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; |