UniProtKB - P04156 (PRIO_HUMAN)
Major prion protein
PRNP
Functioni
Its primary physiological function is unclear. May play a role in neuronal development and synaptic plasticity. May be required for neuronal myelin sheath maintenance. May promote myelin homeostasis through acting as an agonist for ADGRG6 receptor. May play a role in iron uptake and iron homeostasis. Soluble oligomers are toxic to cultured neuroblastoma cells and induce apoptosis (in vitro) (By similarity).
Association with GPC1 (via its heparan sulfate chains) targets PRNP to lipid rafts. Also provides Cu2+ or Zn2+ for the ascorbate-mediated GPC1 deaminase degradation of its heparan sulfate side chains (By similarity).
By similarityCurated3 PublicationsMiscellaneous
Caution
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Metal bindingi | 61 | Cu(2+) 11 Publication | 1 | |
Metal bindingi | 62 | Cu(2+) 1; via amide nitrogen1 Publication | 1 | |
Metal bindingi | 63 | Cu(2+) 1; via amide nitrogen and carbonyl oxygen1 Publication | 1 | |
Metal bindingi | 69 | Cu(2+) 21 Publication | 1 | |
Metal bindingi | 70 | Cu(2+) 2; via amide nitrogen1 Publication | 1 | |
Metal bindingi | 71 | Cu(2+) 2; via amide nitrogen and carbonyl oxygen1 Publication | 1 | |
Metal bindingi | 77 | Cu(2+) 31 Publication | 1 | |
Metal bindingi | 78 | Cu(2+) 3; via amide nitrogen1 Publication | 1 | |
Metal bindingi | 79 | Cu(2+) 3; via amide nitrogen and carbonyl oxygen1 Publication | 1 | |
Metal bindingi | 85 | Cu(2+) 41 Publication | 1 | |
Metal bindingi | 86 | Cu(2+) 4; via amide nitrogen1 Publication | 1 | |
Metal bindingi | 87 | Cu(2+) 4; via amide nitrogen and carbonyl oxygen1 Publication | 1 |
GO - Molecular functioni
- amyloid-beta binding Source: ARUK-UCL
- aspartic-type endopeptidase inhibitor activity Source: ARUK-UCL
- ATP-dependent protein binding Source: Ensembl
- calcium ion binding Source: GO_Central
- chaperone binding Source: Ensembl
- copper ion binding Source: UniProtKB
- cupric ion binding Source: Ensembl
- cuprous ion binding Source: CAFA
- glycosaminoglycan binding Source: ARUK-UCL
- identical protein binding Source: IntAct
- lamin binding Source: Ensembl
- microtubule binding Source: UniProtKB
- protease binding Source: ARUK-UCL
- protein-containing complex binding Source: ARUK-UCL
- signaling receptor activity Source: ARUK-UCL
- transmembrane transporter binding Source: Ensembl
- tubulin binding Source: UniProtKB
- type 5 metabotropic glutamate receptor binding Source: ARUK-UCL
GO - Biological processi
- activation of protein kinase activity Source: Ensembl
- calcium-mediated signaling using intracellular calcium source Source: ARUK-UCL
- cell cycle Source: UniProtKB-KW
- cellular copper ion homeostasis Source: UniProtKB
- cellular response to amyloid-beta Source: ARUK-UCL
- cellular response to copper ion Source: MGI
- cellular response to xenobiotic stimulus Source: Ensembl
- dendritic spine maintenance Source: ARUK-UCL
- learning or memory Source: ARUK-UCL
- long-term memory Source: ARUK-UCL
- modulation of age-related behavioral decline Source: ARUK-UCL
- negative regulation of activated T cell proliferation Source: BHF-UCL
- negative regulation of amyloid-beta formation Source: ARUK-UCL
- negative regulation of amyloid precursor protein catabolic process Source: ARUK-UCL
- negative regulation of apoptotic process Source: Ensembl
- negative regulation of calcineurin-NFAT signaling cascade Source: BHF-UCL
- negative regulation of dendritic spine maintenance Source: ARUK-UCL
- negative regulation of DNA-binding transcription factor activity Source: BHF-UCL
- negative regulation of interferon-gamma production Source: BHF-UCL
- negative regulation of interleukin-17 production Source: BHF-UCL
- negative regulation of interleukin-2 production Source: BHF-UCL
- negative regulation of long-term synaptic potentiation Source: Ensembl
- negative regulation of protein phosphorylation Source: BHF-UCL
- negative regulation of protein processing Source: ARUK-UCL
- negative regulation of T cell receptor signaling pathway Source: BHF-UCL
- neuron projection maintenance Source: ARUK-UCL
- positive regulation of neuron apoptotic process Source: CAFA
- positive regulation of neuron death Source: ARUK-UCL
- positive regulation of peptidyl-tyrosine phosphorylation Source: ARUK-UCL
- positive regulation of protein localization to plasma membrane Source: Ensembl
- positive regulation of protein targeting to membrane Source: ARUK-UCL
- positive regulation of protein tyrosine kinase activity Source: ARUK-UCL
- protein destabilization Source: CAFA
- protein homooligomerization Source: InterPro
- regulation of calcium ion import across plasma membrane Source: ARUK-UCL
- regulation of glutamate receptor signaling pathway Source: ARUK-UCL
- regulation of intracellular calcium activated chloride channel activity Source: ARUK-UCL
- regulation of peptidyl-tyrosine phosphorylation Source: ARUK-UCL
- regulation of potassium ion transmembrane transport Source: Ensembl
- response to amyloid-beta Source: ARUK-UCL
- response to cadmium ion Source: Ensembl
- response to oxidative stress Source: UniProtKB
Keywordsi
Molecular function | Prion |
Biological process | Cell cycle, Growth arrest |
Ligand | Copper, Metal-binding, Zinc |
Enzyme and pathway databases
PathwayCommonsi | P04156 |
Reactomei | R-HSA-419037, NCAM1 interactions R-HSA-9609523, Insertion of tail-anchored proteins into the endoplasmic reticulum membrane |
SignaLinki | P04156 |
Protein family/group databases
MoonDBi | P04156, Predicted |
TCDBi | 1.C.48.1.2, the prion peptide (prp) family |
Names & Taxonomyi
Protein namesi | Recommended name: Major prion proteinShort name: PrP Alternative name(s): ASCR PrP27-30 PrP33-35C CD_antigen: CD230 |
Gene namesi | Name:PRNP Synonyms:ALTPRP, PRIP, PRP |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:9449, PRNP |
MIMi | 176640, gene |
neXtProti | NX_P04156 |
VEuPathDBi | HostDB:ENSG00000171867 |
Subcellular locationi
Golgi apparatus
- Golgi apparatus By similarity
Plasma membrane
- Cell membrane ; GPI-anchor 1 Publication
Note: Targeted to lipid rafts via association with the heparan sulfate chains of GPC1. Colocates, in the presence of Cu2+, to vesicles in para- and perinuclear regions, where both proteins undergo internalization. Heparin displaces PRNP from lipid rafts and promotes endocytosis.1 Publication
Cytosol
- cytosol Source: HPA
Endoplasmic reticulum
- endoplasmic reticulum Source: UniProtKB
Extracellular region or secreted
- extracellular exosome Source: UniProtKB
Golgi apparatus
- Golgi apparatus Source: UniProtKB
Nucleus
- nuclear membrane Source: HPA
- nucleus Source: GO_Central
Plasma Membrane
- anchored component of external side of plasma membrane Source: ARUK-UCL
- plasma membrane Source: UniProtKB
Other locations
- cell surface Source: UniProtKB
- cytoplasm Source: GO_Central
- dendrite Source: Ensembl
- extrinsic component of membrane Source: UniProtKB
- inclusion body Source: CAFA
- intracellular membrane-bounded organelle Source: HPA
- membrane raft Source: MGI
- postsynapse Source: ARUK-UCL
- postsynaptic density Source: ARUK-UCL
- terminal bouton Source: Ensembl
Keywords - Cellular componenti
Amyloid, Cell membrane, Golgi apparatus, MembranePathology & Biotechi
Involvement in diseasei
Creutzfeldt-Jakob disease (CJD)10 Publications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_006469 | 178 | D → N in FFI and CJD. 4 PublicationsCorresponds to variant dbSNP:rs74315403EnsemblClinVar. | 1 | |
Natural variantiVAR_006470 | 180 | V → I in CJD. 3 PublicationsCorresponds to variant dbSNP:rs74315408EnsemblClinVar. | 1 | |
Natural variantiVAR_008749 | 196 | E → K in CJD. 1 Publication | 1 | |
Natural variantiVAR_006473 | 200 | E → K in CJD. 3 PublicationsCorresponds to variant dbSNP:rs28933385EnsemblClinVar. | 1 | |
Natural variantiVAR_008751 | 203 | V → I in CJD; unknown pathological significance. 1 PublicationCorresponds to variant dbSNP:rs776593792Ensembl. | 1 | |
Natural variantiVAR_006474 | 208 | R → H in CJD. 1 PublicationCorresponds to variant dbSNP:rs74315412EnsemblClinVar. | 1 | |
Natural variantiVAR_006475 | 210 | V → I in CJD. 1 PublicationCorresponds to variant dbSNP:rs74315407EnsemblClinVar. | 1 | |
Natural variantiVAR_008752 | 211 | E → Q in CJD. 1 PublicationCorresponds to variant dbSNP:rs398122370EnsemblClinVar. | 1 | |
Natural variantiVAR_006478 | 232 | M → R in CJD. 1 PublicationCorresponds to variant dbSNP:rs74315409EnsemblClinVar. | 1 |
Fatal familial insomnia (FFI)3 Publications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_006469 | 178 | D → N in FFI and CJD. 4 PublicationsCorresponds to variant dbSNP:rs74315403EnsemblClinVar. | 1 |
Gerstmann-Straussler disease (GSD)11 Publications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_006464 | 102 | P → L in GSD and early-onset dementia. 5 PublicationsCorresponds to variant dbSNP:rs74315401EnsemblClinVar. | 1 | |
Natural variantiVAR_006465 | 105 | P → L in GSD. 2 PublicationsCorresponds to variant dbSNP:rs11538758EnsemblClinVar. | 1 | |
Natural variantiVAR_014264 | 131 | G → V in GSD. 1 PublicationCorresponds to variant dbSNP:rs74315410EnsemblClinVar. | 1 | |
Natural variantiVAR_008746 | 187 | H → R in GSD. 1 PublicationCorresponds to variant dbSNP:rs74315413EnsemblClinVar. | 1 | |
Natural variantiVAR_006472 | 198 | F → S in GSD; atypical form with neurofibrillary tangles. 1 PublicationCorresponds to variant dbSNP:rs74315405EnsemblClinVar. | 1 | |
Natural variantiVAR_008750 | 202 | D → N in GSD. 1 PublicationCorresponds to variant dbSNP:rs761807915Ensembl. | 1 | |
Natural variantiVAR_008753 | 212 | Q → P in GSD. 1 PublicationCorresponds to variant dbSNP:rs751882709Ensembl. | 1 | |
Natural variantiVAR_006476 | 217 | Q → R in GSD; with neurofibrillary tangles. 1 PublicationCorresponds to variant dbSNP:rs74315406EnsemblClinVar. | 1 |
Huntington disease-like 1 (HDL1)1 Publication
Kuru (KURU)2 Publications
Spongiform encephalopathy with neuropsychiatric features (SENF)2 Publications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_006471 | 183 | T → A in SENF and early-onset dementia; induces loss of glycosylation at N-181. 3 PublicationsCorresponds to variant dbSNP:rs74315411EnsemblClinVar. | 1 |
Keywords - Diseasei
Amyloidosis, Disease variantOrganism-specific databases
DisGeNETi | 5621 |
GeneReviewsi | PRNP |
MalaCardsi | PRNP |
MIMi | 123400, phenotype 137440, phenotype 245300, phenotype 600072, phenotype 603218, phenotype 606688, phenotype |
OpenTargetsi | ENSG00000171867 |
Orphaneti | 280397, Familial Alzheimer-like prion disease 466, Fatal familial insomnia 356, Gerstmann-Straussler-Scheinker syndrome 157941, Huntington disease-like 1 282166, Inherited Creutzfeldt-Jakob disease 454745, Kuru 397606, PrP systemic amyloidosis 586130, Sporadic fatal insomnia |
PharmGKBi | PA33796 |
Miscellaneous databases
Pharosi | P04156, Tchem |
Chemistry databases
ChEMBLi | CHEMBL4869 |
DrugBanki | DB09130, Copper DB00759, Tetracycline |
DrugCentrali | P04156 |
Genetic variation databases
BioMutai | PRNP |
DMDMi | 130912 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Signal peptidei | 1 – 22 | By similarityAdd BLAST | 22 | |
ChainiPRO_0000025675 | 23 – 230 | Major prion proteinAdd BLAST | 208 | |
PropeptideiPRO_0000025676 | 231 – 253 | Removed in mature formBy similarityAdd BLAST | 23 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Disulfide bondi | 179 ↔ 214 | 1 Publication | ||
Glycosylationi | 181 | N-linked (GlcNAc...) asparagine1 Publication | 1 | |
Glycosylationi | 197 | N-linked (GlcNAc...) asparagine1 Publication | 1 | |
Lipidationi | 230 | GPI-anchor amidated serineBy similarity | 1 |
Post-translational modificationi
Keywords - PTMi
Disulfide bond, Glycoprotein, GPI-anchor, LipoproteinProteomic databases
EPDi | P04156 |
jPOSTi | P04156 |
MassIVEi | P04156 |
PaxDbi | P04156 |
PeptideAtlasi | P04156 |
PRIDEi | P04156 |
ProteomicsDBi | 51667 [P04156-1] |
PTM databases
GlyConnecti | 2056, 3 N-Linked glycans (1 site) |
GlyGeni | P04156, 2 sites, 6 N-linked glycans (1 site) |
iPTMneti | P04156 |
MetOSitei | P04156 |
PhosphoSitePlusi | P04156 |
SwissPalmi | P04156 |
Expressioni
Gene expression databases
Bgeei | ENSG00000171867, Expressed in Brodmann (1909) area 23 and 254 other tissues |
ExpressionAtlasi | P04156, baseline and differential |
Genevisiblei | P04156, HS |
Organism-specific databases
HPAi | ENSG00000171867, Low tissue specificity |
Interactioni
Subunit structurei
Monomer and homodimer. Has a tendency to aggregate into amyloid fibrils containing a cross-beta spine, formed by a steric zipper of superposed beta-strands. Soluble oligomers may represent an intermediate stage on the path to fibril formation. Copper binding may promote oligomerization (PubMed:11524679, PubMed:11900542, PubMed:14623188, PubMed:17468747, PubMed:19204296, PubMed:19927125, PubMed:20375014, PubMed:20564047).
Interacts with GRB2, APP, ERI3/PRNPIP and SYN1. Mislocalized cytosolically exposed PrP interacts with MGRN1; this interaction alters MGRN1 subcellular location and causes lysosomal enlargement (By similarity).
Interacts with KIAA1191 (PubMed:21153684).
Interacts with ADGRG6 (By similarity).
By similarity9 PublicationsBinary interactionsi
P04156
Major prion protein (PRO_0000025675)
With | #Exp. | IntAct |
---|---|---|
Grm5 - isoform 1 [P31424-2] from Rattus norvegicus. | 4 | EBI-8830282,EBI-8830305 |
Pkm [P52480] from Mus musculus. | 5 | EBI-8830282,EBI-647785 |
GO - Molecular functioni
- ATP-dependent protein binding Source: Ensembl
- chaperone binding Source: Ensembl
- identical protein binding Source: IntAct
- lamin binding Source: Ensembl
- microtubule binding Source: UniProtKB
- protease binding Source: ARUK-UCL
- transmembrane transporter binding Source: Ensembl
- tubulin binding Source: UniProtKB
- type 5 metabotropic glutamate receptor binding Source: ARUK-UCL
Protein-protein interaction databases
BioGRIDi | 111606, 442 interactors |
CORUMi | P04156 |
DIPi | DIP-29933N |
ELMi | P04156 |
IntActi | P04156, 441 interactors |
MINTi | P04156 |
STRINGi | 9606.ENSP00000368752 |
Chemistry databases
BindingDBi | P04156 |
Miscellaneous databases
RNActi | P04156, protein |
Structurei
Secondary structure
3D structure databases
BMRBi | P04156 |
SASBDBi | P04156 |
SMRi | P04156 |
ModBasei | Search... |
PDBe-KBi | Search... |
Miscellaneous databases
EvolutionaryTracei | P04156 |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Repeati | 51 – 59 | 1 | 9 | |
Repeati | 60 – 67 | 2 | 8 | |
Repeati | 68 – 75 | 3 | 8 | |
Repeati | 76 – 83 | 4 | 8 | |
Repeati | 84 – 91 | 5 | 8 |
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 23 – 230 | Interaction with GRB2, ERI3 and SYN1By similarityAdd BLAST | 208 | |
Regioni | 23 – 38 | Interaction with ADGRG6By similarityAdd BLAST | 16 | |
Regioni | 26 – 108 | DisorderedSequence analysisAdd BLAST | 83 | |
Regioni | 51 – 91 | 5 X 8 AA tandem repeats of P-H-G-G-G-W-G-QAdd BLAST | 41 |
Domaini
Sequence similaritiesi
Keywords - Domaini
Repeat, SignalPhylogenomic databases
eggNOGi | ENOG502S2A8, Eukaryota |
GeneTreei | ENSGT00510000049083 |
InParanoidi | P04156 |
OMAi | HNPGYPH |
OrthoDBi | 957343at2759 |
PhylomeDBi | P04156 |
TreeFami | TF105188 |
Family and domain databases
DisProti | DP00466 |
Gene3Di | 1.10.790.10, 1 hit |
InterProi | View protein in InterPro IPR000817, Prion IPR036924, Prion/Doppel_b-ribbon_dom_sf IPR022416, Prion/Doppel_prot_b-ribbon_dom IPR025860, Prion_N_dom |
Pfami | View protein in Pfam PF00377, Prion, 1 hit PF11587, Prion_bPrPp, 1 hit |
PRINTSi | PR00341, PRION |
SMARTi | View protein in SMART SM00157, PRP, 1 hit |
SUPFAMi | SSF54098, SSF54098, 1 hit |
PROSITEi | View protein in PROSITE PS00291, PRION_1, 1 hit PS00706, PRION_2, 1 hit |
s (2+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry describes 2 produced by isoformsialternative initiation. AlignAdd to basketThis entry has 2 described isoforms and 2 potential isoforms that are computationally mapped.Show allAlign All
This isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
10 20 30 40 50
MANLGCWMLV LFVATWSDLG LCKKRPKPGG WNTGGSRYPG QGSPGGNRYP
60 70 80 90 100
PQGGGGWGQP HGGGWGQPHG GGWGQPHGGG WGQPHGGGWG QGGGTHSQWN
110 120 130 140 150
KPSKPKTNMK HMAGAAAAGA VVGGLGGYML GSAMSRPIIH FGSDYEDRYY
160 170 180 190 200
RENMHRYPNQ VYYRPMDEYS NQNNFVHDCV NITIKQHTVT TTTKGENFTE
210 220 230 240 250
TDVKMMERVV EQMCITQYER ESQAYYQRGS SMVLFSSPPV ILLISFLIFL
IVG
Computationally mapped potential isoform sequencesi
There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketX6RKS3 | X6RKS3_HUMAN | Major prion protein | PRNP | 217 | Annotation score: | ||
A2A2V1 | A2A2V1_HUMAN | Major prion protein | PRNP | 249 | Annotation score: |
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 118 | Missing in AAA19664 (PubMed:3014653).Curated | 1 | |
Sequence conflicti | 118 | Missing in BAA00011 (PubMed:3014653).Curated | 1 | |
Sequence conflicti | 169 | Y → H in ABD63004 (Ref. 6) Curated | 1 | |
Sequence conflicti | 227 | Q → K in AAH22532 (PubMed:15489334).Curated | 1 |
Polymorphismi
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_013763 | 56 – 63 | Missing 3 Publications | 8 | |
Natural variantiVAR_006464 | 102 | P → L in GSD and early-onset dementia. 5 PublicationsCorresponds to variant dbSNP:rs74315401EnsemblClinVar. | 1 | |
Natural variantiVAR_006465 | 105 | P → L in GSD. 2 PublicationsCorresponds to variant dbSNP:rs11538758EnsemblClinVar. | 1 | |
Natural variantiVAR_006466 | 117 | A → V Linked to development of dementing Gerstmann-Straussler disease. 1 PublicationCorresponds to variant dbSNP:rs74315402EnsemblClinVar. | 1 | |
Natural variantiVAR_073722 | 127 | G → V Variant that has been selected for in response to the Kuru epidemic and confers resistance to prion disease by acting as a 'dominant negative' inhibitor of prion conversion; is not only itself resistant to conformational conversion, but also inhibits conversion of wild-type proteins; confers protection against classical Creutzfeldt-Jakob disease (CJD) and Kuru in the heterozygous state, but can be infected with variant CJD prions, resulting from exposure to bovine spongiform encephalopathy prions; confers complete resistance to all prion strains when homozygous. Always associated with M-129 variant. 2 PublicationsCorresponds to variant dbSNP:rs267606980EnsemblClinVar. | 1 | |
Natural variantiVAR_006467 | 129 | M → V Confers relative protection against acquired, sporadic and some inherited prion diseases in the heterozygous state, possibly by preventing homodimerization; determines the disease phenotype in patients who have a PrP mutation at position 178; patients with M-129 develop FFI, those with V-129 develop CJD. 4 PublicationsCorresponds to variant dbSNP:rs1799990EnsemblClinVar. | 1 | |
Natural variantiVAR_014264 | 131 | G → V in GSD. 1 PublicationCorresponds to variant dbSNP:rs74315410EnsemblClinVar. | 1 | |
Natural variantiVAR_006468 | 171 | N → S in schizoaffective disorder. 1 PublicationCorresponds to variant dbSNP:rs16990018EnsemblClinVar. | 1 | |
Natural variantiVAR_006469 | 178 | D → N in FFI and CJD. 4 PublicationsCorresponds to variant dbSNP:rs74315403EnsemblClinVar. | 1 | |
Natural variantiVAR_006470 | 180 | V → I in CJD. 3 PublicationsCorresponds to variant dbSNP:rs74315408EnsemblClinVar. | 1 | |
Natural variantiVAR_006471 | 183 | T → A in SENF and early-onset dementia; induces loss of glycosylation at N-181. 3 PublicationsCorresponds to variant dbSNP:rs74315411EnsemblClinVar. | 1 | |
Natural variantiVAR_008746 | 187 | H → R in GSD. 1 PublicationCorresponds to variant dbSNP:rs74315413EnsemblClinVar. | 1 | |
Natural variantiVAR_008748 | 188 | T → K in early-onset dementia; dementia associated to prion diseases. 1 Publication | 1 | |
Natural variantiVAR_008747 | 188 | T → R1 PublicationCorresponds to variant dbSNP:rs372878791Ensembl. | 1 | |
Natural variantiVAR_008749 | 196 | E → K in CJD. 1 Publication | 1 | |
Natural variantiVAR_006472 | 198 | F → S in GSD; atypical form with neurofibrillary tangles. 1 PublicationCorresponds to variant dbSNP:rs74315405EnsemblClinVar. | 1 | |
Natural variantiVAR_006473 | 200 | E → K in CJD. 3 PublicationsCorresponds to variant dbSNP:rs28933385EnsemblClinVar. | 1 | |
Natural variantiVAR_008750 | 202 | D → N in GSD. 1 PublicationCorresponds to variant dbSNP:rs761807915Ensembl. | 1 | |
Natural variantiVAR_008751 | 203 | V → I in CJD; unknown pathological significance. 1 PublicationCorresponds to variant dbSNP:rs776593792Ensembl. | 1 | |
Natural variantiVAR_006474 | 208 | R → H in CJD. 1 PublicationCorresponds to variant dbSNP:rs74315412EnsemblClinVar. | 1 | |
Natural variantiVAR_006475 | 210 | V → I in CJD. 1 PublicationCorresponds to variant dbSNP:rs74315407EnsemblClinVar. | 1 | |
Natural variantiVAR_008752 | 211 | E → Q in CJD. 1 PublicationCorresponds to variant dbSNP:rs398122370EnsemblClinVar. | 1 | |
Natural variantiVAR_008753 | 212 | Q → P in GSD. 1 PublicationCorresponds to variant dbSNP:rs751882709Ensembl. | 1 | |
Natural variantiVAR_006476 | 217 | Q → R in GSD; with neurofibrillary tangles. 1 PublicationCorresponds to variant dbSNP:rs74315406EnsemblClinVar. | 1 | |
Natural variantiVAR_006477 | 219 | E → K Confers relative protection against sporadic Creutzfeldt-Jakob disease (CJD) in the heterozygous state. 2 PublicationsCorresponds to variant dbSNP:rs1800014EnsemblClinVar. | 1 | |
Natural variantiVAR_006478 | 232 | M → R in CJD. 1 PublicationCorresponds to variant dbSNP:rs74315409EnsemblClinVar. | 1 | |
Natural variantiVAR_008754 | 238 | P → S1 Publication | 1 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | M13899 mRNA Translation: AAA60182.1 X83416 Genomic DNA Translation: CAA58442.1 U29185 Genomic DNA Translation: AAC78725.1 AF076976 Genomic DNA Translation: AAD46098.1 AY008282 mRNA Translation: AAG21693.1 DQ408531 Genomic DNA Translation: ABD63004.1 AL133396 Genomic DNA No translation available. BC012844 mRNA Translation: AAH12844.1 BC022532 mRNA Translation: AAH22532.1 D00015 mRNA Translation: BAA00011.1 M13667 mRNA Translation: AAA19664.1 M81929 Genomic DNA Translation: AAB59442.1 M81930 Genomic DNA Translation: AAB59443.1 AF030575 Genomic DNA Translation: AAC05365.1 S80732 Genomic DNA Translation: AAB50648.2 S80743 Genomic DNA Translation: AAB50649.2 S71208 Genomic DNA Translation: AAB20521.1 S71210 Genomic DNA Translation: AAB20522.1 S71212 Genomic DNA Translation: AAB20523.1 |
CCDSi | CCDS13080.1 [P04156-1] |
PIRi | A24173, UJHU |
RefSeqi | NP_000302.1, NM_000311.4 [P04156-1] NP_001073590.1, NM_001080121.2 [P04156-1] NP_001073591.1, NM_001080122.2 [P04156-1] NP_001073592.1, NM_001080123.2 [P04156-1] NP_001258490.1, NM_001271561.2 NP_898902.1, NM_183079.3 [P04156-1] |
Genome annotation databases
Ensembli | ENST00000379440; ENSP00000368752; ENSG00000171867 ENST00000424424; ENSP00000411599; ENSG00000171867 ENST00000430350; ENSP00000399376; ENSG00000171867 ENST00000457586; ENSP00000415284; ENSG00000171867 |
GeneIDi | 5621 |
KEGGi | hsa:5621 |
MANE-Selecti | ENST00000379440.9; ENSP00000368752.4; NM_000311.5; NP_000302.1 |
Keywords - Coding sequence diversityi
Alternative initiationSimilar proteinsi
Cross-referencesi
Web resourcesi
The Official Mad Cow Disease Home Page |
Wikipedia PRNP entry |
Protein Spotlight The shape of harm - Issue 179 of May 2016 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | M13899 mRNA Translation: AAA60182.1 X83416 Genomic DNA Translation: CAA58442.1 U29185 Genomic DNA Translation: AAC78725.1 AF076976 Genomic DNA Translation: AAD46098.1 AY008282 mRNA Translation: AAG21693.1 DQ408531 Genomic DNA Translation: ABD63004.1 AL133396 Genomic DNA No translation available. BC012844 mRNA Translation: AAH12844.1 BC022532 mRNA Translation: AAH22532.1 D00015 mRNA Translation: BAA00011.1 M13667 mRNA Translation: AAA19664.1 M81929 Genomic DNA Translation: AAB59442.1 M81930 Genomic DNA Translation: AAB59443.1 AF030575 Genomic DNA Translation: AAC05365.1 S80732 Genomic DNA Translation: AAB50648.2 S80743 Genomic DNA Translation: AAB50649.2 S71208 Genomic DNA Translation: AAB20521.1 S71210 Genomic DNA Translation: AAB20522.1 S71212 Genomic DNA Translation: AAB20523.1 |
CCDSi | CCDS13080.1 [P04156-1] |
PIRi | A24173, UJHU |
RefSeqi | NP_000302.1, NM_000311.4 [P04156-1] NP_001073590.1, NM_001080121.2 [P04156-1] NP_001073591.1, NM_001080122.2 [P04156-1] NP_001073592.1, NM_001080123.2 [P04156-1] NP_001258490.1, NM_001271561.2 NP_898902.1, NM_183079.3 [P04156-1] |
3D structure databases
Select the link destinations: PDBei RCSB PDBi PDBji Links Updated | PDB entry | Method | Resolution (Å) | Chain | Positions | PDBsum |
1E1G | NMR | - | A | 125-228 | [»] | |
1E1J | NMR | - | A | 125-228 | [»] | |
1E1P | NMR | - | A | 125-228 | [»] | |
1E1S | NMR | - | A | 125-228 | [»] | |
1E1U | NMR | - | A | 125-228 | [»] | |
1E1W | NMR | - | A | 125-228 | [»] | |
1FKC | NMR | - | A | 90-231 | [»] | |
1FO7 | NMR | - | A | 90-231 | [»] | |
1H0L | NMR | - | A | 121-230 | [»] | |
1HJM | NMR | - | A | 125-228 | [»] | |
1HJN | NMR | - | A | 125-228 | [»] | |
1I4M | X-ray | 2.00 | A | 119-226 | [»] | |
1OEH | NMR | - | A | 77-84 | [»] | |
1OEI | NMR | - | A | 61-84 | [»] | |
1QLX | NMR | - | A | 23-230 | [»] | |
1QLZ | NMR | - | A | 23-230 | [»] | |
1QM0 | NMR | - | A | 90-230 | [»] | |
1QM1 | NMR | - | A | 90-230 | [»] | |
1QM2 | NMR | - | A | 121-230 | [»] | |
1QM3 | NMR | - | A | 121-230 | [»] | |
2IV4 | NMR | - | A | 180-195 | [»] | |
2IV5 | NMR | - | A | 173-195 | [»] | |
2IV6 | NMR | - | A | 173-195 | [»] | |
2K1D | NMR | - | A | 90-231 | [»] | |
2KUN | NMR | - | A | 90-231 | [»] | |
2LBG | NMR | - | A | 110-136 | [»] | |
2LEJ | NMR | - | A | 90-231 | [»] | |
2LFT | NMR | - | A | 90-231 | [»] | |
2LSB | NMR | - | A | 90-231 | [»] | |
2LV1 | NMR | - | A | 90-231 | [»] | |
2M8T | NMR | - | A | 90-231 | [»] | |
2OL9 | X-ray | 0.85 | A | 170-175 | [»] | |
2W9E | X-ray | 2.90 | A | 119-231 | [»] | |
3HAF | X-ray | 2.26 | A | 90-231 | [»] | |
3HAK | X-ray | 1.80 | A | 125-227 | [»] | |
3HEQ | X-ray | 1.80 | A/B | 90-231 | [»] | |
3HER | X-ray | 1.85 | A/B | 90-231 | [»] | |
3HES | X-ray | 2.00 | A/B | 90-231 | [»] | |
3HJ5 | X-ray | 3.10 | A/B | 90-231 | [»] | |
3HJX | X-ray | 2.00 | A | 126-231 | [»] | |
3MD4 | X-ray | 1.15 | A/B | 127-132 | [»] | |
3MD5 | X-ray | 1.40 | A/B | 127-132 | [»] | |
3NHC | X-ray | 1.57 | A/B | 127-132 | [»] | |
3NHD | X-ray | 1.92 | A/B | 127-132 | [»] | |
3NVF | X-ray | 1.80 | A | 138-143 | [»] | |
4DGI | X-ray | 2.40 | A | 120-230 | [»] | |
4E1H | X-ray | 1.40 | A/C/E/G/I/K | 177-182 | [»] | |
B/D/F/H/J/L | 211-216 | [»] | ||||
4E1I | X-ray | 2.03 | A/C/E/G/I/K | 177-182 | [»] | |
B/D/F/H/J/L | 211-216 | [»] | ||||
4KML | X-ray | 1.50 | A | 24-231 | [»] | |
4N9O | X-ray | 1.50 | A | 90-231 | [»] | |
5L6R | NMR | - | A | 90-226 | [»] | |
5YJ4 | NMR | - | A | 91-231 | [»] | |
5YJ5 | NMR | - | A | 91-231 | [»] | |
6DU9 | X-ray | 2.33 | A | 90-230 | [»] | |
6LNI | electron microscopy | 2.70 | A/B/C/D/E/F/G/H/I/J | 23-231 | [»] | |
6PQ5 | X-ray | 1.50 | A/B | 113-118 | [»] | |
6PQA | X-ray | 1.46 | A | 119-124 | [»] | |
6SUZ | X-ray | 2.50 | A | 125-223 | [»] | |
6SV2 | X-ray | 2.30 | A | 119-231 | [»] | |
6UUR | electron microscopy | 3.50 | A/B/C/D/E/F/G/H/I/J | 94-178 | [»] | |
7DWV | electron microscopy | 3.07 | A/B/C/D/E/F | 23-231 | [»] | |
BMRBi | P04156 | |||||
SASBDBi | P04156 | |||||
SMRi | P04156 | |||||
ModBasei | Search... | |||||
PDBe-KBi | Search... |
Protein-protein interaction databases
BioGRIDi | 111606, 442 interactors |
CORUMi | P04156 |
DIPi | DIP-29933N |
ELMi | P04156 |
IntActi | P04156, 441 interactors |
MINTi | P04156 |
STRINGi | 9606.ENSP00000368752 |
Chemistry databases
BindingDBi | P04156 |
ChEMBLi | CHEMBL4869 |
DrugBanki | DB09130, Copper DB00759, Tetracycline |
DrugCentrali | P04156 |
Protein family/group databases
MoonDBi | P04156, Predicted |
TCDBi | 1.C.48.1.2, the prion peptide (prp) family |
PTM databases
GlyConnecti | 2056, 3 N-Linked glycans (1 site) |
GlyGeni | P04156, 2 sites, 6 N-linked glycans (1 site) |
iPTMneti | P04156 |
MetOSitei | P04156 |
PhosphoSitePlusi | P04156 |
SwissPalmi | P04156 |
Genetic variation databases
BioMutai | PRNP |
DMDMi | 130912 |
Proteomic databases
EPDi | P04156 |
jPOSTi | P04156 |
MassIVEi | P04156 |
PaxDbi | P04156 |
PeptideAtlasi | P04156 |
PRIDEi | P04156 |
ProteomicsDBi | 51667 [P04156-1] |
Protocols and materials databases
ABCDi | P04156, 3 sequenced antibodies |
Antibodypediai | 3351, 844 antibodies from 47 providers |
DNASUi | 5621 |
Genome annotation databases
Ensembli | ENST00000379440; ENSP00000368752; ENSG00000171867 ENST00000424424; ENSP00000411599; ENSG00000171867 ENST00000430350; ENSP00000399376; ENSG00000171867 ENST00000457586; ENSP00000415284; ENSG00000171867 |
GeneIDi | 5621 |
KEGGi | hsa:5621 |
MANE-Selecti | ENST00000379440.9; ENSP00000368752.4; NM_000311.5; NP_000302.1 |
Organism-specific databases
CTDi | 5621 |
DisGeNETi | 5621 |
GeneCardsi | PRNP |
GeneReviewsi | PRNP |
HGNCi | HGNC:9449, PRNP |
HPAi | ENSG00000171867, Low tissue specificity |
MalaCardsi | PRNP |
MIMi | 123400, phenotype 137440, phenotype 176640, gene 245300, phenotype 600072, phenotype 603218, phenotype 606688, phenotype |
neXtProti | NX_P04156 |
OpenTargetsi | ENSG00000171867 |
Orphaneti | 280397, Familial Alzheimer-like prion disease 466, Fatal familial insomnia 356, Gerstmann-Straussler-Scheinker syndrome 157941, Huntington disease-like 1 282166, Inherited Creutzfeldt-Jakob disease 454745, Kuru 397606, PrP systemic amyloidosis 586130, Sporadic fatal insomnia |
PharmGKBi | PA33796 |
VEuPathDBi | HostDB:ENSG00000171867 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | ENOG502S2A8, Eukaryota |
GeneTreei | ENSGT00510000049083 |
InParanoidi | P04156 |
OMAi | HNPGYPH |
OrthoDBi | 957343at2759 |
PhylomeDBi | P04156 |
TreeFami | TF105188 |
Enzyme and pathway databases
PathwayCommonsi | P04156 |
Reactomei | R-HSA-419037, NCAM1 interactions R-HSA-9609523, Insertion of tail-anchored proteins into the endoplasmic reticulum membrane |
SignaLinki | P04156 |
Miscellaneous databases
BioGRID-ORCSi | 5621, 4 hits in 1053 CRISPR screens |
ChiTaRSi | PRNP, human |
EvolutionaryTracei | P04156 |
GenomeRNAii | 5621 |
Pharosi | P04156, Tchem |
RNActi | P04156, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000171867, Expressed in Brodmann (1909) area 23 and 254 other tissues |
ExpressionAtlasi | P04156, baseline and differential |
Genevisiblei | P04156, HS |
Family and domain databases
DisProti | DP00466 |
Gene3Di | 1.10.790.10, 1 hit |
InterProi | View protein in InterPro IPR000817, Prion IPR036924, Prion/Doppel_b-ribbon_dom_sf IPR022416, Prion/Doppel_prot_b-ribbon_dom IPR025860, Prion_N_dom |
Pfami | View protein in Pfam PF00377, Prion, 1 hit PF11587, Prion_bPrPp, 1 hit |
PRINTSi | PR00341, PRION |
SMARTi | View protein in SMART SM00157, PRP, 1 hit |
SUPFAMi | SSF54098, SSF54098, 1 hit |
PROSITEi | View protein in PROSITE PS00291, PRION_1, 1 hit PS00706, PRION_2, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | PRIO_HUMAN | |
Accessioni | P04156Primary (citable) accession number: P04156 Secondary accession number(s): O60489 Q9UP19 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | November 1, 1986 |
Last sequence update: | November 1, 1986 | |
Last modified: | February 23, 2022 | |
This is version 261 of the entry and version 1 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. |
Miscellaneousi
Keywords - Technical termi
3D-structure, Direct protein sequencing, Reference proteomeDocuments
- Human cell differentiation molecules
CD nomenclature of surface proteins of human leucocytes and list of entries - Human chromosome 20
Human chromosome 20: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PDB cross-references
Index of Protein Data Bank (PDB) cross-references - SIMILARITY comments
Index of protein domains and families - Protein Spotlight
Protein Spotlight articles and cited UniProtKB/Swiss-Prot entries