Reviewed,
UniProtKB/Swiss-Prot P04925 (PRIO_MOUSE)
Last modified
June 16, 2009.
Version 108.
History...
Clusters with 100%,
90%,
50% identity |
Documents (3) |
Third-party data |
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Names and origin
| Protein names | Recommended name: Major prion protein Short name=PrP Alternative name(s): PrP27-30 PrP33-35C CD_antigen=CD230 | ||||
| Gene names |
| ||||
| Organism | Mus musculus (Mouse) | ||||
| Taxonomic identifier | 10090 [NCBI] | ||||
| Taxonomic lineage | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Glires › Rodentia › Sciurognathi › Muroidea › Muridae › Murinae › Mus |
Protein attributes
| Sequence length | 254 AA. |
| Sequence status | Complete. |
| Sequence processing | The displayed sequence is further processed into a mature form. |
| Protein existence | Evidence at protein level. |
General annotation (Comments)
| Function | The function of PrP is not known. PrP is encoded in the host genome and is expressed both in normal and infected cells. |
| Subunit structure | PrP has a tendency to aggregate yielding polymers called "rods". Interacts with GRB2, PRNPIP and SYN1. Ref.8 |
| Subcellular location | Cell membrane; Lipid-anchor › GPI-anchor. Golgi apparatus. Ref.8 |
| Tissue specificity | Highly expressed in the brain, lung, kidney and heart. Expressed at low levels in the liver and spleen. |
| Involvement in disease | Found in high quantity in the brain of humans and animals infected with degenerative neurological diseases such as kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler syndrome (GSS), scrapie, bovine spongiform encephalopathy (BSE), transmissible mink encephalopathy (TME), etc. |
| Sequence similarities | Belongs to the prion family. |
Ontologies
Binary interactions
With | Entry | #Exp. | IntAct | Notes |
|---|---|---|---|---|
| HNRNPA2B1 | P22626 | 1 | EBI-768613,EBI-299649 | From a different organism. |
Sequence annotation (Features)
| Feature key | Position(s) | Length | Description | Graphical view | Feature identifier | ||||||||||||||||||
Molecule processing | |||||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Signal peptide | 1 – 22 | 22 | |||||||||||||||||||||
| Chain | 23 – 230 | 208 | Major prion protein | PRO_0000025697 | |||||||||||||||||||
| Propeptide | 231 – 254 | 24 | Removed in mature form By similarity | PRO_0000025698 | |||||||||||||||||||
Regions | |||||||||||||||||||||||
| Repeat | 51 – 58 | 8 | 1 | ||||||||||||||||||||
| Repeat | 59 – 66 | 8 | 2 | ||||||||||||||||||||
| Repeat | 67 – 74 | 8 | 3 | ||||||||||||||||||||
| Repeat | 75 – 82 | 8 | 4 | ||||||||||||||||||||
| Repeat | 83 – 90 | 8 | 5 | ||||||||||||||||||||
| Region | 23 – 231 | 209 | Interaction with GRB2, PRNPIP and SYN1 | ||||||||||||||||||||
| Region | 51 – 90 | 40 | 5 X 8 AA tandem repeats of P-H-G-G-G-W-G-Q | ||||||||||||||||||||
Amino acid modifications | |||||||||||||||||||||||
| Modified residue | 44 | 1 | Hydroxyproline | ||||||||||||||||||||
| Lipidation | 230 | 1 | GPI-anchor amidated serine By similarity | ||||||||||||||||||||
| Glycosylation | 180 | 1 | N-linked (GlcNAc...) Probable | ||||||||||||||||||||
| Glycosylation | 196 | 1 | N-linked (GlcNAc...) Probable | ||||||||||||||||||||
| Disulfide bond | 178 ↔ 213 | ||||||||||||||||||||||
Natural variations | |||||||||||||||||||||||
| Natural variant | 108 | 1 | L → F Linked to long incubation time. | ||||||||||||||||||||
| Natural variant | 189 | 1 | T → V Linked to long incubation time. | ||||||||||||||||||||
Experimental info | |||||||||||||||||||||||
| Mutagenesis | 101 | 1 | P → L: No effect on interaction with GRB2. Ref.8 | ||||||||||||||||||||
| Mutagenesis | 104 | 1 | P → L: No effect on interaction with GRB2. Ref.8 | ||||||||||||||||||||
| Sequence conflict | 133 | 1 | M → V Ref.2 | ||||||||||||||||||||
| Sequence conflict | 133 | 1 | M → V Ref.6 | ||||||||||||||||||||
Secondary structure | |||||||||||||||||||||||
Helix Strand Turn | |||||||||||||||||||||||
| Turn | 124 – 126 | 3 | |||||||||||||||||||||
| Beta strand | 127 – 129 | 3 | |||||||||||||||||||||
| Helix | 143 – 152 | 10 | |||||||||||||||||||||
| Turn | 153 – 155 | 3 | |||||||||||||||||||||
| Beta strand | 166 – 168 | 3 | |||||||||||||||||||||
| Helix | 171 – 191 | 21 | |||||||||||||||||||||
| Turn | 192 – 194 | 3 | |||||||||||||||||||||
| Helix | 199 – 221 | 23 | |||||||||||||||||||||
| Turn | 222 – 224 | 3 | |||||||||||||||||||||
Sequences
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References
| « Hide 'large scale' references | |
| [1] | "Distinct prion proteins in short and long scrapie incubation period mice." Westaway D., Goodman P.A., Mirenda C.A., McKinley M.P., Carlson G.A., Prusiner S.B. Cell 51:651-662(1987) [PubMed: 2890436] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA]. Strain: I/LnJ and NZW. |
| [2] | "Molecular cloning and complete sequence of prion protein cDNA from mouse brain infected with the scrapie agent." Locht C., Chesebro B., Race R., Keith J.M. Proc. Natl. Acad. Sci. U.S.A. 83:6372-6376(1986) [PubMed: 3462700] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA]. |
| [3] | "Molecular pathology of scrapie-associated fibril protein (PrP) in mouse brain affected by the ME7 strain of scrapie." Hope J., Multhaup G., Reekie L.J.D., Kimberlin R.H., Beyreuther K. Eur. J. Biochem. 172:271-277(1988) [PubMed: 2894984] [Abstract] Cited for: NUCLEOTIDE SEQUENCE. |
| [4] | "Complete genomic sequence and analysis of the prion protein gene region from three mammalian species." Lee I.Y., Westaway D., Smit A.F.A., Wang K., Seto J., Chen L., Acharya C., Ankener M., Baskin D., Cooper C., Yao H., Prusiner S.B., Hood L.E. Genome Res. 8:1022-1037(1998) [PubMed: 9799790] [Abstract] Cited for: NUCLEOTIDE SEQUENCE. Strain: NZW. Tissue: Brain. |
| [5] | "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed: 15489334] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. |
| [6] | "Identification of scrapie prion protein-specific mRNA in scrapie-infected and uninfected brain." Chesebro B., Race R., Wehrly K., Nishio J., Bloom M., Lechner D., Bergstrom S., Robbins K., Mayer L., Keith J.M., Garon C., Haase A. Nature 315:331-333(1985) [PubMed: 3923361] [Abstract] Cited for: NUCLEOTIDE SEQUENCE OF 87-164. |
| [7] | "Post-translational hydroxylation at the N-terminus of the prion protein reveals presence of PPII structure in vivo." Gill A.C., Ritchie M.A., Hunt L.G., Steane S.E., Davies K.G., Bocking S.P., Rhie A.G.O., Bennett A.D., Hope J. EMBO J. 19:5324-5331(2000) [PubMed: 11032800] [Abstract] Cited for: HYDROXYLATION AT PRO-44. |
| [8] | "PrPC directly interacts with proteins involved in signaling pathways." Spielhaupter C., Schaetzl H.M. J. Biol. Chem. 276:44604-44612(2001) [PubMed: 11571277] [Abstract] Cited for: SUBCELLULAR LOCATION, INTERACTION WITH GRB2; PRNPIP AND SYN1, MUTAGENESIS OF PRO-101 AND PRO-104. |
| [9] | "NMR structure of the mouse prion protein domain PrP(121-321)." Riek R., Hornemann S., Wider G., Bileter M., Glockshuber R., Wuethrich K. Nature 382:180-182(1996) [PubMed: 8700211] [Abstract] Cited for: STRUCTURE BY NMR OF 120-230. |
| [10] | "NMR characterization of the full-length recombinant murine prion protein, mPrP(23-231)." Riek R., Hornemann S., Wider G., Glockshuber R., Wuethrich K. FEBS Lett. 413:282-288(1997) [PubMed: 9280298] [Abstract] Cited for: STRUCTURE BY NMR OF 23-231. |
| + | Additional computationally mapped references. |
Cross-references
Sequence databases | |||||||||||||||||||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| M18070 Genomic DNA. Translation: AAA39997.1. M18071 Genomic DNA. Translation: AAA39998.1. M13685 mRNA. Translation: AAA39996.1. U29186 Genomic DNA. Translation: AAC02804.1. BC006703 mRNA. Translation: AAH06703.1. M30384 mRNA. Translation: AAA39999.1. | |||||||||||||||||||||||||||||||||||||
| IPI | IPI00120793. | ||||||||||||||||||||||||||||||||||||
| PIR | A23544. A29669. | ||||||||||||||||||||||||||||||||||||
| RefSeq | NP_035300.1. | ||||||||||||||||||||||||||||||||||||
| UniGene | Mm.648 | ||||||||||||||||||||||||||||||||||||
3D structure databases | |||||||||||||||||||||||||||||||||||||
| |||||||||||||||||||||||||||||||||||||
| DisProt | DP00265. | ||||||||||||||||||||||||||||||||||||
| ModBase | Search... | ||||||||||||||||||||||||||||||||||||
Protein-protein interaction databases | |||||||||||||||||||||||||||||||||||||
| DIP | DIP:4N. | ||||||||||||||||||||||||||||||||||||
| IntAct | P04925. 3 interactions. | ||||||||||||||||||||||||||||||||||||
Proteomic databases | |||||||||||||||||||||||||||||||||||||
| PRIDE | P04925. | ||||||||||||||||||||||||||||||||||||
Genome annotation databases | |||||||||||||||||||||||||||||||||||||
| Ensembl | ENSMUSG00000079037. Mus musculus. [Contig view] | ||||||||||||||||||||||||||||||||||||
| GeneID | 19122. | ||||||||||||||||||||||||||||||||||||
| KEGG | mmu:19122. | ||||||||||||||||||||||||||||||||||||
Organism-specific databases | |||||||||||||||||||||||||||||||||||||
| MGI | MGI:97769. Prnp. | ||||||||||||||||||||||||||||||||||||
Phylogenomic databases | |||||||||||||||||||||||||||||||||||||
| HOGENOM | P04925. | ||||||||||||||||||||||||||||||||||||
| HOVERGEN | P04925. | ||||||||||||||||||||||||||||||||||||
| OMA | P04925. PNQVYYR. | ||||||||||||||||||||||||||||||||||||
Gene expression databases | |||||||||||||||||||||||||||||||||||||
| ArrayExpress | P04925. | ||||||||||||||||||||||||||||||||||||
| Bgee | P04925. | ||||||||||||||||||||||||||||||||||||
| CleanEx | MM_PRNP. | ||||||||||||||||||||||||||||||||||||
| GermOnline | ENSMUSG00000027338. Mus musculus. | ||||||||||||||||||||||||||||||||||||
Family and domain databases | |||||||||||||||||||||||||||||||||||||
| InterPro | IPR000817. Prion. [Graphical view] | ||||||||||||||||||||||||||||||||||||
| Gene3D | G3DSA:1.10.790.10. Prion. 1 hit. | ||||||||||||||||||||||||||||||||||||
| PANTHER | PTHR11522. Prion. 1 hit. | ||||||||||||||||||||||||||||||||||||
| Pfam | PF00377. Prion. 1 hit. PF03991. Prion_octapep. 5 hits. [Graphical view] | ||||||||||||||||||||||||||||||||||||
| PRINTS | PR00341. PRION. | ||||||||||||||||||||||||||||||||||||
| SMART | SM00157. PRP. 1 hit. [Graphical view] | ||||||||||||||||||||||||||||||||||||
| PROSITE | PS00291. PRION_1. 1 hit. PS00706. PRION_2. 1 hit. [Graphical view] | ||||||||||||||||||||||||||||||||||||
| ProtoNet | Search... | ||||||||||||||||||||||||||||||||||||
Other Resources | |||||||||||||||||||||||||||||||||||||
| NextBio | 295714. | ||||||||||||||||||||||||||||||||||||
| PMAP-CutDB | P04925. | ||||||||||||||||||||||||||||||||||||
| SOURCE | Search... | ||||||||||||||||||||||||||||||||||||
Entry information
| Entry name | PRIO_MOUSE | ||||||||
| Accession | Primary (citable) accession number: P04925 | ||||||||
| Entry history |
| ||||||||
| Entry status | Reviewed (UniProtKB/Swiss-Prot) | ||||||||
| Annotation project | HPI (Human Proteome Initiative) | ||||||||
Relevant documents
| MGD cross-references Mouse Genome Database (MGD) 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 |

Clusters with


