surface
cytoplasm
endoplasmic reticulum
extracellular exosome
extrinsic component of membrane
Golgi apparatus
membrane raft
nucleus
plasma membrane
Keywords:
3D-structure
Alternative initiation
Amyloid
Amyloidosis
Cell cycle
Cell membrane
Complete proteome
Copper
Cytoplasm
Direct protein sequencing
Disease mutation
Disulfide bond
GPI-anchor
Glycoprotein
Golgi apparatus
Growth arrest
Lipoprotein
Membrane
Metal-binding
Nucleus
Polymorphism
Prion
Reference proteome
Repeat
Signal
Ubl conjugation
Zinc
Interacts With:
Itself; P05067; P05067-4; Q9BSJ6; P31424-2; P49639; P10636; P29372; P52480; Q9H4B4; P10279; P23907; P04925

Publication

PubMed ID:
3755672 1678248 9799790 10581485 11780052 15489334 3014653 1363802 9384372 7485229 1672107 1683708 9792871 12732622 12214108 16144413 18034490 19059915 19349973 19936054 19381258 20375014 20564047 21478263 21153684 10954699 10618385 10900000 11524679 11900542 14623188 8364585 8105771 17468747 19204296 19927125 2564168 2783132 1347910 1671440 1975028 1363810 1439789 8461023 7902693 7902972 7699395 7906019 7913755 7783876 8797472 8909447 9266722 9786248 9482303 10987652 10631141 10790216 11709001 12690204 19923577 26061765