| IED ID |
IndEnz0002012628 |
| Enzyme Type ID |
protease012628 |
| Protein Name |
von Willebrand factor
vWF
Cleaved into: von Willebrand antigen 2
von Willebrand antigen II
|
| Gene Name |
VWF F8VWF |
| 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 |
MIPARFAGVLLALALILPGTLCAEGTRGRSSTARCSLFGSDFVNTFDGSMYSFAGYCSYLLAGGCQKRSFSIIGDFQNGKRVSLSVYLGEFFDIHLFVNGTVTQGDQRVSMPYASKGLYLETEAGYYKLSGEAYGFVARIDGSGNFQVLLSDRYFNKTCGLCGNFNIFAEDDFMTQEGTLTSDPYDFANSWALSSGEQWCERASPPSSSCNISSGEMQKGLWEQCQLLKSTSVFARCHPLVDPEPFVALCEKTLCECAGGLECACPALLEYARTCAQEGMVLYGWTDHSACSPVCPAGMEYRQCVSPCARTCQSLHINEMCQERCVDGCSCPEGQLLDEGLCVESTECPCVHSGKRYPPGTSLSRDCNTCICRNSQWICSNEECPGECLVTGQSHFKSFDNRYFTFSGICQYLLARDCQDHSFSIVIETVQCADDRDAVCTRSVTVRLPGLHNSLVKLKHGAGVAMDGQDVQLPLLKGDLRIQHTVTASVRLSYGEDLQMDWDGRGRLLVKLSPVYAGKTCGLCGNYNGNQGDDFLTPSGLAEPRVEDFGNAWKLHGDCQDLQKQHSDPCALNPRMTRFSEEACAVLTSPTFEACHRAVSPLPYLRNCRYDVCSCSDGRECLCGALASYAAACAGRGVRVAWREPGRCELNCPKGQVYLQCGTPCNLTCRSLSYPDEECNEACLEGCFCPPGLYMDERGDCVPKAQCPCYYDGEIFQPEDIFSDHHTMCYCEDGFMHCTMSGVPGSLLPDAVLSSPLSHRSKRSLSCRPPMVKLVCPADNLRAEGLECTKTCQNYDLECMSMGCVSGCLCPPGMVRHENRCVALERCPCFHQGKEYAPGETVKIGCNTCVCQDRKWNCTDHVCDATCSTIGMAHYLTFDGLKYLFPGECQYVLVQDYCGSNPGTFRILVGNKGCSHPSVKCKKRVTILVEGGEIELFDGEVNVKRPMKDETHFEVVESGRYIILLLGKALSVVWDRHLSISVVLKQTYQEKVCGLCGNFDGIQNNDLTSSNLQVEEDPVDFGNSWKVSSQCADTRKVPLDSSPATCHNNIMKQTMVDSSCRILTSDVFQDCNKLVDPEPYLDVCIYDTCSCESIGDCACFCDTIAAYAHVCAQHGKVVTWRTATLCPQSCEERNLRENGYECEWRYNSCAPACQVTCQHPEPLACPVQCVEGCHAHCPPGKILDELLQTCVDPEDCPVCEVAGRRFASGKKVTLNPSDPEHCQICHCDVVNLTCEACQEPGGLVVPPTDAPVSPTTLYVEDISEPPLHDFYCSRLLDLVFLLDGSSRLSEAEFEVLKAFVVDMMERLRISQKWVRVAVVEYHDGSHAYIGLKDRKRPSELRRIASQVKYAGSQVASTSEVLKYTLFQIFSKIDRPEASRITLLLMASQEPQRMSRNFVRYVQGLKKKKVIVIPVGIGPHANLKQIRLIEKQAPENKAFVLSSVDELEQQRDEIVSYLCDLAPEAPPPTLPPDMAQVTVGPGLLGVSTLGPKRNSMVLDVAFVLEGSDKIGEADFNRSKEFMEEVIQRMDVGQDSIHVTVLQYSYMVTVEYPFSEAQSKGDILQRVREIRYQGGNRTNTGLALRYLSDHSFLVSQGDREQAPNLVYMVTGNPASDEIKRLPGDIQVVPIGVGPNANVQELERIGWPNAPILIQDFETLPREAPDLVLQRCCSGEGLQIPTLSPAPDCSQPLDVILLLDGSSSFPASYFDEMKSFAKAFISKANIGPRLTQVSVLQYGSITTIDVPWNVVPEKAHLLSLVDVMQREGGPSQIGDALGFAVRYLTSEMHGARPGASKAVVILVTDVSVDSVDAAADAARSNRVTVFPIGIGDRYDAAQLRILAGPAGDSNVVKLQRIEDLPTMVTLGNSFLHKLCSGFVRICMDEDGNEKRPGDVWTLPDQCHTVTCQPDGQTLLKSHRVNCDRGLRPSCPNSQSPVKVEETCGCRWTCPCVCTGSSTRHIVTFDGQNFKLTGSCSYVLFQNKEQDLEVILHNGACSPGARQGCMKSIEVKHSALSVELHSDMEVTVNGRLVSVPYVGGNMEVNVYGAIMHEVRFNHLGHIFTFTPQNNEFQLQLSPKTFASKTYGLCGICDENGANDFMLRDGTVTTDWKTLVQEWTVQRPGQTCQPILEEQCLVPDSSHCQVLLLPLFAECHKVLAPATFYAICQQDSCHQEQVCEVIASYAHLCRTNGVCVDWRTPDFCAMSCPPSLVYNHCEHGCPRHCDGNVSSCGDHPSEGCFCPPDKVMLEGSCVPEEACTQCIGEDGVQHQFLEAWVPDHQPCQICTCLSGRKVNCTTQPCPTAKAPTCGLCEVARLRQNADQCCPEYECVCDPVSCDLPPVPHCERGLQPTLTNPGECRPNFTCACRKEECKRVSPPSCPPHRLPTLRKTQCCDEYECACNCVNSTVSCPLGYLASTATNDCGCTTTTCLPDKVCVHRSTIYPVGQFWEEGCDVCTCTDMEDAVMGLRVAQCSQKPCEDSCRSGFTYVLHEGECCGRCLPSACEVVTGSPRGDSQSSWKSVGSQWASPENPCLINECVRVKEEVFIQQRNVSCPQLEVPVCPSGFQLSCKTSACCPSCRCERMEACMLNGTVIGPGKTVMIDVCTTCRCMVQVGVISGFKLECRKTTCNPCPLGYKEENNTGECCGRCLPTACTIQLRGGQIMTLKRDETLQDGCDTHFCKVNERGEYFWEKRVTGCPPFDEHKCLAEGGKIMKIPGTCCDTCEEPECNDITARLQYVKVGSCKSEVEVDIHYCQGKCASKAMYSIDINDVQDQCSCCSPTRTEPMQVALHCTNGSVVYHEVLNAMECKCSPRKCSK |
| Enzyme Length |
2813 |
| Uniprot Accession Number |
P04275 |
| Absorption |
|
| Active Site |
|
| Activity Regulation |
|
| Binding Site |
|
| Calcium Binding |
|
| catalytic Activity |
|
| DNA Binding |
|
| EC Number |
|
| Enzyme Function |
FUNCTION: Important in the maintenance of hemostasis, it promotes adhesion of platelets to the sites of vascular injury by forming a molecular bridge between sub-endothelial collagen matrix and platelet-surface receptor complex GPIb-IX-V. Also acts as a chaperone for coagulation factor VIII, delivering it to the site of injury, stabilizing its heterodimeric structure and protecting it from premature clearance from plasma. |
| Temperature Dependency |
|
| PH Dependency |
|
| Pathway |
|
| nucleotide Binding |
|
| Features |
Alternative sequence (3); Beta strand (73); Chain (2); Disulfide bond (34); Domain (15); Glycosylation (26); Helix (39); Motif (1); Mutagenesis (2); Natural variant (60); Region (4); Sequence caution (1); Sequence conflict (5); Signal peptide (1); Turn (10) |
| Keywords |
3D-structure;Alternative splicing;Blood coagulation;Cell adhesion;Cleavage on pair of basic residues;Direct protein sequencing;Disease variant;Disulfide bond;Extracellular matrix;Glycoprotein;Hemostasis;Reference proteome;Repeat;Secreted;Signal;von Willebrand disease |
| Interact With |
Q76LX8; P00451; P00451; P07359; Itself; Q6XD76; Q99944; Q6ZQX7-4; Q12888 |
| Induction |
|
| Subcellular Location |
SUBCELLULAR LOCATION: Secreted {ECO:0000269|PubMed:10961880}. Secreted, extracellular space, extracellular matrix {ECO:0000269|PubMed:10961880}. Note=Localized to storage granules. |
| Modified Residue |
|
| Post Translational Modification |
PTM: All cysteine residues are involved in intrachain or interchain disulfide bonds.; PTM: N- and O-glycosylated. {ECO:0000269|PubMed:14760718, ECO:0000269|PubMed:19139490, ECO:0000269|PubMed:19159218}. |
| Signal Peptide |
SIGNAL 1..22; /evidence=ECO:0000269|PubMed:3495266 |
| Structure 3D |
NMR spectroscopy (3); Electron microscopy (1); X-ray crystallography (30) |
| Cross Reference PDB |
1AO3;
1ATZ;
1AUQ;
1FE8;
1FNS;
1IJB;
1IJK;
1M10;
1OAK;
1SQ0;
1U0N;
1UEX;
2ADF;
2MHP;
2MHQ;
3GXB;
3HXO;
3HXQ;
3PPV;
3PPW;
3PPX;
3PPY;
3ZQK;
4C29;
4C2A;
4C2B;
4DMU;
4NT5;
5BV8;
6FWN;
6N29;
7EOW;
7F49;
7KWO;
|
| Mapped Pubmed ID |
10209122;
10361125;
10409742;
10446041;
10627461;
10725339;
10760079;
10777559;
10887121;
10891492;
10910910;
11017197;
11098050;
11236861;
11476890;
11529461;
11583318;
11700320;
11713259;
11722428;
11736957;
11755948;
11756664;
11776313;
11776314;
11843285;
11843287;
11843298;
11859851;
11864703;
11869299;
11877281;
11914659;
11920243;
11943773;
11992236;
11992244;
12005431;
12006654;
12036871;
12036879;
12068308;
12069583;
12070018;
12083486;
12091053;
12121649;
12163004;
12183630;
12215337;
12361948;
12362242;
12381897;
12393671;
12393698;
12393736;
12445472;
12447349;
12456504;
12473660;
12511565;
12543870;
12579041;
12582178;
12585966;
12624629;
12640026;
12646253;
12649144;
12668663;
12676191;
12729795;
12737944;
12775718;
12791651;
12792699;
12815111;
12832465;
12851390;
12851529;
12855811;
12860973;
12871466;
12883448;
12958606;
12969981;
14507115;
14512308;
14517489;
14525793;
14604963;
14630802;
14636584;
14691575;
14717782;
14717980;
14717981;
14727254;
14727255;
14754902;
14755371;
14970219;
14995987;
15005754;
15035987;
15039442;
15087444;
15102023;
15150074;
15157149;
15182579;
15203711;
15219197;
15226181;
15231748;
15249683;
15257287;
15322948;
15331450;
15377475;
15382239;
15459008;
15467896;
15471879;
15514009;
15520807;
15543157;
15630448;
15665869;
15705787;
15735796;
15748246;
15748247;
15764659;
15809291;
15816602;
15824096;
15831502;
15842373;
15842374;
15842375;
15849757;
15886321;
15886817;
15933060;
15946218;
15946221;
15975930;
16005629;
16040750;
16102036;
16102037;
16102041;
16115959;
16135787;
16141351;
16194200;
16203734;
16221672;
16247740;
16286459;
16314412;
16320153;
16321553;
16324758;
16364920;
16373331;
16418330;
16420575;
16424652;
16435187;
16449527;
16581250;
16634765;
16676067;
16735600;
16774914;
16805860;
16835381;
16839358;
16870550;
16879721;
16894469;
16899464;
16919435;
1694173;
16953269;
16953272;
16985174;
16990571;
17010412;
17052980;
17053057;
17090649;
17098004;
17100655;
17109387;
17119126;
17121983;
17137217;
17146059;
17159336;
17159339;
17190853;
1722201;
17275499;
17296575;
17371490;
17393012;
17393013;
17408405;
17408410;
17414220;
17446270;
17472573;
17490730;
17493665;
17496912;
17563371;
17598011;
17598021;
17603482;
17603483;
17619827;
17622488;
17650077;
17681836;
17721608;
17723135;
17764538;
17786534;
17849054;
17883593;
17890957;
17901248;
17908278;
17922807;
17949477;
17958741;
17975018;
17996280;
18025536;
18042361;
18045606;
18064343;
18094571;
18194418;
18263586;
18315546;
18315556;
18327408;
18344423;
18347221;
18388497;
18433456;
18433458;
18449422;
18485092;
18492805;
18503632;
1851341;
18515885;
18521502;
18521510;
18535282;
18549909;
18562239;
18577516;
18600088;
18612539;
18618671;
18647229;
18665926;
18690339;
18712522;
18724972;
18725999;
18752579;
18761720;
18771467;
18776767;
18809794;
18841300;
18848323;
18855616;
18923835;
18927433;
18945966;
18974108;
18981290;
18983499;
18983500;
18985028;
18996572;
19021456;
19029465;
19060241;
19091303;
19110358;
19175495;
19190805;
19190813;
19190814;
19192112;
19219045;
19240027;
19242111;
19277422;
19279408;
19286880;
19289057;
19298374;
19346881;
19350125;
19372260;
1939645;
19420105;
19422343;
19422452;
19422453;
19436051;
19470641;
19477487;
19496923;
19498171;
19506352;
19506353;
19506355;
19506356;
19506357;
19506358;
19506359;
19506360;
19506361;
19506362;
19541819;
19566550;
19570985;
19587373;
19608674;
19624459;
19630761;
19630816;
19647361;
19682236;
19687512;
19718479;
19726719;
19729451;
19730683;
19732980;
1973901;
19740526;
19765212;
19765213;
19773258;
19812385;
19817991;
19820200;
19839997;
19840363;
19874459;
19875727;
19897581;
19897584;
19913121;
19913482;
19933846;
19933990;
19943880;
19944670;
19948975;
19965639;
20021313;
20030949;
20032502;
20036902;
20052757;
20058209;
20060388;
20062916;
20063990;
20075158;
2010539;
20118404;
20130576;
20141835;
20142117;
20156642;
20179705;
20197612;
20201926;
20218000;
20231421;
20231535;
20303469;
20335223;
20346360;
20351307;
20354169;
20403097;
20424473;
20431063;
20452482;
20491956;
20492463;
20498367;
20505748;
20508517;
20526349;
20561182;
20565774;
20570857;
20586924;
20589313;
20589314;
20589320;
20605782;
20627205;
20628086;
20650506;
20651279;
20673868;
20675279;
20682599;
20695979;
20696945;
20701764;
20704649;
20705333;
20711500;
20723310;
20725043;
20729548;
20737318;
20806106;
20806123;
20851871;
20854374;
20858867;
20936779;
20940418;
20941784;
20946172;
20958794;
20979592;
21029358;
21037087;
21044367;
2104766;
21048155;
21078624;
21083900;
21094987;
2109644;
21136020;
21143374;
21150180;
21174003;
21266777;
21304226;
21342431;
21343614;
21346256;
21352469;
21362127;
21385852;
21393328;
21410641;
21424530;
21471547;
21474661;
21507943;
21512165;
21518247;
21592973;
21596755;
21605335;
21629201;
21638248;
21705658;
21715306;
21729570;
21750539;
21779388;
21779496;
21792464;
21810271;
21831097;
21839029;
21844207;
21847508;
21857647;
21896483;
21904649;
21909383;
21909423;
21911836;
21917758;
21937160;
21940821;
21988832;
22004444;
22032085;
22056526;
22070827;
22077817;
22096550;
22100896;
22110247;
22125593;
22163125;
22177953;
22178067;
22197721;
22207689;
22219226;
22244714;
22257027;
22267483;
22268915;
22282355;
22298244;
22357761;
22370688;
22429825;
22431572;
22431729;
22440751;
22452980;
22466813;
22479377;
22487084;
22490677;
22503077;
22507569;
22507806;
22510884;
22517896;
22532296;
22537243;
22545467;
22559004;
22568520;
22569528;
22593709;
22596213;
22612323;
22674667;
22688555;
22718838;
22740102;
22745804;
22781599;
22812646;
22826437;
22849435;
22854959;
22896002;
22922961;
22923007;
22952809;
22972845;
22982001;
23006049;
23020229;
23041092;
23104847;
23168412;
23179108;
23216583;
23266519;
23267103;
23311757;
23335371;
23349392;
23352452;
23354996;
23355534;
23358615;
23383177;
23407766;
23414517;
23426949;
23433535;
23481506;
23520336;
23529929;
23539537;
23583981;
23614898;
23621778;
23636243;
23640497;
23667175;
23682841;
23690449;
23702511;
23720451;
23737952;
23757730;
23775583;
23775931;
23812299;
23819767;
23834637;
23875798;
23902764;
23934108;
23991205;
24008159;
24010664;
24010820;
24014239;
24056772;
24075746;
24097977;
24106205;
24121058;
24135138;
24202393;
24211824;
24243921;
24270421;
24327155;
24338608;
24344794;
24345920;
24351199;
24357063;
24391089;
24394662;
24406064;
24415745;
24441931;
24449821;
24465435;
24550384;
24558203;
24626470;
24640675;
24651010;
24681085;
24700780;
24733925;
24740645;
24746704;
24754487;
24769235;
24790143;
24825900;
24937073;
24937142;
24951428;
24954072;
24957944;
25010251;
2502206;
2505252;
25103891;
25105915;
25113276;
25185554;
25195011;
25196897;
25201004;
25204415;
25230768;
25250329;
25274249;
25300810;
25300811;
25305202;
25313996;
25363217;
25381443;
25387000;
25390649;
25395424;
25397410;
25420483;
25425019;
25435214;
25439775;
25472874;
25498844;
25512528;
25605439;
25608503;
25612915;
25646961;
25650553;
25670067;
25673639;
25756206;
25779970;
25780857;
25782102;
25810255;
25846964;
25876231;
25886574;
25904363;
25907612;
25916953;
25973092;
25983111;
26019279;
26047609;
26088471;
26121272;
26168189;
26178390;
26223854;
26228839;
26245874;
26266817;
26307534;
26345337;
26366828;
26473492;
26566941;
26581637;
26589836;
26595154;
26600159;
26616301;
26635035;
26645283;
26670633;
26677223;
26744078;
26759371;
26771163;
26773038;
26784534;
26827609;
26871104;
26872106;
26962963;
26988807;
27034431;
27052664;
27060324;
27068535;
27069929;
27105433;
27106123;
27166361;
27207416;
27207787;
27207796;
27227380;
27236750;
27297464;
27317792;
27344059;
27353798;
27359253;
27412500;
27416003;
27434669;
27483487;
27485105;
27533707;
27536857;
27543438;
27554083;
27559008;
27562418;
27581227;
27584569;
27587878;
27598970;
27619398;
27669148;
27698012;
27717713;
27732771;
27734074;
27737864;
27746209;
27766025;
28004844;
28007459;
28035064;
28071872;
28087247;
28102428;
28134282;
28139439;
28139814;
28209710;
28234916;
28279966;
28294518;
28374895;
28403644;
28453889;
28457019;
28521259;
28533135;
28544236;
28570705;
28586149;
28591212;
28642239;
28652401;
28692141;
28726980;
28765701;
28775074;
28831047;
28832079;
28916584;
28924049;
28939558;
28952167;
29079767;
29097365;
29130325;
29251812;
29304532;
29367644;
29409455;
29467321;
29615758;
29636514;
29685722;
29872003;
29880488;
29887594;
29972862;
29980574;
29985231;
30034615;
30053340;
30111575;
30175488;
30279208;
30281535;
30305279;
30354363;
30471183;
30471507;
30542696;
30573509;
30605489;
30642920;
30645640;
30665023;
30722078;
30745808;
30770394;
30794926;
3082357;
30864273;
30896677;
30929293;
30936056;
31018945;
31040240;
31067148;
31261173;
31276978;
31322547;
3134349;
31498279;
3155520;
31557676;
31558672;
31588918;
31628947;
31758651;
31793247;
31860769;
31921147;
31990275;
31990334;
32011054;
32032127;
32065383;
32135566;
32188625;
32189311;
32196607;
32204913;
32234826;
32441353;
32518657;
32544272;
32614949;
32663297;
32663299;
32716345;
32722784;
32726854;
3287375;
32920806;
32932544;
32961556;
33096906;
33221577;
33230904;
33242156;
33278841;
33301925;
33343584;
33369998;
33403757;
33404693;
33449299;
33502080;
33539188;
33550613;
33550700;
33556167;
33556175;
33569592;
33606732;
33883551;
33893632;
33930144;
33974165;
34144500;
34231349;
34245915;
34494337;
34516971;
34662354;
34763490;
34882208;
34889809;
3872140;
6438527;
6457647;
7505120;
7513017;
7565670;
7565795;
7731720;
7756647;
7929275;
7935411;
7997267;
8034572;
8051079;
8052958;
8157000;
8467233;
8621540;
8631758;
8649368;
9020159;
9351824;
9425086;
9501911;
9566877;
9858547;
|
| Motif |
MOTIF 2507..2509; /note=Cell attachment site |
| Gene Encoded By |
|
| Mass |
309,265 |
| Kinetics |
|
| Metal Binding |
|
| Rhea ID |
|
| Cross Reference Brenda |
|