KEGG ENZYME: 3.4.24.87

ENZYME: 3.4.24.87

Entry

EC 3.4.24.87                Enzyme

Name

ADAMTS13 endopeptidase;
ADAMTS VWF cleaving metalloprotease;
ADAMTS-13;
ADAMTS13;
vWF-cleaving protease;
VWF-CP;
vWF-degrading protease;
Upshaw factor;
von Willebrand factor cleaving protease;
ADAMTS13 peptidase

Class

Hydrolases;
Acting on peptide bonds (peptidases);
Metalloendopeptidases

Reaction(IUBMB)

The enzyme cleaves the von Willebrand factor at bond Tyr842!Met843 within the A2 domain

Comment

In peptidase family M12.

History

EC 3.4.24.87 created 2009

Orthology

K08627

a disintegrin and metalloproteinase with thrombospondin motifs 13

Genes

HSA:	11093(ADAMTS13)

PTR:	473153(ADAMTS13)

PPS:	100978943(ADAMTS13)

GGO:	101151958(ADAMTS13)

PON:	100459418(ADAMTS13)

PPYG:

129044125(ADAMTS13)

NLE:	100580216(ADAMTS13)

HMH:	116475825(ADAMTS13)

SSYN:

129478722(ADAMTS13)

MCC:	722202(ADAMTS13)

MCF:	102145096(ADAMTS13)

MTHB:

126937135

MNI:	105471867(ADAMTS13)

CSAB:

103239684(ADAMTS13)

CATY:

105580513(ADAMTS13)

PANU:

101018650(ADAMTS13)

TGE:	112607583(ADAMTS13)

MLEU:

105528703(ADAMTS13)

RRO:	104669720(ADAMTS13)

RBB:	108513335(ADAMTS13)

TFN:	117099210(ADAMTS13)

PTEH:

111523982(ADAMTS13)

CANG:

105503983(ADAMTS13)

CJC:	100404947(ADAMTS13)

SBQ:	101038728(ADAMTS13)

CIMI:

108292312(ADAMTS13)

ANAN:

105712188(ADAMTS13)

CSYR:

103273391(ADAMTS13)

MMUR:

105880760(ADAMTS13)

LCAT:

123646680(ADAMTS13)

PCOQ:

105826699(ADAMTS13)

OGA:	100960489(ADAMTS13)

MMU:	279028(Adamts13)

MCAL:

110312498(Adamts13)

MPAH:

110317485(Adamts13)

RNO:	102554393(Adamts13)

MCOC:

116091905(Adamts13)

ANU:	117701923 117703988(Adamts13)

ASYL:

127685103(Adamts13)

MUN:	110557514(Adamts13)

CGE:	100770010(Adamts13)

MAUA:

101827371(Adamts13)

PROB:

127222654(Adamts13)

PLEU:

114684226(Adamts13)

MORG:

121462687(Adamts13)

MFOT:

126509437

AAMP:

119818562(Adamts13)

NGI:	103742812(Adamts13)

HGL:	101713585(Adamts13)

CPOC:

100727045(Adamts13)

CCAN:

109696127(Adamts13)

DORD:

105995613(Adamts13)

DSP:	122108880(Adamts13)

PLOP:

125353276(Adamts13)

NCAR:

124964314

MMMA:

107159168(Adamts13)

ITI:	101958991(Adamts13)

OPI:

101517758

TUP:	102474078(ADAMTS13)

GVR:	103608948(ADAMTS13)

CFA:	100630922(ADAMTS13)

CLUD:

112677392(ADAMTS13)

VVP:	112935883(ADAMTS13)

VLG:	121473072(ADAMTS13)

NPO:	129512693(ADAMTS13)

AML:	100481099(ADAMTS13)

UMR:	103679919(ADAMTS13)

UAH:	113266572(ADAMTS13)

UAR:	123775676(ADAMTS13)

ELK:

111145642

LLV:

125083853

MPUF:

101694125(ADAMTS13)

MNP:	132024277(ADAMTS13)

MLK:	131813263(ADAMTS13)

NVS:	122917096(ADAMTS13)

ORO:	101385635(ADAMTS13)

EJU:	114201001(ADAMTS13)

ZCA:	113934005(ADAMTS13)

MLX:	118018186(ADAMTS13)

NSU:	110581846(ADAMTS13)

LWW:	102729565(ADAMTS13)

FCA:	101086761(ADAMTS13)

PYU:	121021243(ADAMTS13)

PCOO:

112860307(ADAMTS13)

PBG:	122475156(ADAMTS13)

PVIV:

125150198(ADAMTS13)

LRUF:

124522418

LGF:	123592432(ADAMTS13)

AJU:

106988263

PTG:	102959927(ADAMTS13)

PPAD:

109258253(ADAMTS13)

PUC:

125923109

PLEZ:

122204474(ADAMTS13)

HHV:	120247311(ADAMTS13)

BTA:	532272(ADAMTS13)

BOM:	102287814(ADAMTS13)

BIU:	109565686(ADAMTS13)

BBUB:

102412616(ADAMTS13)

BBIS:

104987950(ADAMTS13)

CHX:	102174580(ADAMTS13)

OAS:	101112214(ADAMTS13)

BTAX:

128056485(ADAMTS13)

CSUM:

138088193(ADAMTS13)

ODA:	120852324(ADAMTS13)

CCAD:

122442334(ADAMTS13)

MREE:

136163897(ADAMTS13)

OVR:	110134151(ADAMTS13)

MBEZ:

129539798(ADAMTS13)

SSC:	100627950(ADAMTS13)

CFR:	102506864(ADAMTS13)

CBAI:

105068608(ADAMTS13)

CDK:	105106329(ADAMTS13)

VPC:	102538566(ADAMTS13)

BACU:

102999611(ADAMTS13)

BMUS:

118897540(ADAMTS13)

LVE:	103074204(ADAMTS13)

OOR:	101281877(ADAMTS13)

LALB:

132523221(ADAMTS13)

GMF:	115842929(ADAMTS13)

DLE:	111177028(ADAMTS13)

PCAD:

102996576(ADAMTS13)

PSIU:

116755106(ADAMTS13)

PPHC:

136125156(ADAMTS13)

NASI:

112411327(ADAMTS13)

ECB:	100069281(ADAMTS13)

EPZ:	103565346(ADAMTS13)

EAI:	106845293(ADAMTS13)

MYB:	102240335(ADAMTS13)

MYD:	102764800(ADAMTS13)

MMYO:

118666904(ADAMTS13)

MLF:	102443299(ADAMTS13)

MDT:	132212526(ADAMTS13)

MYUM:

139020967(ADAMTS13)

PKL:	118708873(ADAMTS13)

EFUS:

103296148(ADAMTS13)

MNA:	107524621(ADAMTS13)

DRO:	112306578(ADAMTS13)

SHON:

118980285(ADAMTS13)

AJM:	119036040(ADAMTS13)

PDIC:

114511173(ADAMTS13)

PHAS:

123823380(ADAMTS13)

MMF:	118629978(ADAMTS13)

PPAM:

129079464(ADAMTS13)

HAI:

109382381

RFQ:	117032429(ADAMTS13)

PALE:

102885523(ADAMTS13)

PGIG:

120599714(ADAMTS13)

PVP:	105298645(ADAMTS13)

RAY:	107514854(ADAMTS13)

MJV:	108398385(ADAMTS13)

TOD:	119230354(ADAMTS13)

SARA:

101548412(ADAMTS13)

SFUM:

130040677(ADAMTS13)

SETR:

126009354(ADAMTS13)

LAV:	100667919(ADAMTS13)

TMU:

101354225

ETF:	101657978(ADAMTS13)

DNM:	101433690(ADAMTS13)

MDO:	100018539(ADAMTS13)

GAS:	123235198(ADAMTS13)

SHR:	100924870(ADAMTS13)

AFZ:

127546843

PCW:	110205378(ADAMTS13)

TVP:	118844739(ADAMTS13)

PBRV:

138147022(ADAMTS13)

MLAO:

141505191(ADAMTS13)

OAA:	100077268(ADAMTS13)

TACU:

119926568(ADAMTS13)

GGA:	417152(ADAMTS13)

PCOC:

116234434(ADAMTS13)

MGP:	100544155(ADAMTS13)

CJO:	107321769(ADAMTS13)

TPAI:

128073573(ADAMTS13)

LMUT:

125702505(ADAMTS13)

NMEL:

110406845(ADAMTS13)

APLA:

101800615(ADAMTS13)

ACYG:

106044108(ADAMTS13)

CATA:

118252273(ADAMTS13)

AFUL:

116496934(ADAMTS13)

TGU:	100226372(ADAMTS13)

LSR:	110477621(ADAMTS13)

SCAN:

103819131(ADAMTS13)

PMOA:

120512419(ADAMTS13)

OTC:	121336974(ADAMTS13)

PRUF:

121362425(ADAMTS13)

ATRC:

139603238(ADAMTS13)

GFR:	102043124(ADAMTS13)

FAB:	101809354(ADAMTS13)

OMA:	130260252(ADAMTS13)

PHI:	102109550(ADAMTS13)

PMAJ:

107212053(ADAMTS13)

CCAE:

111937039(ADAMTS13)

CCW:	104688336(ADAMTS13)

CBRC:

103623248(ADAMTS13)

ACOE:

138119541(ADAMTS13)

ETL:	114059081(ADAMTS13)

ZAB:	102072739(ADAMTS13)

ZLE:	135454931(ADAMTS13)

ACHL:

103809287(ADAMTS13)

SVG:	106851369(ADAMTS13)

MMEA:

130576694(ADAMTS13)

HRT:	120761613(ADAMTS13)

SATI:

136369780(ADAMTS13)

CCIC:

134051848(ADAMTS13)

FPG:	101910275(ADAMTS13)

FCH:	102055781(ADAMTS13)

CCRI:

104163978(ADAMTS13)

NNT:	104406931(ADAMTS13)

SHAB:

115617127(ADAMTS13)

ACUN:

113486351(ADAMTS13)

TALA:

104363650(ADAMTS13)

ACHC:

115335017(ADAMTS13)

HALD:

104323021(ADAMTS13)

HLE:	104833867(ADAMTS13)

AGEN:

126051980

HHAR:

128154268(ADAMTS13)

GCL:

127023651

CSTI:

104561782(ADAMTS13)

LDI:	104354039(ADAMTS13)

MNB:	103774154(ADAMTS13)

DPUB:

104296479(ADAMTS13)

PPUS:

135190258(ADAMTS13)

AVIT:

104278033(ADAMTS13)

BRHI:

104489548(ADAMTS13)

EGZ:	104129330(ADAMTS13)

NNI:	104023191(ADAMTS13)

PCRI:

104028886(ADAMTS13)

PCAO:

104047343(ADAMTS13)

PADL:

103915348(ADAMTS13)

AFOR:

103905329(ADAMTS13)

FGA:	104075580(ADAMTS13)

GSTE:

104261947(ADAMTS13)

CBOY:

140661095(ADAMTS13)

CLV:	102096762(ADAMTS13)

CNIC:

135996448(ADAMTS13)

MUI:	104545065(ADAMTS13)

PGUU:

104469452(ADAMTS13)

PLET:

104624643(ADAMTS13)

EHS:	104506956(ADAMTS13)

CMAC:

104483793(ADAMTS13)

CUCA:

104066938(ADAMTS13)

TEO:	104371507(ADAMTS13)

BREG:

104632222(ADAMTS13)

OHA:	104329094(ADAMTS13)

ACAR:

104527328(ADAMTS13)

CPEA:

104389819(ADAMTS13)

CVF:	104292822(ADAMTS13)

RTD:	128917504(ADAMTS13)

CRID:

134523685(ADAMTS13)

AAM:	106483743 106483751

AROW:

112964357(ADAMTS13)

NPD:	112956002(ADAMTS13)

TGT:	104568104(ADAMTS13)

DNE:	112979905(ADAMTS13)

SCAM:

104138242(ADAMTS13)

ASN:	102379615(ADAMTS13)

AMJ:	102565959(ADAMTS13)

CPOO:

109313985(ADAMTS13)

GGN:	109293005(ADAMTS13)

PSS:	102455064(ADAMTS13)

CMY:	102937118(ADAMTS13)

CCAY:

125623621(ADAMTS13)

DCC:	119844204(ADAMTS13)

CPIC:

101940111

TST:	117889553(ADAMTS13)

CABI:

116817469(ADAMTS13)

MRV:	120386668(ADAMTS13)

PVT:	110079591(ADAMTS13)

PBI:	103050431(ADAMTS13)

PMUR:

107300476 107302802(ADAMTS13)

CTIG:

120298847(ADAMTS13)

PGUT:

117656271(ADAMTS13)

PCAE:

139325633(ADAMTS13)

APRI:

131186207(ADAMTS13)

PTEX:

113443776(ADAMTS13)

NSS:	113424825(ADAMTS13)

VKO:	123028314(ADAMTS13)

PMUA:

114589118(ADAMTS13)

PRAF:

128406410(ADAMTS13)

ZVI:	118083798(ADAMTS13)

HCG:	128339105(ADAMTS13)

GJA:	107113557(ADAMTS13)

EMC:	129342084(ADAMTS13)

XLA:

108698851

XTR:	100485801(adamts13)

NPR:	108792836(ADAMTS13)

RTEM:

120913870(ADAMTS13)

ACAT:

141108253(ADAMTS13)

BBUF:

120977996(ADAMTS13)

BGAR:

122946443(ADAMTS13)

DTC:	141046621(ADAMTS13)

PWL:	138301373(ADAMTS13)

MUO:	115473072(ADAMTS13)

GSH:	117368365(ADAMTS13)

DRE:	100320289(adamts13)

SRX:	107725310 107740991(adamts13)

SANH:

107691575(adamts13) 107702807

SGH:	107548275 107565123(adamts13)

CCAR:

109087872(adamts13) 109107832

CAUA:

113070702(adamts13)

CGIB:

128018344

PTET:

122350338(adamts13)

LROH:

127169433(adamts13)

GRF:	141286526(adamts13)

OMC:	131545657(adamts13)

PPRM:

120473833(adamts13)

RKG:	130081960(adamts13)

MAMB:

125279476(adamts13)

CIDE:

127518153

CERY:

137027282(adamts13)

MASI:

127424297

TROS:

130568092(adamts13)

TDW:	130407255(adamts13)

MANU:

129415088(adamts13)

PDAB:

135729706(adamts13)

IPU:

108265332

IFU:	128607488(adamts13)

PHYP:

113533593(adamts13)

SMEO:

124399846(adamts13)

TFD:	113656007(adamts13)

TVC:	132853223(adamts13)

CGAR:

128530605(adamts13)

NGRA:

132892442(adamts13)

TRN:	134318387(adamts13)

AMEX:

103029987(adamts13)

CMAO:

118813326(adamts13)

EEE:	113578195(adamts13)

CHAR:

105894886(adamts13)

SPIL:

134088429(adamts13) 134088430

TRU:	101074374(adamts13)

TFS:	130525865(adamts13)

TNG:	GSTEN00025091G001

LCO:	104923091(adamts13)

NCC:

104954446

TBEN:

117472614(adamts13)

CGOB:

115013592(adamts13)

PGEO:

117452414(adamts13)

GACU:

117543933(adamts13)

EMAC:

134873591(adamts13)

ELY:	117252782(adamts13)

EFO:	125889721(adamts13)

PLEP:

121944087(adamts13)

SLUC:

116050540(adamts13)

ECRA:

117944827(adamts13)

ESP:	116674422 116689894

PFLV:

114555418(adamts13)

GAT:	120830829(adamts13)

PPUG:

119225063(adamts13)

AFB:	129100952(adamts13)

CLUM:

117735888(adamts13)

PSWI:

130196825(adamts13)

CANA:

137778938(adamts13)

CVE:	137866749(adamts13)

MSAM:

119891655(adamts13)

SCHU:

122876145(adamts13)

CUD:	121509701(adamts13)

SPUL:

138610197(adamts13)

LMIX:

132973953(adamts13)

ALAT:

119019795(adamts13)

ASTR:

137592671(adamts13)

MZE:	101477146(adamts13)

ONL:	100696354(adamts13)

OAU:	116323422(adamts13)

OLA:	101165315(adamts13)

OML:	112148121(adamts13)

CSAI:

133451247(adamts13)

XMA:	102237546(adamts13)

XCO:	114154695(adamts13)

XHE:	116729900(adamts13)

PRET:

103470625(adamts13)

PFOR:

103147041(adamts13)

PLAI:

106939142(adamts13)

PMEI:

106917656(adamts13)

GAF:	122828117(adamts13)

PPRL:

129372958(adamts13)

CVG:	107099575(adamts13)

CTUL:

119785140(adamts13)

GMU:	124877659(adamts13)

NFU:	107393634(adamts13)

KMR:	108236553(adamts13)

ALIM:

106516687(adamts13)

NWH:	119414061(adamts13)

AOCE:

111586394(adamts13)

EJA:	138215976(adamts13)

BFRE:

138625665(adamts13)

MCEP:

125012297(adamts13)

POV:	109633866(adamts13)

SSEN:

122769584(adamts13)

SSOE:

131463548(adamts13)

HHIP:

117767414(adamts13)

HSP:	118115158(adamts13)

PPLT:

128438214(adamts13)

SMAU:

118313805(adamts13)

LCF:

108878613

SDU:	111226862(adamts13)

SLAL:

111644942(adamts13)

XGL:	120805232(adamts13)

HCQ:	109511597(adamts13)

SSCV:

125994297(adamts13)

SBIA:

133499364(adamts13)

PEE:	133399665(adamts13)

PTAO:

133475627(adamts13)

BPEC:

110169902(adamts13)

ENY:	140375964(adamts13)

MALB:

109954821(adamts13)

BSPL:

114861250(adamts13)

SJO:	128365262(adamts13)

SSCO:

133979245(adamts13)

GMH:	115545342(adamts13)

SASA:

106579902(adamts13)

STRU:

115200048(adamts13)

OTW:	112262981(adamts13)

OMY:	110535958(adamts13)

OGO:	124036245(adamts13)

ONE:	115139975(adamts13)

OKI:	109895835(adamts13)

OKE:	118358273(adamts13)

OMM:	135514166(adamts13)

OCLA:

139389333

SALP:

111957513(adamts13)

SNH:	120063193(adamts13)

CCLU:

121551928(adamts13)

ELS:	105025189(adamts13)

OEP:	134012118(adamts13)

SFM:	108938135(adamts13)

PKI:	111858732(adamts13)

AANG:

118206510(adamts13)

AROT:

135265099(adamts13)

CCOG:

133140427(adamts13)

LOC:	102695423(adamts13)

PSPA:

121309677(adamts13)

ARUT:

117396819 117422042

PSEX:

120536068(adamts13)

LCM:	102349966(ADAMTS13)

CMK:	103184703(adamts13)

CPLA:

122564630(adamts13)

HOC:	132825770(adamts13)

LERI:

129712093(adamts13)

HAKA:

140731064(adamts13)

LRJ:	133354607(ADAMTS13)

AFIL:

140137278 140137942

GAE:

121383603

HRF:

124145568

HRJ:	124257607 124257610

HASI:

137273097

HCRA:

139459946

CRG:

105336114

CANU:

128155433

MYI:

110455596

MMER:

123549785

DPOL:

127839509

OSN:

115220930

LJP:

135481021

LLON:

135501887

» show all

Reference

1 [PMID:11535495]

Authors

Fujikawa K, Suzuki H, McMullen B, Chung D

Title

Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family.

Journal

Blood 98:1662-6 (2001)
DOI:10.1182/blood.v98.6.1662

Sequence

[hsa:11093]

Reference

2 [PMID:12393397]

Authors

Dong JF, Moake JL, Nolasco L, Bernardo A, Arceneaux W, Shrimpton CN, Schade AJ, McIntire LV, Fujikawa K, Lopez JA

Title

ADAMTS-13 rapidly cleaves newly secreted ultralarge von Willebrand factor multimers on the endothelial surface under flowing conditions.

Journal

Blood 100:4033-9 (2002)
DOI:10.1182/blood-2002-05-1401

Other DBs

ExplorEnz - The Enzyme Database:

3.4.24.87

IUBMB Enzyme Nomenclature:

3.4.24.87

ExPASy - ENZYME nomenclature database:

3.4.24.87

BRENDA, the Enzyme Database:

3.4.24.87

All links

Gene (439)   
   MGENES (261)   
   RefGene (178)   
Protein sequence (85)   
   UniProt (71)   
   SWISS-PROT (2)   
   RefSeq(pep) (8)   
   PDBSTR (4)   
DNA sequence (12)   
   RefSeq(nuc) (9)   
   GenBank (3)   
3D Structure (2)   
   PDB (2)   
Protein domain (11)   
   InterPro (11)   
All databases (549)   

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