Prnp prion protein [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Prnpprovided by MGI
Official Full Name: prion proteinprovided by MGI
Primary source: MGI:MGI:97769
See related: Ensembl:ENSMUSG00000079037 AllianceGenome:MGI:97769
Gene type: protein coding
RefSeq status: VALIDATED
Organism: Mus musculus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as: PrP; PrPC; Sinc; CD230; PrPSc; Prn-i; Prn-p; PrP<C>; prP27-30; prP33-35C
Summary: Enables several functions, including amyloid-beta binding activity; aspartic-type endopeptidase inhibitor activity; and cupric ion binding activity. Involved in several processes, including negative regulation of macromolecule metabolic process; positive regulation of protein phosphorylation; and regulation of signal transduction. Acts upstream of or within several processes, including negative regulation of apoptotic process; regulation of potassium ion transmembrane transport; and response to oxidative stress. Located in several cellular components, including Golgi apparatus; membrane raft; and terminal bouton. Colocalizes with dendrite. Is expressed in several structures, including cardiovascular system; genitourinary system; nervous system; sensory organ; and tooth. Used to study Creutzfeldt-Jakob disease; Gerstmann-Straussler-Scheinker syndrome; and fatal familial insomnia. Human ortholog(s) of this gene implicated in Creutzfeldt-Jakob disease; Gerstmann-Straussler-Scheinker syndrome; Huntington's disease-like 1; fatal familial insomnia; and kuru. Orthologous to human PRNP (prion protein (Kanno blood group)). [provided by Alliance of Genome Resources, Nov 2024]
Expression: Broad expression in cortex adult (RPKM 102.3), frontal lobe adult (RPKM 102.0) and 23 other tissues See more
Orthologs: human all
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Genomic context

Location:: 2 F2; 2 64.07 cM

Exon count:: 4

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	2	NC_000068.8 (131751848..131780356)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	2	NC_000068.7 (131909928..131938436)

Chromosome 2 - NC_000068.8 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Expression

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See details

Project title: Mouse ENCODE transcriptome data
Description: RNA profiling data sets generated by the Mouse ENCODE project.
BioProject: PRJNA66167
Publication: PMID 25409824
Analysis date: n/a

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

A PrP EGFR signaling axis controls neural stem cell senescence through modulating cellular energy pathways.
Title: A PrP EGFR signaling axis controls neural stem cell senescence through modulating cellular energy pathways.
Mutations of evolutionarily conserved aromatic residues suggest that misfolding of the mouse prion protein may commence in multiple ways.
Title: Mutations of evolutionarily conserved aromatic residues suggest that misfolding of the mouse prion protein may commence in multiple ways.
Full-length prion protein incorporated into prion aggregates is a marker for prion strain-specific destabilization of aggregate structure following cellular uptake.
Title: Full-length prion protein incorporated into prion aggregates is a marker for prion strain-specific destabilization of aggregate structure following cellular uptake.
Cellular prion protein offers neuroprotection in astrocytes submitted to amyloid beta oligomer toxicity.
Title: Cellular prion protein offers neuroprotection in astrocytes submitted to amyloid β oligomer toxicity.
RAB7A GTPase Is Involved in Mitophagosome Formation and Autophagosome-Lysosome Fusion in N2a Cells Treated with the Prion Protein Fragment 106-126.
Title: RAB7A GTPase Is Involved in Mitophagosome Formation and Autophagosome-Lysosome Fusion in N2a Cells Treated with the Prion Protein Fragment 106-126.
Enhanced M-CSF/CSF1R Signaling Closely Associates with PrP(Sc) Accumulation in the Scrapie-Infected Cell Line and the Brains of Scrapie-Infected Experimental Rodents.
Title: Enhanced M-CSF/CSF1R Signaling Closely Associates with PrP(Sc) Accumulation in the Scrapie-Infected Cell Line and the Brains of Scrapie-Infected Experimental Rodents.
The E3 Ubiquitin Ligase TRAF6 Interacts with the Cellular Prion Protein and Modulates Its Solubility and Recruitment to Cytoplasmic p62/SQSTM1-Positive Aggresome-Like Structures.
Title: The E3 Ubiquitin Ligase TRAF6 Interacts with the Cellular Prion Protein and Modulates Its Solubility and Recruitment to Cytoplasmic p62/SQSTM1-Positive Aggresome-Like Structures.
Dissociation of genotype-dependent cognitive and motor behavior in a strain of aging mice devoid of the prion protein.
Title: Dissociation of genotype-dependent cognitive and motor behavior in a strain of aging mice devoid of the prion protein.
A Soluble PrP(C) Derivative and Membrane-Anchored PrP(C) in Extracellular Vesicles Attenuate Innate Immunity by Engaging the NMDA-R/LRP1 Receptor Complex.
Title: A Soluble PrP(C) Derivative and Membrane-Anchored PrP(C) in Extracellular Vesicles Attenuate Innate Immunity by Engaging the NMDA-R/LRP1 Receptor Complex.
The Effects of Fibrinogen's Interactions with Its Neuronal Receptors, Intercellular Adhesion Molecule-1 and Cellular Prion Protein.
Title: The Effects of Fibrinogen's Interactions with Its Neuronal Receptors, Intercellular Adhesion Molecule-1 and Cellular Prion Protein.

Submit: New GeneRIF Correction See all GeneRIFs (508)

Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Endonuclease-mediated (6) 1 citation
Spontaneous (3) 1 citation
Targeted (29) 1 citation

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

M13685 (e-PCR)
- UniSTS:178717

AI325101 (e-PCR)
- UniSTS:178720

AA960666 (e-PCR)
- UniSTS:159038

px-35d12 (e-PCR)
- UniSTS:257632

px-50g7 (e-PCR)
- UniSTS:257891

px-36f4 (e-PCR)
- UniSTS:257660

NoName (e-PCR)
- UniSTS:548231

Prnp (e-PCR), detects polymorphism
- UniSTS:144265

Prnp (e-PCR), detects polymorphism
- UniSTS:536461

PMC125625P1 (e-PCR)
- UniSTS:270503

px-58e10 (e-PCR)
- UniSTS:257966

PMC136595P2 (e-PCR)
- UniSTS:270799

PMC136859P1 (e-PCR)
- UniSTS:270806

NoName (e-PCR)
- UniSTS:224477

Readthrough Prn

Readthrough gene: Prn, Included gene: Prnd

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables ATP-dependent protein binding	IEA Inferred from Electronic Annotation more info
enables ATP-dependent protein binding	ISO Inferred from Sequence Orthology more info
enables amyloid-beta binding	IDA Inferred from Direct Assay more info	PubMed
enables amyloid-beta binding	ISO Inferred from Sequence Orthology more info
enables amyloid-beta binding	TAS Traceable Author Statement more info	PubMed
enables aspartic-type endopeptidase inhibitor activity	IDA Inferred from Direct Assay more info	PubMed
enables copper ion binding	IDA Inferred from Direct Assay more info	PubMed
enables copper ion binding	ISO Inferred from Sequence Orthology more info
enables copper ion binding	ISS Inferred from Sequence or Structural Similarity more info
enables cupric ion binding	IMP Inferred from Mutant Phenotype more info	PubMed
enables cuprous ion binding	IEA Inferred from Electronic Annotation more info
enables cuprous ion binding	ISO Inferred from Sequence Orthology more info
enables glycosaminoglycan binding	IDA Inferred from Direct Assay more info	PubMed
enables identical protein binding	IPI Inferred from Physical Interaction more info	PubMed
enables identical protein binding	ISO Inferred from Sequence Orthology more info
enables lamin binding	IEA Inferred from Electronic Annotation more info
enables lamin binding	ISO Inferred from Sequence Orthology more info
enables metal ion binding	EXP Inferred from Experiment more info	PubMed
enables metal ion binding	IDA Inferred from Direct Assay more info	PubMed
enables microtubule binding	IEA Inferred from Electronic Annotation more info
enables microtubule binding	ISO Inferred from Sequence Orthology more info
enables molecular adaptor activity	EXP Inferred from Experiment more info	PubMed
enables molecular adaptor activity	IPI Inferred from Physical Interaction more info	PubMed
enables molecular adaptor activity	ISO Inferred from Sequence Orthology more info
enables molecular condensate scaffold activity	EXP Inferred from Experiment more info	PubMed
enables molecular condensate scaffold activity	IDA Inferred from Direct Assay more info	PubMed
enables molecular condensate scaffold activity	ISO Inferred from Sequence Orthology more info
enables protease binding	IPI Inferred from Physical Interaction more info	PubMed
enables protein binding	IPI Inferred from Physical Interaction more info	PubMed
enables protein-containing complex binding	IPI Inferred from Physical Interaction more info	PubMed
enables protein-containing complex binding	ISO Inferred from Sequence Orthology more info
enables protein-folding chaperone binding	IEA Inferred from Electronic Annotation more info
enables protein-folding chaperone binding	ISO Inferred from Sequence Orthology more info
enables signaling receptor activity	IMP Inferred from Mutant Phenotype more info	PubMed
enables transmembrane transporter binding	IEA Inferred from Electronic Annotation more info
enables transmembrane transporter binding	ISO Inferred from Sequence Orthology more info
enables tubulin binding	ISO Inferred from Sequence Orthology more info
enables type 5 metabotropic glutamate receptor binding	IPI Inferred from Physical Interaction more info	PubMed
enables type 5 metabotropic glutamate receptor binding	ISO Inferred from Sequence Orthology more info

Process	Evidence Code	Pubs
involved_in activation of protein kinase activity	IGI Inferred from Genetic Interaction more info	PubMed
NOT involved_in amyloid precursor protein metabolic process	IGI Inferred from Genetic Interaction more info	PubMed
involved_in cellular response to amyloid-beta	IGI Inferred from Genetic Interaction more info	PubMed
involved_in cellular response to amyloid-beta	ISO Inferred from Sequence Orthology more info
acts_upstream_of_or_within cellular response to copper ion	ISO Inferred from Sequence Orthology more info	PubMed
acts_upstream_of_or_within cellular response to xenobiotic stimulus	IDA Inferred from Direct Assay more info	PubMed
acts_upstream_of_or_within intracellular copper ion homeostasis	TAS Traceable Author Statement more info	PubMed
involved_in learning or memory	IGI Inferred from Genetic Interaction more info	PubMed
involved_in learning or memory	ISO Inferred from Sequence Orthology more info
involved_in negative regulation of DNA-binding transcription factor activity	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in negative regulation of T cell receptor signaling pathway	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in negative regulation of activated T cell proliferation	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in negative regulation of amyloid precursor protein catabolic process	IGI Inferred from Genetic Interaction more info	PubMed
involved_in negative regulation of amyloid-beta formation	IGI Inferred from Genetic Interaction more info	PubMed
acts_upstream_of_or_within negative regulation of apoptotic process	IGI Inferred from Genetic Interaction more info	PubMed
involved_in negative regulation of apoptotic process	ISO Inferred from Sequence Orthology more info
involved_in negative regulation of calcineurin-NFAT signaling cascade	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in negative regulation of dendritic spine maintenance	IGI Inferred from Genetic Interaction more info	PubMed
involved_in negative regulation of interleukin-17 production	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in negative regulation of interleukin-2 production	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in negative regulation of long-term synaptic potentiation	IEA Inferred from Electronic Annotation more info
involved_in negative regulation of long-term synaptic potentiation	ISO Inferred from Sequence Orthology more info
involved_in negative regulation of protein phosphorylation	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in negative regulation of type II interferon production	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in neuron projection maintenance	IGI Inferred from Genetic Interaction more info	PubMed
acts_upstream_of_or_within nucleobase-containing compound metabolic process	TAS Traceable Author Statement more info	PubMed
involved_in positive regulation of calcium-mediated signaling	IGI Inferred from Genetic Interaction more info	PubMed
involved_in positive regulation of calcium-mediated signaling	ISO Inferred from Sequence Orthology more info
involved_in positive regulation of glutamate receptor signaling pathway	IEA Inferred from Electronic Annotation more info
involved_in positive regulation of glutamate receptor signaling pathway	ISO Inferred from Sequence Orthology more info
involved_in positive regulation of neuron apoptotic process	IEA Inferred from Electronic Annotation more info
involved_in positive regulation of neuron apoptotic process	ISO Inferred from Sequence Orthology more info
involved_in positive regulation of peptidyl-tyrosine phosphorylation	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in positive regulation of peptidyl-tyrosine phosphorylation	ISO Inferred from Sequence Orthology more info
involved_in positive regulation of protein localization to plasma membrane	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in positive regulation of protein localization to plasma membrane	ISO Inferred from Sequence Orthology more info
involved_in positive regulation of protein targeting to membrane	IGI Inferred from Genetic Interaction more info	PubMed
involved_in protein destabilization	IEA Inferred from Electronic Annotation more info
involved_in protein destabilization	ISO Inferred from Sequence Orthology more info
involved_in protein homooligomerization	IEA Inferred from Electronic Annotation more info
involved_in regulation of calcium ion import across plasma membrane	IGI Inferred from Genetic Interaction more info	PubMed
involved_in regulation of glutamate receptor signaling pathway	IGI Inferred from Genetic Interaction more info	PubMed
involved_in regulation of peptidyl-tyrosine phosphorylation	IGI Inferred from Genetic Interaction more info	PubMed
acts_upstream_of_or_within regulation of potassium ion transmembrane transport	IGI Inferred from Genetic Interaction more info	PubMed
acts_upstream_of_or_within regulation of protein localization	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in response to amyloid-beta	IGI Inferred from Genetic Interaction more info	PubMed
involved_in response to cadmium ion	IEA Inferred from Electronic Annotation more info
involved_in response to cadmium ion	ISO Inferred from Sequence Orthology more info
involved_in response to copper ion	ISO Inferred from Sequence Orthology more info
acts_upstream_of_or_within response to oxidative stress	IDA Inferred from Direct Assay more info	PubMed

Component	Evidence Code	Pubs
located_in Golgi apparatus	IDA Inferred from Direct Assay more info	PubMed
located_in cell surface	IDA Inferred from Direct Assay more info	PubMed
located_in cell surface	ISO Inferred from Sequence Orthology more info
located_in cytosol	IEA Inferred from Electronic Annotation more info
located_in cytosol	ISO Inferred from Sequence Orthology more info
colocalizes_with dendrite	IDA Inferred from Direct Assay more info	PubMed
is_active_in dendrite	IDA Inferred from Direct Assay more info	PubMed
is_active_in dendrite	ISO Inferred from Sequence Orthology more info
located_in endoplasmic reticulum	IDA Inferred from Direct Assay more info	PubMed
located_in inclusion body	IEA Inferred from Electronic Annotation more info
located_in inclusion body	ISO Inferred from Sequence Orthology more info
located_in intracellular membrane-bounded organelle	ISO Inferred from Sequence Orthology more info
located_in membrane	IDA Inferred from Direct Assay more info	PubMed
located_in membrane raft	IDA Inferred from Direct Assay more info	PubMed
located_in membrane raft	IMP Inferred from Mutant Phenotype more info	PubMed
located_in membrane raft	ISO Inferred from Sequence Orthology more info	PubMed
located_in membrane raft	TAS Traceable Author Statement more info	PubMed
located_in mitochondrial outer membrane	ISO Inferred from Sequence Orthology more info
located_in nuclear membrane	IEA Inferred from Electronic Annotation more info
located_in nuclear membrane	ISO Inferred from Sequence Orthology more info
located_in plasma membrane	IDA Inferred from Direct Assay more info	PubMed
located_in plasma membrane	ISO Inferred from Sequence Orthology more info	PubMed
located_in plasma membrane	TAS Traceable Author Statement more info
located_in postsynaptic density	IEA Inferred from Electronic Annotation more info
located_in postsynaptic density	ISO Inferred from Sequence Orthology more info
located_in side of membrane	IEA Inferred from Electronic Annotation more info
located_in terminal bouton	IDA Inferred from Direct Assay more info	PubMed

General protein information

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Preferred Names: major prion protein

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

NM_001278256.1 → NP_001265185.1 major prion protein precursor

See identical proteins and their annotated locations for NP_001265185.1

Status: VALIDATED

Description

Transcript Variant: This variant (2) differs in the 5' UTR compared to variant 1. Variants 1 and 2 encode the same protein.

Source sequence(s)

AK158908, AK159056, AV361844, CK622972

Consensus CDS

CCDS16766.1

UniProtKB/Swiss-Prot

P04925

UniProtKB/TrEMBL

Q3TZI7, Q4FJQ7

Conserved Domains (2) summary

smart00157
Location:23 → 241

PRP; Major prion protein

pfam11587
Location:1 → 28

Prion_bPrPp; Major prion protein bPrPp - N terminal
NM_011170.3 → NP_035300.1 major prion protein precursor

See identical proteins and their annotated locations for NP_035300.1

Status: VALIDATED

Description

Transcript Variant: This variant (1) represents the shorter transcript. Variants 1 and 2 encode the same protein.

Source sequence(s)

AK148061, AK158908, AV361844, CK622972

Consensus CDS

CCDS16766.1

UniProtKB/Swiss-Prot

P04925

UniProtKB/TrEMBL

Q3TZI7, Q4FJQ7

Related

ENSMUSP00000088833.7, ENSMUST00000091288.13

Conserved Domains (2) summary

smart00157
Location:23 → 241

PRP; Major prion protein

pfam11587
Location:1 → 28

Prion_bPrPp; Major prion protein bPrPp - N terminal