IED Industry Enzyme Database

Detail Information for IndEnz0002007982
IED ID IndEnz0002007982
Enzyme Type ID protease007982
Protein Name Major prion protein
PrP
ASCR
PrP27-30
PrP33-35C
CD antigen CD230
Gene Name PRNP ALTPRP PRIP PRP
Organism Homo sapiens (Human)
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 Primates Haplorrhini Simiiformes Catarrhini Hominoidea (apes) Hominidae (great apes) Homininae Homo Homo sapiens (Human)
Enzyme Sequence MANLGCWMLVLFVATWSDLGLCKKRPKPGGWNTGGSRYPGQGSPGGNRYPPQGGGGWGQPHGGGWGQPHGGGWGQPHGGGWGQPHGGGWGQGGGTHSQWNKPSKPKTNMKHMAGAAAAGAVVGGLGGYMLGSAMSRPIIHFGSDYEDRYYRENMHRYPNQVYYRPMDEYSNQNNFVHDCVNITIKQHTVTTTTKGENFTETDVKMMERVVEQMCITQYERESQAYYQRGSSMVLFSSPPVILLISFLIFLIVG
Enzyme Length 253
Uniprot Accession Number P04156
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 (By similarity). {ECO:0000250|UniProtKB:P04925, ECO:0000269|PubMed:12732622, ECO:0000269|PubMed:19936054, ECO:0000269|PubMed:20564047, ECO:0000305}.
Temperature Dependency
PH Dependency
Pathway
nucleotide Binding
Features Beta strand (12); Chain (1); Disulfide bond (1); Glycosylation (2); Helix (4); Lipidation (1); Metal binding (12); Natural variant (27); Propeptide (1); Region (4); Repeat (5); Sequence conflict (4); Signal peptide (1); Turn (6)
Keywords 3D-structure;Alternative initiation;Amyloid;Amyloidosis;Cell cycle;Cell membrane;Copper;Direct protein sequencing;Disease variant;Disulfide bond;GPI-anchor;Glycoprotein;Golgi apparatus;Growth arrest;Lipoprotein;Membrane;Metal-binding;Prion;Reference proteome;Repeat;Signal;Zinc
Interact With Q9UL18; Q9UKV8; P05067; P05067-4; P05067; Q8WXF7; P25311; P55085; Q13642; O75084; P49639; P42858; P10636; P29372; Q9BSJ6; Q9H4B4; Q06830; Itself; Q8N6K7-2; Q8CJG0; P04925; P10279; P23907; P31424-2; P52480
Induction
Subcellular Location SUBCELLULAR LOCATION: Cell membrane; Lipid-anchor, GPI-anchor {ECO:0000269|PubMed:19936054}. Golgi apparatus {ECO:0000250|UniProtKB:P04925}. 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:19936054}.
Modified Residue
Post Translational Modification PTM: The glycosylation pattern (the amount of mono-, di- and non-glycosylated forms or glycoforms) seems to differ in normal and CJD prion. {ECO:0000269|PubMed:12214108}.
Signal Peptide SIGNAL 1..22; /evidence=ECO:0000250|UniProtKB:P04925
Structure 3D NMR spectroscopy (33); Electron microscopy (3); X-ray crystallography (25)
Cross Reference PDB 1E1G; 1E1J; 1E1P; 1E1S; 1E1U; 1E1W; 1FKC; 1FO7; 1H0L; 1HJM; 1HJN; 1I4M; 1OEH; 1OEI; 1QLX; 1QLZ; 1QM0; 1QM1; 1QM2; 1QM3; 2IV4; 2IV5; 2IV6; 2K1D; 2KUN; 2LBG; 2LEJ; 2LFT; 2LSB; 2LV1; 2M8T; 2OL9; 2W9E; 3HAF; 3HAK; 3HEQ; 3HER; 3HES; 3HJ5; 3HJX; 3MD4; 3MD5; 3NHC; 3NHD; 3NVF; 4DGI; 4E1H; 4E1I; 4KML; 4N9O; 5L6R; 5YJ4; 5YJ5; 6DU9; 6LNI; 6PQ5; 6PQA; 6SUZ; 6SV2; 6UUR; 7DWV;
Mapped Pubmed ID 10953203; 11120925; 11220690; 11244488; 11559357; 11584448; 11593450; 11684342; 11704923; 11743735; 11756421; 11771989; 11775001; 11787070; 11792705; 11833672; 11840201; 11961239; 11986958; 12070046; 12084159; 12161431; 12186633; 12205650; 12356762; 12359724; 12392052; 12399017; 12451210; 12459456; 12464104; 12543108; 12547204; 12568340; 12590162; 12601712; 12659837; 12679875; 12682740; 12684540; 12692258; 12694397; 12719777; 12796830; 12805563; 12826672; 12867116; 12891686; 12917418; 12917444; 12946346; 12952977; 12970341; 14519851; 14576159; 14593432; 14610121; 14616310; 14668351; 14744790; 14761942; 14970845; 14983221; 15123682; 15131108; 15140132; 15148589; 15203115; 15208260; 15215178; 15247220; 15258222; 15266305; 15277640; 15304595; 15469448; 15488240; 15539564; 15555583; 15557533; 15583862; 15591591; 15609351; 15639746; 15684434; 15775715; 15837581; 15846375; 15850959; 15862295; 15946217; 15987701; 15997418; 16004966; 16009550; 16025285; 16051190; 16099550; 16099923; 16119432; 16120605; 16156720; 16169070; 16175355; 16187142; 16192387; 16215457; 16215462; 16217673; 16263114; 16286452; 16287045; 16294306; 16298483; 16314483; 16315279; 16324095; 16342955; 16432880; 16434486; 16443601; 16460908; 16478730; 16519692; 16533975; 16537913; 16543824; 16545382; 16547836; 16580884; 16582585; 16672189; 16674609; 16713569; 16750169; 16764594; 16824036; 16825951; 16825956; 16831968; 16847141; 16847689; 16858508; 16889908; 16897605; 16908519; 16914329; 16921242; 16925523; 16934224; 16950206; 16987816; 17023178; 17029361; 17047093; 17092648; 17121821; 17134829; 17149767; 17156017; 17192785; 17202849; 17242357; 17260961; 17276393; 17313881; 17334659; 17385076; 17409275; 17410475; 17414209; 17449139; 17472702; 17497959; 17500595; 17519231; 17539938; 17560545; 17570906; 17709704; 17827389; 17873292; 17965961; 17987393; 17996224; 18006836; 18025469; 18038270; 18188498; 18191917; 18204788; 18217885; 18218718; 18236005; 18236081; 18247504; 18275852; 18325785; 18332630; 18347820; 18372408; 18406463; 18413481; 18419754; 18423780; 18425766; 18436646; 18443555; 18445040; 18478114; 18482256; 18505059; 18508914; 18539633; 18549395; 18549399; 18563793; 18597782; 18619462; 18638557; 18665216; 18706660; 18720902; 18722532; 18786636; 18810471; 18830724; 18930924; 18955686; 18959744; 18990686; 19008948; 19010951; 19030774; 19035579; 19051123; 19056496; 19064990; 19074151; 19077115; 19081515; 19099191; 19129193; 19140013; 19158507; 19164910; 19172188; 19196429; 19204171; 19210573; 19212444; 19218199; 19226372; 19228673; 19249347; 19278656; 19321423; 19327369; 19351416; 19363267; 19369250; 19416900; 19476383; 19477226; 19495414; 19524515; 19534429; 19542614; 19556894; 19558790; 19583442; 19587281; 19597535; 19601795; 19602567; 19606064; 19607920; 19643043; 19646442; 19675240; 19690385; 19696976; 19697238; 19698114; 19709627; 19710507; 19725833; 19728151; 19734292; 19807656; 19822779; 19882604; 19889475; 19910028; 19911184; 19917818; 19996123; 20004419; 20005032; 20028338; 20035629; 20036811; 20036833; 20049591; 20077484; 20105449; 20109837; 20175205; 20195357; 20195363; 20198483; 20392961; 20413850; 20422111; 20453509; 20465257; 20473510; 20487506; 20506117; 20515742; 20515743; 20515747; 20526696; 20529115; 20541558; 20547212; 20547859; 20552563; 20562404; 20564346; 20574532; 20576610; 20583301; 20583902; 20592456; 20593190; 20613639; 20625387; 20650901; 20661422; 20676083; 20685658; 20694796; 20711061; 20718410; 20724601; 20799315; 20804519; 20816195; 20838930; 20861579; 20870462; 20880607; 20919751; 20930299; 20932979; 20949975; 20970434; 21029243; 21058033; 21062360; 21071944; 21094273; 21107135; 21107851; 21116546; 21119307; 21193246; 21209079; 21212268; 21232818; 21257747; 21265952; 21293298; 21296677; 21298055; 21301993; 21320996; 21323366; 21338080; 21356381; 21385869; 21416485; 21439722; 21451573; 21453198; 21478678; 21508834; 21552571; 21559407; 21593310; 21597335; 21600043; 21616973; 21631281; 21636303; 21654203; 21656680; 21689534; 21743439; 21743475; 21795680; 21799773; 21804240; 21827207; 21833705; 21839748; 21841253; 21857997; 21875156; 21900252; 21911696; 21920025; 21943430; 21957246; 21957261; 21980292; 21980981; 21983261; 22002245; 22028931; 22031292; 22032174; 22036844; 22102029; 22128151; 22129783; 22155634; 22184125; 22210626; 22252492; 22285492; 22318125; 22356913; 22363722; 22384235; 22411239; 22484317; 22505365; 22511770; 22558368; 22561193; 22584955; 22612156; 22615124; 22658899; 22669942; 22675855; 22676969; 22763467; 22788868; 22791135; 22796214; 22820080; 22842913; 22860629; 22861352; 22874673; 22876179; 22895088; 22895089; 22915585; 22918447; 22930754; 22936677; 22947063; 22967749; 22972305; 22978166; 22987042; 23022479; 23090399; 23115236; 23129660; 23131565; 23175838; 23225001; 23225009; 23225390; 23236467; 23246001; 23256626; 23276223; 23283514; 23319218; 23324596; 23386614; 23392670; 23399523; 23405858; 23406905; 23406923; 23436635; 23449776; 23467330; 23533635; 23565236; 23577068; 23614720; 23625312; 23627023; 23637596; 23638794; 23764834; 23785217; 23792955; 23808589; 23825952; 23843953; 23857314; 23857619; 23871665; 23907583; 23911565; 23959875; 23967259; 23974118; 24028865; 24047819; 24086135; 24091711; 24112521; 24121542; 24145555; 24224623; 24225951; 24329154; 24338015; 24398683; 24400836; 24493558; 24498083; 24519981; 24606939; 24620982; 24706505; 24714645; 24838726; 24857020; 24945274; 24958194; 24965601; 25027605; 25179605; 25220284; 25239885; 25279981; 25280631; 25281825; 25435015; 25450391; 25522698; 25572400; 25643046; 25922234; 25978088; 25983001; 25998112; 26022925; 26104335; 26107283; 26157118; 26159734; 26268049; 26268531; 26323476; 26368533; 26528810; 26667279; 26683373; 26710111; 26778001; 26836195; 26864450; 26946358; 27056979; 27057694; 27107654; 27112151; 27184108; 27193484; 27216988; 27310471; 27344333; 27535221; 27576687; 27606840; 27611644; 27639164; 27793473; 27803163; 27803245; 27818203; 27959938; 28004805; 28030950; 28102071; 28102851; 28104892; 28109886; 28205568; 28260678; 28271922; 28314738; 28341739; 28412969; 28415023; 28533442; 28692055; 28693923; 28795797; 28800967; 28838676; 28901450; 28931606; 29107182; 29127190; 29141869; 29382530; 29393338; 29460268; 29478593; 29569252; 29679649; 30181558; 30336980; 30359282; 30478887; 30569742; 30600179; 30606247; 30654447; 30826454; 30830863; 30922182; 30926338; 30938429; 31020572; 31020675; 31079927; 31161647; 31189938; 31340582; 31477838; 31511544; 31547531; 31583788; 31651418; 31713950; 31844149; 31870551; 31931179; 31989677; 31991323; 32001774; 32098855; 32284600; 32321762; 32458274; 32514176; 32560489; 32612191; 32683652; 32728168; 32875355; 32889654; 32988885; 33092220; 33092231; 33477465; 33542378; 33767435; 33896033; 33980968; 34064393; 34102212; 34246750; 34487324; 34516876; 34656326; 34663460; 34705895; 34782343; 34810220; 34831353; 34948096; 7823070; 8100163; 9270595;
Motif
Gene Encoded By
Mass 27,661
Kinetics
Metal Binding METAL 61; /note=Cu(2+) 1; /evidence=ECO:0000269|PubMed:11900542; METAL 62; /note=Cu(2+) 1; via amide nitrogen; /evidence=ECO:0000269|PubMed:11900542; METAL 63; /note=Cu(2+) 1; via amide nitrogen and carbonyl oxygen; /evidence=ECO:0000269|PubMed:11900542; METAL 69; /note=Cu(2+) 2; /evidence=ECO:0000305|PubMed:11900542; METAL 70; /note=Cu(2+) 2; via amide nitrogen; /evidence=ECO:0000305|PubMed:11900542; METAL 71; /note=Cu(2+) 2; via amide nitrogen and carbonyl oxygen; /evidence=ECO:0000305|PubMed:11900542; METAL 77; /note=Cu(2+) 3; /evidence=ECO:0000305|PubMed:11900542; METAL 78; /note=Cu(2+) 3; via amide nitrogen; /evidence=ECO:0000305|PubMed:11900542; METAL 79; /note=Cu(2+) 3; via amide nitrogen and carbonyl oxygen; /evidence=ECO:0000305|PubMed:11900542; METAL 85; /note=Cu(2+) 4; /evidence=ECO:0000305|PubMed:11900542; METAL 86; /note=Cu(2+) 4; via amide nitrogen; /evidence=ECO:0000305|PubMed:11900542; METAL 87; /note=Cu(2+) 4; via amide nitrogen and carbonyl oxygen; /evidence=ECO:0000305|PubMed:11900542
Rhea ID
Cross Reference Brenda