Detail Information for IndEnz0002013331
IED ID IndEnz0002013331
Enzyme Type ID protease013331
Protein Name von Hippel-Lindau disease tumor suppressor
Protein G7
pVHL
Gene Name VHL
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 MPRRAENWDEAEVGAEEAGVEEYGPEEDGGEESGAEESGPEESGPEELGAEEEMEAGRPRPVLRSVNSREPSQVIFCNRSPRVVLPVWLNFDGEPQPYPTLPPGTGRRIHSYRGHLWLFRDAGTHDGLLVNQTELFVPSLNVDGQPIFANITLPVYTLKERCLQVVRSLVKPENYRRLDIVRSLYEDLEDHPNVQKDLERLTQERIAHQRMGD
Enzyme Length 213
Uniprot Accession Number P40337
Absorption
Active Site
Activity Regulation
Binding Site
Calcium Binding
catalytic Activity
DNA Binding
EC Number
Enzyme Function FUNCTION: Involved in the ubiquitination and subsequent proteasomal degradation via the von Hippel-Lindau ubiquitination complex. Seems to act as a target recruitment subunit in the E3 ubiquitin ligase complex and recruits hydroxylated hypoxia-inducible factor (HIF) under normoxic conditions. Involved in transcriptional repression through interaction with HIF1A, HIF1AN and histone deacetylases. Ubiquitinates, in an oxygen-responsive manner, ADRB2. {ECO:0000269|PubMed:10944113, ECO:0000269|PubMed:17981124, ECO:0000269|PubMed:19584355}.
Temperature Dependency
PH Dependency
Pathway PATHWAY: Protein modification; protein ubiquitination.
nucleotide Binding
Features Alternative sequence (2); Beta strand (11); Chain (1); Compositional bias (1); Helix (5); Mutagenesis (1); Natural variant (133); Region (4); Repeat (8); Turn (1)
Keywords 3D-structure;Alternative initiation;Alternative splicing;Congenital erythrocytosis;Cytoplasm;Disease variant;Membrane;Nucleus;Reference proteome;Repeat;Tumor suppressor;Ubl conjugation pathway
Interact With Q13617; P02751; Q9UM11; Q16665; P14866; Q99750; Q05513; Q99873; P63244; Q8WU17; P21980; Q61221; P0C6X7; Q9UM11; P08151; Q8WXK3; Q9Y575-3; Q9H765; Q99700-5; O14867; Q8TAB7; Q13939; P24863; Q8TBB7; Q5TAQ9-2; Q92466; Q9NX09; G5E9A7; Q01658; Q9NZJ0; O75530-2; Q9UI10; Q8IYF1; Q9H2C0; P62879; Q9Y5Q9; O75031; P42858; Q8WXH2; Q9BVA0; O60333-2; Q9NR64; Q53G59; Q6TDP4; Q8NBE8; O60662; Q8IXQ5-4; Q08380; Q99750; Q96BF6; P35240-4; O00746; C9J082; Q96FW1; Q9NV79; Q8WWQ0; Q8TCD6; O60260-5; O14744; Q8TAS3; Q9Y3C5; Q15393; Q8IYM2; P37840; O14508; Q7Z699; Q96A44; Q13148; O43493-5; Q8NFB2; P61086; Q9C0C9; Q16763; Q5VVX9-2; Q8N0U8; Q9GZL7; Q9GZS3; Q8N5D0-4; Q9Y6I7; Q8NCP5-3; Q6ZSB9; Q15916; P52739-2; Q96BH6; O75912; Q9UM11; P08151; Q15311
Induction
Subcellular Location SUBCELLULAR LOCATION: [Isoform 1]: Cytoplasm. Membrane; Peripheral membrane protein. Nucleus. Note=Found predominantly in the cytoplasm and with less amounts nuclear or membrane-associated. Colocalizes with ADRB2 at the cell membrane.; SUBCELLULAR LOCATION: [Isoform 3]: Cytoplasm. Nucleus. Note=Equally distributed between the nucleus and the cytoplasm but not membrane-associated.
Modified Residue
Post Translational Modification
Signal Peptide
Structure 3D Electron microscopy (2); X-ray crystallography (59)
Cross Reference PDB 1LM8; 1LQB; 1VCB; 3ZRC; 3ZRF; 3ZTC; 3ZTD; 3ZUN; 4AJY; 4AWJ; 4B95; 4B9K; 4BKS; 4BKT; 4W9C; 4W9D; 4W9E; 4W9F; 4W9G; 4W9H; 4W9I; 4W9J; 4W9K; 4W9L; 4WQO; 5LLI; 5N4W; 5NVV; 5NVW; 5NVX; 5NVY; 5NVZ; 5NW0; 5NW1; 5NW2; 5T35; 6BVB; 6FMI; 6FMJ; 6FMK; 6GFX; 6GFY; 6GFZ; 6GMN; 6GMQ; 6GMR; 6GMX; 6HAX; 6HAY; 6HR2; 6I7Q; 6I7R; 6R6H; 6R7F; 6SIS; 6ZHC; 7CJB; 7JTO; 7JTP; 7KHH; 7PI4;
Mapped Pubmed ID 10535940; 10823831; 10878807; 10973499; 11024059; 11292861; 11292862; 11358837; 11454738; 11504942; 11598268; 11749004; 11840338; 11865075; 11908068; 11921283; 11961546; 11987242; 11990703; 12016154; 12036906; 12048197; 12086860; 12097293; 12101228; 12114475; 12114495; 12209156; 12351678; 12374282; 12378530; 12414898; 12460920; 12468553; 12482756; 12504025; 12504026; 12510195; 12511881; 12538644; 12609565; 12609982; 12615973; 12640117; 12646216; 12673678; 12682018; 12682336; 12684640; 12692265; 12697815; 12743597; 12767066; 12781449; 12788921; 12810083; 12821933; 12839965; 12912907; 12912922; 12937142; 13679920; 14506252; 14517280; 14531799; 14556007; 14604959; 14636579; 14722919; 14726398; 14767570; 14963040; 14985465; 15122713; 15162797; 15231748; 15247232; 15300849; 15448019; 15467305; 15474027; 15530850; 15537541; 15601820; 15604095; 15607616; 15608669; 15611064; 15688063; 15696489; 15721254; 15750626; 15777842; 15799966; 15805242; 15809750; 15824735; 15849821; 15893810; 15921386; 15932632; 15962286; 15985433; 15998523; 16107702; 16142346; 16144691; 16170373; 16208141; 16210343; 16261165; 16278378; 16314641; 16401342; 16407835; 16506218; 16511565; 16669786; 16678111; 16707008; 16738327; 16768548; 16769276; 16847331; 16856496; 16884327; 16892044; 16943200; 16951198; 16954532; 16983094; 17006605; 17060462; 17069461; 17101696; 17102080; 17132228; 17183367; 17200123; 17245122; 17255293; 17264095; 17296901; 17350623; 17353276; 17353931; 17407064; 17434750; 17438007; 17450132; 17486080; 17497243; 17526729; 17555795; 17568584; 17639058; 17661816; 17680521; 17688370; 17696210; 17786294; 17825299; 17846357; 17898043; 17906660; 17912253; 17919893; 17936701; 17954272; 17967880; 17986458; 17997830; 17998064; 18072267; 18073334; 18079682; 18085246; 18093314; 18094722; 18162774; 18184961; 18212329; 18223282; 18245539; 18323857; 18337469; 18359287; 18389622; 18408496; 18416845; 18423895; 18424433; 18426857; 18446368; 18474617; 18517280; 18551016; 18559510; 18607865; 18635227; 18676741; 18685280; 18694926; 18751708; 18751980; 18775313; 18780286; 18805092; 18806787; 18836774; 18851830; 18926707; 18950731; 18985005; 18998488; 19018165; 19019082; 19028597; 19029228; 19030229; 19056930; 19061835; 19062180; 19064569; 19073886; 19133167; 19145771; 19158274; 19159641; 19162062; 19208735; 19215943; 19216840; 19223516; 19228690; 19238077; 19252526; 19258401; 19280651; 19288063; 19305426; 19336759; 19340311; 19343621; 19351817; 19399650; 19401348; 19408298; 19411751; 19419968; 19458911; 19464057; 19464396; 19489725; 19490898; 19525194; 19574279; 19602254; 19620968; 19629420; 19649731; 19690016; 19748355; 19759417; 19764026; 19801654; 19806577; 19808854; 19825962; 19874575; 19906784; 19913121; 19915015; 19915370; 19933033; 19940550; 20068166; 20137853; 20145706; 20185829; 20205103; 20208144; 20300531; 20375333; 20382342; 20382408; 20431476; 20447124; 20453000; 20560986; 20567917; 20599784; 20628086; 20637892; 20651059; 20680678; 20711500; 20802534; 20844582; 20855504; 20863722; 20871634; 20952286; 20964835; 20973793; 20978146; 20978319; 20980436; 21042786; 21151099; 21204227; 21212839; 21233420; 21258414; 21299348; 21335549; 21358672; 21362373; 21386872; 21415706; 21436690; 21463266; 21463634; 21493813; 21528828; 21547579; 21556796; 21602888; 21602890; 21625219; 21632985; 21642472; 21685785; 21715564; 21725364; 21763962; 21778237; 21778301; 21791076; 21798997; 21863050; 21876117; 21888897; 21942715; 21949687; 21962529; 21972040; 21993671; 22020339; 22022277; 22071692; 22084938; 22086907; 22105711; 22150821; 22156657; 22234250; 22245496; 22270996; 22284679; 22286234; 22351759; 22357542; 22369643; 22389506; 22393103; 22438210; 22462637; 22466964; 22516261; 22532874; 22537386; 22585604; 22642103; 22649212; 22673518; 22673568; 22709582; 22714833; 22799452; 22806541; 22825683; 22906772; 22946750; 22959436; 23011899; 23063455; 23065727; 23074928; 23079206; 23102223; 23151618; 23159849; 23203444; 23217559; 23224817; 23255108; 23298237; 23315288; 23318261; 23338840; 23387829; 23393199; 23395608; 23403324; 23407919; 23434161; 23453757; 23455319; 23455924; 23473032; 23481210; 23535662; 23535663; 23563313; 23583660; 23601303; 23611775; 23612971; 23626751; 23673869; 23730213; 23785518; 23840444; 23842656; 23872148; 23881929; 23902947; 23961993; 23990666; 24002598; 24029645; 24067370; 24112038; 24145166; 24149047; 24149212; 24166983; 24288256; 24335534; 24359042; 24394472; 24422631; 24436777; 24446253; 24469044; 24549640; 24550497; 24581539; 24583008; 24650032; 24678776; 24704013; 24715760; 24727139; 24899725; 24915993; 24977658; 24998140; 25027579; 25036637; 25069792; 25078357; 25166285; 25217002; 25313256; 25338163; 25355947; 25416944; 25416956; 25451921; 25490036; 25500545; 25501229; 25531723; 25557216; 25589003; 25661653; 25683602; 25732088; 25749518; 25750939; 25773797; 25890500; 25915846; 25966224; 25987131; 26179906; 26191297; 26211615; 26215701; 26224408; 26296657; 26304926; 26308528; 26496610; 26500060; 26506913; 26572705; 26592496; 26620126; 26632597; 26647960; 26707870; 26735018; 26735336; 26751167; 26841847; 26895810; 26973240; 26973243; 27029034; 27034144; 27090638; 27179072; 27196060; 27222660; 27323688; 27329246; 27439424; 27460078; 27473082; 27491826; 27511743; 27517496; 27527340; 27530247; 27563096; 27617348; 27623354; 27693634; 27777301; 27811928; 27836247; 27841867; 27902314; 27902963; 27940592; 27997886; 28036268; 28052007; 28089774; 28089820; 28103578; 28112200; 28114281; 28288108; 28329682; 28425505; 28432847; 28534376; 28549422; 28578000; 28591624; 28643803; 28666999; 28701475; 28710314; 28710479; 28853079; 28853884; 28893800; 28952225; 28952293; 29033184; 29053101; 29068413; 29075773; 29169846; 29172931; 29340905; 29373688; 29425832; 29463811; 29525880; 29595810; 29650462; 29749453; 29813026; 29845253; 29891534; 29949369; 30006056; 30006568; 30040896; 30053437; 30135421; 30355451; 30477447; 30512164; 30514329; 30553971; 30602302; 30635121; 30655088; 30902965; 30943211; 31178587; 31368132; 31436504; 31444342; 31575731; 31625068; 31690629; 31746102; 31773698; 31810986; 31823746; 31924838; 31980715; 31996412; 31999483; 32023483; 32075951; 32106822; 32161311; 32179488; 32209418; 32291411; 32323143; 32325871; 32396860; 32513235; 32535973; 32537867; 32603638; 32640193; 32697072; 32739800; 32756923; 32817424; 32826868; 32912113; 32924118; 33004005; 33121461; 33137104; 33151962; 33400184; 33488018; 33596073; 33774214; 33799686; 33823241; 33828526; 33851686; 34009695; 34080067; 34135317; 34290272; 34301760; 34416073; 34459035; 34475049; 34477178; 34537235; 34563045; 34575959; 34586829; 34719159; 34860252; 7923076; 8758207; 8863170; 9137812; 9398721; 9497878; 9891082;
Motif
Gene Encoded By
Mass 24,153
Kinetics
Metal Binding
Rhea ID
Cross Reference Brenda 2.3.2.B13;