ID   A2A2V1_HUMAN            Unreviewed;       249 AA.
AC   A2A2V1;
DT   20-FEB-2007, integrated into UniProtKB/TrEMBL.
DT   20-FEB-2007, sequence version 1.
DT   16-JAN-2019, entry version 93.
DE   RecName: Full=Major prion protein {ECO:0000256|RuleBase:RU003882};
DE   Flags: Fragment;
GN   Name=PRNP {ECO:0000313|Ensembl:ENSP00000411599};
OS   Homo sapiens (Human).
OC   Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi;
OC   Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini;
OC   Catarrhini; Hominidae; Homo.
OX   NCBI_TaxID=9606 {ECO:0000313|Ensembl:ENSP00000411599, ECO:0000313|Proteomes:UP000005640};
RN   [1] {ECO:0000313|Ensembl:ENSP00000411599, ECO:0000313|Proteomes:UP000005640}
RP   NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
RX   PubMed=11780052; DOI=10.1038/414865a;
RA   Deloukas P., Matthews L.H., Ashurst J.L., Burton J., Gilbert J.G.R.,
RA   Jones M., Stavrides G., Almeida J.P., Babbage A.K., Bagguley C.L.,
RA   Bailey J., Barlow K.F., Bates K.N., Beard L.M., Beare D.M.,
RA   Beasley O.P., Bird C.P., Blakey S.E., Bridgeman A.M., Brown A.J.,
RA   Buck D., Burrill W.D., Butler A.P., Carder C., Carter N.P.,
RA   Chapman J.C., Clamp M., Clark G., Clark L.N., Clark S.Y., Clee C.M.,
RA   Clegg S., Cobley V.E., Collier R.E., Connor R.E., Corby N.R.,
RA   Coulson A., Coville G.J., Deadman R., Dhami P.D., Dunn M.,
RA   Ellington A.G., Frankland J.A., Fraser A., French L., Garner P.,
RA   Grafham D.V., Griffiths C., Griffiths M.N.D., Gwilliam R., Hall R.E.,
RA   Hammond S., Harley J.L., Heath P.D., Ho S., Holden J.L., Howden P.J.,
RA   Huckle E., Hunt A.R., Hunt S.E., Jekosch K., Johnson C.M., Johnson D.,
RA   Kay M.P., Kimberley A.M., King A., Knights A., Laird G.K., Lawlor S.,
RA   Lehvaeslaiho M.H., Leversha M.A., Lloyd C., Lloyd D.M., Lovell J.D.,
RA   Marsh V.L., Martin S.L., McConnachie L.J., McLay K., McMurray A.A.,
RA   Milne S.A., Mistry D., Moore M.J.F., Mullikin J.C., Nickerson T.,
RA   Oliver K., Parker A., Patel R., Pearce T.A.V., Peck A.I.,
RA   Phillimore B.J.C.T., Prathalingam S.R., Plumb R.W., Ramsay H.,
RA   Rice C.M., Ross M.T., Scott C.E., Sehra H.K., Shownkeen R., Sims S.,
RA   Skuce C.D., Smith M.L., Soderlund C., Steward C.A., Sulston J.E.,
RA   Swann R.M., Sycamore N., Taylor R., Tee L., Thomas D.W., Thorpe A.,
RA   Tracey A., Tromans A.C., Vaudin M., Wall M., Wallis J.M.,
RA   Whitehead S.L., Whittaker P., Willey D.L., Williams L., Williams S.A.,
RA   Wilming L., Wray P.W., Hubbard T., Durbin R.M., Bentley D.R., Beck S.,
RA   Rogers J.;
RT   "The DNA sequence and comparative analysis of human chromosome 20.";
RL   Nature 414:865-871(2001).
RN   [2] {ECO:0000313|Ensembl:ENSP00000411599}
RP   IDENTIFICATION.
RG   Ensembl;
RL   Submitted (AUG-2012) to UniProtKB.
CC   -!- SUBUNIT: Monomer and homodimer. Has a tendency to aggregate into
CC       amyloid fibrils containing a cross-beta spine, formed by a steric
CC       zipper of superposed beta-strands. Soluble oligomers may represent
CC       an intermediate stage on the path to fibril formation. Copper
CC       binding may promote oligomerization.
CC       {ECO:0000256|SAAS:SAAS00537365}.
CC   -!- SUBCELLULAR LOCATION: Cell membrane
CC       {ECO:0000256|SAAS:SAAS00541856}; Lipid-anchor, GPI-anchor
CC       {ECO:0000256|SAAS:SAAS00541856}.
CC   -!- SIMILARITY: Belongs to the prion family.
CC       {ECO:0000256|RuleBase:RU003882, ECO:0000256|SAAS:SAAS00541861}.
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DR   EMBL; AL133396; -; NOT_ANNOTATED_CDS; Genomic_DNA.
DR   ProteinModelPortal; A2A2V1; -.
DR   SMR; A2A2V1; -.
DR   EPD; A2A2V1; -.
DR   jPOST; A2A2V1; -.
DR   MaxQB; A2A2V1; -.
DR   PeptideAtlas; A2A2V1; -.
DR   PRIDE; A2A2V1; -.
DR   Ensembl; ENST00000424424; ENSP00000411599; ENSG00000171867.
DR   UCSC; uc061vez.1; human.
DR   EuPathDB; HostDB:ENSG00000171867.16; -.
DR   HGNC; HGNC:9449; PRNP.
DR   OpenTargets; ENSG00000171867; -.
DR   eggNOG; ENOG410IJMM; Eukaryota.
DR   eggNOG; ENOG410YXUU; LUCA.
DR   GeneTree; ENSGT00510000049083; -.
DR   ChiTaRS; PRNP; human.
DR   Proteomes; UP000005640; Chromosome 20.
DR   Bgee; ENSG00000171867; Expressed in 242 organ(s), highest expression level in Brodmann (1909) area 23.
DR   ExpressionAtlas; A2A2V1; baseline and differential.
DR   GO; GO:0005829; C:cytosol; IDA:HPA.
DR   GO; GO:0043231; C:intracellular membrane-bounded organelle; IDA:HPA.
DR   GO; GO:0031965; C:nuclear membrane; IDA:HPA.
DR   GO; GO:0005886; C:plasma membrane; IEA:UniProtKB-SubCell.
DR   GO; GO:0046872; F:metal ion binding; IEA:UniProtKB-KW.
DR   GO; GO:0051260; P:protein homooligomerization; IEA:InterPro.
DR   Gene3D; 1.10.790.10; -; 1.
DR   InterPro; IPR000817; Prion.
DR   InterPro; IPR036924; Prion/Doppel_b-ribbon_dom_sf.
DR   InterPro; IPR022416; Prion/Doppel_prot_b-ribbon_dom.
DR   InterPro; IPR025860; Prion_N_dom.
DR   PANTHER; PTHR10502:SF134; PTHR10502:SF134; 1.
DR   Pfam; PF00377; Prion; 1.
DR   Pfam; PF11587; Prion_bPrPp; 1.
DR   PRINTS; PR00341; PRION.
DR   SMART; SM00157; PRP; 1.
DR   SUPFAM; SSF54098; SSF54098; 1.
DR   PROSITE; PS00291; PRION_1; 1.
DR   PROSITE; PS00706; PRION_2; 1.
PE   1: Evidence at protein level;
KW   Amyloid {ECO:0000256|RuleBase:RU003882};
KW   Cell membrane {ECO:0000256|RuleBase:RU003882,
KW   ECO:0000256|SAAS:SAAS00017656};
KW   Complete proteome {ECO:0000313|Proteomes:UP000005640};
KW   Copper {ECO:0000256|SAAS:SAAS00944119};
KW   Disulfide bond {ECO:0000256|SAAS:SAAS00017659};
KW   Membrane {ECO:0000256|RuleBase:RU003882,
KW   ECO:0000256|SAAS:SAAS00017656};
KW   Metal-binding {ECO:0000256|SAAS:SAAS00944118};
KW   Prion {ECO:0000256|RuleBase:RU003882};
KW   Proteomics identification {ECO:0000213|EPD:A2A2V1,
KW   ECO:0000213|MaxQB:A2A2V1, ECO:0000213|PeptideAtlas:A2A2V1};
KW   Reference proteome {ECO:0000313|Proteomes:UP000005640};
KW   Repeat {ECO:0000256|SAAS:SAAS00007072};
KW   Signal {ECO:0000256|SAM:SignalP}.
FT   SIGNAL        1     22       {ECO:0000256|SAM:SignalP}.
FT   CHAIN        23    249       Major prion protein.
FT                                {ECO:0000256|SAM:SignalP}.
FT                                /FTId=PRO_5002641817.
FT   DOMAIN      113    128       Prion/Doppel_prot_b-ribbon_dom.
FT                                {ECO:0000259|PROSITE:PS00291}.
FT   DOMAIN      200    218       Prion/Doppel_prot_b-ribbon_dom.
FT                                {ECO:0000259|PROSITE:PS00706}.
FT   NON_TER     249    249       {ECO:0000313|Ensembl:ENSP00000411599}.
SQ   SEQUENCE   249 AA;  27279 MW;  FAA66484345A5A87 CRC64;
     MANLGCWMLV LFVATWSDLG LCKKRPKPGG WNTGGSRYPG QGSPGGNRYP PQGGGGWGQP
     HGGGWGQPHG GGWGQPHGGG WGQPHGGGWG QGGGTHSQWN KPSKPKTNMK HMAGAAAAGA
     VVGGLGGYML GSAMSRPIIH FGSDYEDRYY RENMHRYPNQ VYYRPMDEYS NQNNFVHDCV
     NITIKQHTVT TTTKGENFTE TDVKMMERVV EQMCITQYER ESQAYYQRGS SMVLFSSPPV
     ILLISFLIF
//