Entity Details

Primary name PRIO_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionP04156
EntryNamePRIO_HUMAN
FullNameMajor prion protein
TaxID9606
Evidenceevidence at protein level
Length253
SequenceStatuscomplete
DateCreated1986-11-01
DateModified2021-06-02

Ontological Relatives

GenesPRNP

GO terms

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GOName
GO:0001540 amyloid-beta binding
GO:0001933 negative regulation of protein phosphorylation
GO:0002020 protease binding
GO:0005507 copper ion binding
GO:0005509 calcium ion binding
GO:0005539 glycosaminoglycan binding
GO:0005634 nucleus
GO:0005737 cytoplasm
GO:0005783 endoplasmic reticulum
GO:0005794 Golgi apparatus
GO:0005829 cytosol
GO:0005886 plasma membrane
GO:0006878 cellular copper ion homeostasis
GO:0006979 response to oxidative stress
GO:0007611 learning or memory
GO:0007616 long-term memory
GO:0008017 microtubule binding
GO:0009986 cell surface
GO:0010955 negative regulation of protein processing
GO:0014069 postsynaptic density
GO:0015631 tubulin binding
GO:0016234 inclusion body
GO:0019828 aspartic-type endopeptidase inhibitor activity
GO:0019898 extrinsic component of membrane
GO:0031362 anchored component of external side of plasma membrane
GO:0031648 protein destabilization
GO:0031802 type 5 metabotropic glutamate receptor binding
GO:0031965 nuclear membrane
GO:0032689 negative regulation of interferon-gamma production
GO:0032700 negative regulation of interleukin-17 production
GO:0032703 negative regulation of interleukin-2 production
GO:0035584 calcium-mediated signaling using intracellular calcium source
GO:0038023 signaling receptor activity
GO:0042802 identical protein binding
GO:0043231 intracellular membrane-bounded organelle
GO:0043433 negative regulation of DNA-binding transcription factor activity
GO:0043525 positive regulation of neuron apoptotic process
GO:0044877 protein-containing complex binding
GO:0045048 protein insertion into ER membrane
GO:0045121 membrane raft
GO:0046007 negative regulation of activated T cell proliferation
GO:0050730 regulation of peptidyl-tyrosine phosphorylation
GO:0050731 positive regulation of peptidyl-tyrosine phosphorylation
GO:0050860 negative regulation of T cell receptor signaling pathway
GO:0051260 protein homooligomerization
GO:0061098 positive regulation of protein tyrosine kinase activity
GO:0070062 extracellular exosome
GO:0070885 negative regulation of calcineurin-NFAT signaling cascade
GO:0071280 cellular response to copper ion
GO:0090314 positive regulation of protein targeting to membrane
GO:0090647 modulation of age-related behavioral decline
GO:0097062 dendritic spine maintenance
GO:0098794 postsynapse
GO:1900449 regulation of glutamate receptor signaling pathway
GO:1901216 positive regulation of neuron death
GO:1902430 negative regulation of amyloid-beta formation
GO:1902938 regulation of intracellular calcium activated chloride channel activity
GO:1902951 negative regulation of dendritic spine maintenance
GO:1902992 negative regulation of amyloid precursor protein catabolic process
GO:1903136 cuprous ion binding
GO:1904645 response to amyloid-beta
GO:1904646 cellular response to amyloid-beta
GO:1905664 regulation of calcium ion import across plasma membrane
GO:1990535 neuron projection maintenance

Subcellular Location

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Subcellular Location
Cell membrane
Golgi apparatus

Domains

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DomainNameCategoryType
IPR000817 Prion proteinFamilyFamily
IPR022416 Prion/Doppel protein, beta-ribbon domainDomainDomain
IPR025860 Major prion protein N-terminal domainDomainDomain
IPR036924 Prion/Doppel beta-ribbon domain superfamilyFamilyHomologous superfamily

Diseases

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Disease IDSourceNameDescription
606688 OMIMSpongiform encephalopathy with neuropsychiatric features (SENF)Autosomal dominant presenile dementia with a rapidly progressive and protracted clinical course. The dementia was characterized clinically by frontotemporal features, including early personality changes. Some patients had memory loss, several showed aggressiveness, hyperorality and verbal stereotypy, others had parkinsonian symptoms. The disease is caused by variants affecting the gene represented in this entry.
603218 OMIMHuntington disease-like 1 (HDL1)Autosomal dominant, early-onset neurodegenerative disorder with prominent psychiatric features. The disease is caused by variants affecting the gene represented in this entry.
137440 OMIMGerstmann-Straussler disease (GSD)A rare inherited prion disease characterized by adult onset of memory loss, dementia, ataxia, and pathologic deposition of amyloid-like plaques in the brain. GSD presents with progressive limb and truncal ataxia, dysarthria, and cognitive decline in the thirties and forties, and the average disease duration is 7 years. The disease is caused by variants affecting the gene represented in this entry.
123400 OMIMCreutzfeldt-Jakob disease (CJD)Occurs primarily as a sporadic disorder (1 per million), while 10-15% are familial. Accidental transmission of CJD to humans appears to be iatrogenic (contaminated human growth hormone (HGH), corneal transplantation, electroencephalographic electrode implantation, etc.). Epidemiologic studies have failed to implicate the ingestion of infected animal meat in the pathogenesis of CJD in human. The triad of microscopic features that characterize the prion diseases consists of (1) spongiform degeneration of neurons, (2) severe astrocytic gliosis that often appears to be out of proportion to the degree of nerve cell loss, and (3) amyloid plaque formation. CJD is characterized by progressive dementia and myoclonic seizures, affecting adults in mid-life. Some patients present sleep disorders, abnormalities of high cortical function, cerebellar and corticospinal disturbances. The disease ends in death after a 3-12 months illness. The disease is caused by variants affecting the gene represented in this entry.
600072 OMIMFatal familial insomnia (FFI)Autosomal dominant disorder and is characterized by neuronal degeneration limited to selected thalamic nuclei and progressive insomnia. The disease is caused by variants affecting the gene represented in this entry.
245300 OMIMKuru (KURU)Kuru is transmitted during ritualistic cannibalism, among natives of the New Guinea highlands. Patients exhibit various movement disorders like cerebellar abnormalities, rigidity of the limbs, and clonus. Emotional lability is present, and dementia is conspicuously absent. Death usually occurs from 3 to 12 month after onset. Disease susceptibility is associated with variants affecting the gene represented in this entry.

Drugs

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DrugNameSourceType
DB00759 TetracyclineDrugbanksmall molecule
DB09130 CopperDrugbanksmall molecule

Interactions

131 interactions

InteractorPartnerSourcesPublicationsLink
PRIO_HUMANBAG6_HUMANBioGRID, HPRD, IntAct16169070 details
PRIO_HUMANT22D4_HUMANBioGRID, HPRD, IntAct16713569 details
PRIO_HUMANPSA3_HUMANBioGRID, HPRD, IntAct16713569 details
PRIO_HUMANPRIO_HUMANDIP, IntAct, MINT16286452 18025469 18436646 19204296 19278656 19927125 21920025 32284600 32514176 details
PRIO_HUMANA4_HUMANIntAct16286452 23236467 24028865 32814053 details
PRIO_HUMANPIMRE_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANPLK3_HUMANBioGRID, IntAct18482256 details
PRIO_HUMAN3MG_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANHXA1_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANBAZ2B_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANCASK_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANCG050_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANSURF2_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANSCNM1_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANTYMOS_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANRBM22_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANCC149_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANTSLP_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANDDX47_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANZKSC8_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANMARK4_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANZN408_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANTBPL1_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANABT1_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANRL41_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANZN740_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANFHL1_HUMANBioGRID, IntAct, UniProt18482256 28671123 details
PRIO_HUMANCWC15_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANTM237_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANIEX1_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANP33MX_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANMYOME_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANPTH_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANMTG1_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANRHG15_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANUTP4_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANAGO1_HUMANBioGRID, DIP, IntAct18482256 22484317 details
PRIO_HUMANWDR5_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANADAP2_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANPP14A_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANLST2_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANCIRBP_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANNHP2_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANPYM1_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANF27E3_HUMANIntAct18482256 details
PRIO_HUMANLIPB2_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANCN37_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANFGF13_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANAIFM3_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANRNPC3_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANNOB1_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANDYRK3_HUMANBioGRID, IntAct18482256 details
PRIO_HUMANHD_HUMANIntAct17500595 32814053 details
PRIO_HUMANPAR2_HUMANBioGRID, MINT28298427 details
PRIO_HUMANFZD7_HUMANBioGRID, MINT28298427 details
PRIO_HUMANADRB2_HUMANBioGRID, MINT28298427 details
PRIO_HUMANCCR1_HUMANBioGRID, MINT28298427 details
PRIO_HUMANPTAFR_HUMANBioGRID, MINT28298427 details
PRIO_HUMANVATB2_HUMANBioGRID, UniProt21301993 29791485 details
PRIO_HUMANSTIP1_HUMANBioGRID, HPRD, UniProt12093732 29791485 details
PRIO_HUMANGRB2_HUMANBioGRID, HPRD, UniProt11571277 29791485 details
PRIO_HUMANNCAM1_HUMANBioGRID, HPRD, UniProt15146195 27535221 29791485 details
PRIO_HUMANSAMD3_HUMANIntAct32814053 details
PRIO_HUMANATLA1_HUMANIntAct32814053 details
PRIO_HUMANCLUS_HUMANBioGRID, HPRD15146195 18786636 details
PRIO_HUMANBIP_HUMANBioGRID, HPRD10970892 15146195 details
PRIO_HUMANTRI15_HUMANBioGRID25450970 details
PRIO_HUMANLMNA_HUMANBioGRID24623722 details
PRIO_HUMANHAX1_HUMANBioGRID21301993 details
PRIO_HUMANMMAD_HUMANBioGRID21301993 details
PRIO_HUMANCRYAB_HUMANBioGRID21301993 details
PRIO_HUMANSTAR5_HUMANBioGRID21301993 details
PRIO_HUMANAPLP1_HUMANBioGRID, HPRD15146195 21301993 details
PRIO_HUMANKIFA3_HUMANBioGRID21301993 details
PRIO_HUMANPSB5_HUMANBioGRID21301993 details
PRIO_HUMANFBSP1_HUMANBioGRID21301993 details
PRIO_HUMANFBLN5_HUMANBioGRID21301993 details
PRIO_HUMANNIPS2_HUMANBioGRID21301993 details
PRIO_HUMANPUR9_HUMANBioGRID21301993 details
PRIO_HUMANDOCK7_HUMANBioGRID21301993 details
PRIO_HUMANWDR61_HUMANBioGRID21301993 details
PRIO_HUMANSC31A_HUMANBioGRID21301993 details
PRIO_HUMANGRM5_HUMANBioGRID25148681 details
PRIO_HUMANPPARG_HUMANIntAct20195357 details
PRIO_HUMANTAU_HUMANIntAct24028865 details
PRIO_HUMANAGO2_HUMANDIP22484317 details
PRIO_HUMANPRDX1_HUMANUniProt28671123 details
PRIO_HUMANZA2G_HUMANUniProt28671123 details
PRIO_HUMANCSTN1_HUMANBioGRID, HPRD, UniProt15146195 29791485 details
PRIO_HUMANPDIA1_HUMANBioGRID, HPRD, UniProt15146195 29791485 details
PRIO_HUMANMAG_HUMANBioGRID, HPRD, UniProt15146195 29791485 details
PRIO_HUMANL1CAM_HUMANBioGRID, HPRD, UniProt15146195 29791485 details
PRIO_HUMANSRRM2_HUMANBioGRID16159877 details
PRIO_HUMANHSP74_HUMANBioGRID20964628 details
PRIO_HUMANARF1_HUMANBioGRID25896910 details
PRIO_HUMANCSK21_HUMANBioGRID, HPRD11062072 28900035 details
PRIO_HUMANZBED3_HUMANBioGRID28900035 details
PRIO_HUMAN1433T_HUMANBioGRID28900035 details
PRIO_HUMANPABP1_HUMANBioGRID28900035 29791485 details
PRIO_HUMANPABP4_HUMANBioGRID28900035 details
PRIO_HUMANCH60_HUMANBioGRID, HPRD28900035 8676499 details
PRIO_HUMANCYLD_HUMANBioGRID28900035 details
PRIO_HUMANHS90A_HUMANBioGRID28900035 details
PRIO_HUMANRSSA_HUMANBioGRID28759037 28900035 details
PRIO_HUMANPSMD2_HUMANBioGRID28900035 details
PRIO_HUMANAMFR_HUMANBioGRID28759037 details
PRIO_HUMANACHA7_HUMANBioGRID20837487 details
PRIO_HUMANNOS1_HUMANBioGRID11032878 details
PRIO_HUMANDAG1_HUMANBioGRID11032878 details
PRIO_HUMANDMD_HUMANBioGRID11032878 details
PRIO_HUMANSNTA1_HUMANBioGRID11032878 details
PRIO_HUMANDCE1_HUMANBioGRID11032878 details
PRIO_HUMANSYPH_HUMANBioGRID11032878 details
PRIO_HUMANPLMN_HUMANHPRD11100730 details
PRIO_HUMANCAV1_HUMANHPRD10988071 details
PRIO_HUMANNCAM2_HUMANHPRD15146195 details
PRIO_HUMANDYN1_HUMANHPRD15146195 details
PRIO_HUMANAPBB1_HUMANHPRD15146195 details
PRIO_HUMANADA23_HUMANHPRD15146195 details
PRIO_HUMANSPRL1_HUMANHPRD15146195 details
PRIO_HUMANCNTN1_HUMANHPRD15146195 details
PRIO_HUMANOPCM_HUMANHPRD15146195 details
PRIO_HUMANNECT1_HUMANHPRD15146195 details
PRIO_HUMANLSAMP_HUMANHPRD15146195 details
PRIO_HUMANMOG_HUMANHPRD15146195 details
PRIO_HUMANERI3_HUMANHPRD11571277 details
PRIO_HUMANAPOE_HUMANHPRD15146195 details
PRIO_HUMANCSK22_HUMANHPRD11062072 details
PRIO_HUMANCSK2B_HUMANHPRD11062072 details
PRIO_HUMANDPP6_HUMANHPRD15146195 details
PRIO_HUMANAPLP2_HUMANHPRD15146195 details