IED ID |
IndEnz0002008014 |
Enzyme Type ID |
protease008014 |
Protein Name |
Major prion protein
PrP
PrP27-30
PrP33-35C
CD antigen CD230
|
Gene Name |
Prnp Prn-p Prp |
Organism |
Mus musculus (Mouse) |
Taxonomic Lineage |
cellular organisms
Eukaryota
Opisthokonta
Metazoa
Eumetazoa
Bilateria
Deuterostomia
Chordata
Craniata
Vertebrata
Gnathostomata (jawed vertebrates)
Teleostomi
Euteleostomi
Sarcopterygii
Dipnotetrapodomorpha
Tetrapoda
Amniota
Mammalia
Theria
Eutheria
Boreoeutheria
Euarchontoglires
Glires (Rodents and rabbits)
Rodentia
Myomorpha (mice and others)
Muroidea
Muridae
Murinae
Mus
Mus
Mus musculus (Mouse)
|
Enzyme Sequence |
MANLGYWLLALFVTMWTDVGLCKKRPKPGGWNTGGSRYPGQGSPGGNRYPPQGGTWGQPHGGGWGQPHGGSWGQPHGGSWGQPHGGGWGQGGGTHNQWNKPSKPKTNLKHVAGAAAAGAVVGGLGGYMLGSAMSRPMIHFGNDWEDRYYRENMYRYPNQVYYRPVDQYSNQNNFVHDCVNITIKQHTVTTTTKGENFTETDVKMMERVVEQMCVTQYQKESQAYYDGRRSSSTVLFSSPPVILLISFLIFLIVG |
Enzyme Length |
254 |
Uniprot Accession Number |
P04925 |
Absorption |
|
Active Site |
|
Activity Regulation |
|
Binding Site |
|
Calcium Binding |
|
catalytic Activity |
|
DNA Binding |
|
EC Number |
|
Enzyme Function |
FUNCTION: 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 Cu(2+) or Zn(2+) for the ascorbate-mediated GPC1 deaminase degradation of its heparan sulfate side chains (PubMed:12732622, PubMed:16492732, PubMed:19242475, PubMed:19568430). {ECO:0000250|UniProtKB:P04156, ECO:0000269|PubMed:12732622, ECO:0000269|PubMed:16492732, ECO:0000269|PubMed:19242475, ECO:0000269|PubMed:19568430}. |
Temperature Dependency |
|
PH Dependency |
|
Pathway |
|
nucleotide Binding |
|
Features |
Beta strand (4); Chain (1); Disulfide bond (1); Glycosylation (2); Helix (6); Lipidation (1); Metal binding (12); Modified residue (1); Mutagenesis (2); Natural variant (2); Propeptide (1); Region (4); Repeat (5); Sequence conflict (2); Signal peptide (1); Turn (1) |
Keywords |
3D-structure;Amyloid;Cell membrane;Copper;Direct protein sequencing;Disulfide bond;GPI-anchor;Glycoprotein;Golgi apparatus;Hydroxylation;Lipoprotein;Membrane;Metal-binding;Prion;Reference proteome;Repeat;Signal;Zinc |
Interact With |
P12023; O08532; Q60631; Itself; P04156 |
Induction |
|
Subcellular Location |
SUBCELLULAR LOCATION: Cell membrane {ECO:0000269|PubMed:11571277, ECO:0000269|PubMed:9837873}; Lipid-anchor, GPI-anchor. Golgi apparatus {ECO:0000269|PubMed:11756421}. Note=Targeted to lipid rafts via association with the heparan sulfate chains of GPC1. Colocates, in the presence of Cu(2+), to. vesicles in para- and perinuclear regions, where both proteins undergo internalization. Heparin displaces PRNP from lipid rafts and promotes endocytosis. {ECO:0000269|PubMed:11571277, ECO:0000269|PubMed:12732622, ECO:0000269|PubMed:16923158, ECO:0000269|PubMed:9837873}. |
Modified Residue |
MOD_RES 44; /note=Hydroxyproline; /evidence=ECO:0000269|PubMed:11032800 |
Post Translational Modification |
PTM: N-glycosylated. {ECO:0000269|PubMed:16492732, ECO:0000269|PubMed:19349973, ECO:0000269|PubMed:19568430}. |
Signal Peptide |
SIGNAL 1..22; /evidence=ECO:0000269|PubMed:2894984 |
Structure 3D |
NMR spectroscopy (15); X-ray crystallography (9) |
Cross Reference PDB |
1AG2;
1XYX;
1Y15;
1Y16;
2K5O;
2KFM;
2KFO;
2KU5;
2KU6;
2L1D;
2L1E;
2L1H;
2L1K;
2L39;
2L40;
3NVG;
3NVH;
4H88;
4J8R;
4MA7;
4MA8;
6AQ7;
6HER;
6HHD;
|
Mapped Pubmed ID |
10037495;
10086355;
10102274;
10352232;
10378511;
10421360;
10461901;
10477271;
10521590;
10525406;
10529232;
10550328;
10581259;
10656806;
10708751;
10805813;
10842180;
10900000;
10930132;
10954563;
10985358;
10987828;
11038396;
11073804;
11130978;
11152514;
11152682;
11179214;
11208919;
11239719;
11259483;
11259607;
11263978;
11274428;
11278539;
11300723;
11300727;
11312611;
11333913;
11353827;
11359769;
11369504;
11546838;
11566872;
11697729;
11697909;
11716303;
11742063;
11743735;
11775003;
11782965;
11792852;
11823413;
11842265;
11846415;
11860274;
11867531;
11904434;
11912192;
11924867;
11950500;
12034448;
12051707;
12070160;
12084159;
12091459;
12093733;
12130636;
12163492;
12206674;
12215421;
12271119;
12379130;
12383347;
12386336;
12386337;
12387871;
12411613;
12425947;
12431994;
12435392;
12446843;
12456643;
12459456;
12481985;
12500977;
12519913;
12529324;
12556465;
12663673;
12664293;
12679564;
12684540;
12758067;
12759361;
12794132;
12805223;
12853456;
12871949;
12876477;
12878182;
12904479;
12911750;
12914944;
12927782;
12933795;
1353438;
1373228;
14511115;
14515989;
14525996;
14570892;
14573822;
14593181;
14597699;
14622086;
14625012;
14633630;
14645231;
14657385;
14668351;
14697265;
14706620;
14747574;
14752167;
14753456;
14999066;
15026410;
15050367;
15055597;
15082172;
15084583;
15090539;
15126640;
15133046;
15133048;
15146195;
15163699;
15170227;
15173173;
15176439;
15194501;
15208260;
15215178;
15229245;
15262264;
15262998;
15286374;
15301538;
15351724;
15354287;
15448157;
15451062;
15459186;
15467011;
15494440;
15509552;
15514463;
15528202;
15530652;
15539455;
15539564;
15542685;
15582152;
15596141;
15607951;
15615717;
15632154;
15647363;
15661974;
15670611;
15670743;
15671025;
15694261;
15706401;
15709746;
15753097;
15767405;
15800202;
15824101;
15827140;
15837581;
15846375;
15851519;
15862314;
15888663;
15890277;
15893468;
15911347;
15917101;
15917252;
15933194;
15936714;
15950943;
15951190;
15962001;
15970215;
16029203;
16084105;
16120605;
16135751;
16141072;
16157591;
16160162;
16177294;
16198494;
16219759;
16239348;
16253245;
16254249;
16258172;
16272297;
16278084;
16282497;
16294306;
16314415;
16339028;
16343423;
16386707;
16441239;
16467153;
16480953;
16517717;
16519879;
16537534;
16547418;
16554307;
16569635;
16574930;
16602821;
16621165;
16632309;
16635246;
1670551;
16707568;
1672162;
16730679;
16731968;
16738485;
16751368;
16766127;
1676894;
16787422;
1681406;
16825571;
16843609;
16844683;
1685143;
16866364;
1687379;
1687380;
1687381;
1687385;
16908519;
16912302;
16935263;
16940522;
16942751;
16945105;
16952355;
17056541;
17062642;
17084974;
17098996;
17106625;
17121821;
17131403;
17135402;
17144900;
17157195;
17165097;
17223707;
17241158;
17244617;
17245436;
17245437;
17251426;
17270731;
17292334;
17304577;
17320038;
17334982;
17337056;
17360589;
17379700;
17388948;
17405937;
17420473;
17433806;
17443816;
17446686;
17462540;
17468101;
17485546;
17494993;
17498663;
17504807;
17510630;
17531449;
17569776;
17570349;
17573247;
17573534;
17611634;
17612503;
17618696;
17626637;
17635996;
17697940;
17703189;
17709374;
17868659;
17876822;
17923484;
17925394;
17934213;
17936697;
17947689;
17964827;
17967411;
17981146;
17987132;
17989297;
17997980;
17999717;
18023289;
18042548;
18066056;
18068119;
18073517;
18088603;
18155836;
18175144;
18205409;
18206163;
18279314;
18316717;
18332141;
18338400;
18339329;
18343219;
18350378;
18358791;
18374587;
18374666;
18400886;
18416605;
18419754;
18431006;
18443219;
18462346;
18473442;
18490487;
18498440;
18514536;
18562311;
18566584;
18580573;
18590772;
18614678;
18628989;
18632556;
18638557;
18667494;
18674620;
18680312;
18687339;
1870200;
18706894;
18722532;
18773909;
18797300;
18804434;
18808201;
18809465;
18815152;
18826953;
18831069;
18834644;
18835352;
18840415;
18973680;
19000842;
19038218;
19046329;
19052217;
19053276;
19056496;
19064990;
19073920;
19074151;
19098904;
19100703;
19143847;
19164910;
19173221;
19204074;
19209230;
19223590;
19233277;
19243016;
19249347;
19283723;
19285121;
19286300;
19385058;
19393664;
19400950;
19457070;
19477226;
19479969;
19523487;
19524041;
19587281;
19597535;
19618915;
19655812;
19664595;
19666958;
19675240;
1968507;
19685161;
19700753;
1970329;
1970330;
19703931;
19709627;
19710140;
19720066;
19738901;
19747526;
19748890;
19760599;
19766283;
19766638;
19786843;
19805070;
19806224;
19823766;
19834742;
19843586;
19844576;
19846876;
19850936;
19854187;
19855845;
19888917;
19910576;
19940127;
20007469;
20040362;
20048341;
20075202;
20085368;
20086009;
20106973;
20110469;
20133875;
20138835;
20145049;
20161712;
20206606;
20219645;
20221436;
20238136;
20345906;
20356832;
20394048;
20395435;
20445063;
20460128;
20472884;
20541558;
20599662;
20610764;
20615969;
20626348;
20649983;
20660771;
20670217;
20679345;
20679477;
20703260;
20717837;
20808809;
20818492;
20876210;
20878768;
20930564;
20970434;
21041683;
21083616;
21123371;
21144827;
21156803;
21193014;
21195746;
21209925;
21212268;
21212283;
21214263;
21267068;
21277044;
21277354;
21323366;
21334441;
21335237;
21350487;
21357423;
21385869;
21406608;
21432853;
21439331;
21441896;
21441913;
21453198;
21466851;
21483752;
21555356;
21559407;
21586567;
21619885;
21641403;
21727894;
21743439;
21763679;
21766339;
21775587;
21777560;
21779169;
21795680;
21800884;
21835918;
21843375;
21847793;
21848803;
21858045;
21871438;
21900234;
21900252;
21909267;
21909425;
21912705;
21924230;
21931781;
21957246;
21957261;
21980292;
21980526;
21987789;
22025612;
22031932;
22038049;
22039779;
22043908;
22072688;
22072968;
22093825;
22100763;
22114562;
22144895;
22163178;
22184125;
22216002;
22222374;
22252492;
22285492;
22307640;
22331873;
22363722;
22398286;
22428057;
22447932;
22449963;
22493452;
22511770;
22529900;
22536327;
22537845;
22542184;
22586108;
22619325;
22648512;
22654104;
22679554;
22687393;
22705206;
22711839;
22743555;
22745483;
22762267;
22770220;
22772447;
22789566;
22820466;
22854022;
22860039;
22866057;
22889724;
22910907;
22912582;
22915801;
22922482;
22927985;
22930754;
22948149;
22956830;
22987112;
23002439;
23022479;
23061439;
23072804;
23121447;
23132868;
23133383;
23139828;
23145988;
23238082;
23256626;
23283973;
23290724;
23335053;
23345215;
23349890;
23386614;
23395905;
23407955;
23415788;
23481568;
23487467;
23580200;
23593266;
23629842;
23637596;
23650394;
23680490;
23729591;
23761404;
23764837;
23770331;
23787699;
23792955;
23805846;
23808898;
23811055;
23825952;
23831375;
23843953;
23851597;
23892077;
23903654;
23936187;
23940658;
23949728;
23959875;
23966386;
24000807;
24012003;
24012669;
24027305;
24038599;
24040215;
24086135;
24117858;
24133259;
24143866;
24145514;
24204258;
24225951;
24244171;
24247244;
24280226;
24307586;
24312586;
24329154;
24338008;
24352451;
24352465;
24373770;
24430187;
24431459;
24493590;
24497115;
24554723;
24574409;
24596090;
24604355;
24608126;
24631389;
24667414;
24685176;
24714645;
24752288;
24766164;
24810941;
24952961;
24965601;
25005024;
25034251;
25058617;
25139744;
25202268;
25242137;
25416551;
25426519;
25447819;
25490046;
25502554;
25510416;
25517431;
25539913;
25572394;
25584902;
25654651;
25658480;
25671600;
25673720;
25692227;
25695140;
25710374;
2575583;
25810062;
25810548;
25862412;
25972546;
26024859;
26152722;
26155834;
26159734;
26165121;
26175152;
26224882;
26250617;
26256540;
26287631;
26295309;
26298321;
26299705;
26423957;
26490404;
26500295;
26500296;
26553874;
26608316;
26627256;
26710111;
26713717;
26721882;
26857744;
26864450;
26873446;
26926995;
26949218;
27128441;
27208175;
27226369;
27227882;
27317661;
27325697;
27325698;
27327609;
27365298;
27440899;
27447728;
27463540;
27535221;
27550517;
27563063;
27591261;
27629560;
27629715;
27641601;
27689697;
27818203;
27849581;
27880767;
27891071;
27936017;
27973593;
27982059;
28178353;
28207746;
28527237;
28545141;
2859120;
28630311;
28669732;
28701513;
28736314;
28739602;
28760925;
28821618;
28827331;
2884041;
28842420;
28842494;
28851903;
28855681;
28874781;
28924012;
28945221;
29070671;
29220360;
29265373;
29337365;
29409004;
29569342;
29574582;
29577883;
29700113;
29700330;
29705776;
29748616;
29846863;
30054538;
30128651;
3015416;
30174115;
30275016;
30513342;
30729399;
30914754;
3094007;
30956132;
31041657;
31147460;
31161647;
31189938;
31291644;
3149717;
31511544;
31624071;
31666632;
31726043;
31815959;
31950499;
31985492;
31987501;
32012886;
32107004;
32165692;
32317725;
32394432;
32473880;
32561641;
32738196;
32829029;
32845931;
32861834;
32875355;
32895288;
32898162;
32959170;
32985542;
33137126;
33481951;
33547547;
33713980;
34502545;
34710372;
34810220;
34847154;
4970191;
6801762;
7494265;
7512619;
7553876;
7558020;
7622046;
7719349;
7842304;
7902565;
7904753;
7907357;
7911243;
7911437;
7912659;
7912827;
7913753;
7913762;
7962730;
7999308;
8030944;
8030958;
8095862;
8100741;
8118101;
8407244;
8462855;
8552188;
8602267;
8606772;
8617512;
8634020;
8635458;
8651915;
8698234;
8734907;
8757019;
8758005;
8790598;
8845956;
8917559;
8944151;
8982352;
9016962;
9030645;
9031631;
9163538;
9186056;
9207082;
9212101;
9242048;
9294164;
9294167;
9321385;
9333232;
9353594;
9363892;
9371744;
9414161;
9450236;
9462739;
9523587;
9568713;
9582258;
9590434;
9636277;
9751723;
9754876;
9770528;
9792790;
9867802;
9876879;
9883727;
9932423;
|
Motif |
|
Gene Encoded By |
|
Mass |
27,977 |
Kinetics |
|
Metal Binding |
METAL 60; /note=Cu(2+) 1; /evidence=ECO:0000250|UniProtKB:P04156; METAL 61; /note=Cu(2+) 1; via amide nitrogen; /evidence=ECO:0000250|UniProtKB:P04156; METAL 62; /note=Cu(2+) 1; via amide nitrogen and carbonyl oxygen; /evidence=ECO:0000250|UniProtKB:P04156; METAL 68; /note=Cu(2+) 2; /evidence=ECO:0000250|UniProtKB:P04156; METAL 69; /note=Cu(2+) 2; via amide nitrogen; /evidence=ECO:0000250|UniProtKB:P04156; METAL 70; /note=Cu(2+) 2; via amide nitrogen and carbonyl oxygen; /evidence=ECO:0000250|UniProtKB:P04156; METAL 76; /note=Cu(2+) 3; /evidence=ECO:0000250|UniProtKB:P04156; METAL 77; /note=Cu(2+) 3; via amide nitrogen; /evidence=ECO:0000250|UniProtKB:P04156; METAL 78; /note=Cu(2+) 3; via amide nitrogen and carbonyl oxygen; /evidence=ECO:0000250|UniProtKB:P04156; METAL 84; /note=Cu(2+) 4; /evidence=ECO:0000250|UniProtKB:P04156; METAL 85; /note=Cu(2+) 4; via amide nitrogen; /evidence=ECO:0000250|UniProtKB:P04156; METAL 86; /note=Cu(2+) 4; via amide nitrogen and carbonyl oxygen; /evidence=ECO:0000250|UniProtKB:P04156 |
Rhea ID |
|
Cross Reference Brenda |
|