KEGG   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
HSA11093(ADAMTS13)
PTR473153(ADAMTS13)
PPS100978943(ADAMTS13)
GGO101151958(ADAMTS13)
PON100459418(ADAMTS13)
NLE100580216(ADAMTS13)
MCC722202(ADAMTS13)
MCF102145096(ADAMTS13)
MTHB126937135
CSAB103239684(ADAMTS13)
CATY105580513(ADAMTS13)
PANU101018650(ADAMTS13)
TGE112607583(ADAMTS13)
RRO104669720(ADAMTS13)
RBB108513335(ADAMTS13)
TFN117099210(ADAMTS13)
PTEH111523982(ADAMTS13)
CJC100404947(ADAMTS13)
SBQ101038728(ADAMTS13)
CSYR103273391(ADAMTS13)
MMUR105880760(ADAMTS13)
LCAT123646680(ADAMTS13)
OGA100960489(ADAMTS13)
MMU279028(Adamts13)
MCAL110312498(Adamts13)
MPAH110317485(Adamts13)
RNO102554393(Adamts13)
MCOC116091905(Adamts13)
MUN110557514(Adamts13)
CGE100770010(Adamts13)
MAUA101827371(Adamts13)
PLEU114684226(Adamts13)
MORG121462687(Adamts13)
MFOT126509437
AAMP119818562(Adamts13)
NGI103742812(Adamts13)
HGL101713585(Adamts13)
CPOC100727045(Adamts13)
CCAN109696127(Adamts13)
DORD105995613(Adamts13)
DSP122108880(Adamts13)
NCAR124964314
OPI101517758
TUP102474078(ADAMTS13)
GVR103608948(ADAMTS13)
CFA100630922(ADAMTS13)
CLUD112677392(ADAMTS13)
VVP112935883(ADAMTS13)
VLG121473072(ADAMTS13)
AML100481099(ADAMTS13)
UMR103679919(ADAMTS13)
UAH113266572(ADAMTS13)
UAR123775676(ADAMTS13)
ELK111145642
LLV125083853
MPUF101694125(ADAMTS13)
ORO101385635(ADAMTS13)
EJU114201001(ADAMTS13)
ZCA113934005(ADAMTS13)
MLX118018186(ADAMTS13)
NSU110581846(ADAMTS13)
LWW102729565(ADAMTS13)
FCA101086761(ADAMTS13)
PYU121021243(ADAMTS13)
PBG122475156(ADAMTS13)
LRUF124522418
PTG102959927(ADAMTS13)
PPAD109258253(ADAMTS13)
AJU106988263(ADAMTS13)
HHV120247311(ADAMTS13)
BTA532272(ADAMTS13)
BOM102287814(ADAMTS13)
BIU109565686(ADAMTS13)
BBUB102412616(ADAMTS13)
BBIS104987950(ADAMTS13)
CHX102174580(ADAMTS13)
OAS101112214(ADAMTS13)
ODA120852324(ADAMTS13)
CCAD122442334(ADAMTS13)
SSC100627950(ADAMTS13)
CFR102506864(ADAMTS13)
CBAI105068608(ADAMTS13)
CDK105106329(ADAMTS13)
VPC102538566(ADAMTS13)
BACU102999611(ADAMTS13)
LVE103074204(ADAMTS13)
OOR101281877(ADAMTS13)
DLE111177028(ADAMTS13)
PCAD102996576
PSIU116755106(ADAMTS13)
ECB100069281(ADAMTS13)
EPZ103565346(ADAMTS13)
EAI106845293(ADAMTS13)
MYB102240335(ADAMTS13)
MYD102764800(ADAMTS13)
MMYO118666904(ADAMTS13)
MLF102443299(ADAMTS13)
MNA107524621(ADAMTS13)
PKL118708873(ADAMTS13)
HAI109382381
DRO112306578(ADAMTS13)
SHON118980285(ADAMTS13)
AJM119036040(ADAMTS13)
PDIC114511173(ADAMTS13)
PHAS123823380(ADAMTS13)
MMF118629978(ADAMTS13)
RFQ117032429(ADAMTS13)
PALE102885523(ADAMTS13)
PGIG120599714(ADAMTS13)
PVP105298645(ADAMTS13)
RAY107514854(ADAMTS13)
MJV108398385(ADAMTS13)
TOD119230354(ADAMTS13)
SARA101548412(ADAMTS13)
LAV100667919(ADAMTS13)
TMU101354225
ETF101657978(ADAMTS13)
DNM101433690(ADAMTS13)
MDO100018539(ADAMTS13)
GAS123235198(ADAMTS13)
SHR100924870(ADAMTS13)
PCW110205378(ADAMTS13)
OAA100077268(ADAMTS13)
GGA417152(ADAMTS13)
PCOC116234434(ADAMTS13)
MGP100544155(ADAMTS13)
CJO107321769(ADAMTS13)
NMEL110406845(ADAMTS13)
APLA101800615(ADAMTS13)
ACYG106044108(ADAMTS13)
AFUL116496934(ADAMTS13)
TGU100226372(ADAMTS13)
LSR110477621(ADAMTS13)
SCAN103819131(ADAMTS13)
PMOA120512419(ADAMTS13)
OTC121336974(ADAMTS13)
PRUF121362425(ADAMTS13)
GFR102043124(ADAMTS13)
FAB101809354(ADAMTS13)
PHI102109550(ADAMTS13)
PMAJ107212053(ADAMTS13)
CCAE111937039(ADAMTS13)
CCW104688336(ADAMTS13)
CBRC103623248(ADAMTS13)
ETL114059081(ADAMTS13)
ZAB102072739(ADAMTS13)
ACHL103809287(ADAMTS13)
FPG101910275(ADAMTS13)
FCH102055781(ADAMTS13)
CLV102096762(ADAMTS13)
EGZ104129330(ADAMTS13)
NNI104023191(ADAMTS13)
PLET104624643(ADAMTS13)
PCRI104028886(ADAMTS13)
PCAO104047343(ADAMTS13)
ACUN113486351(ADAMTS13)
TALA104363650(ADAMTS13)
PADL103915348(ADAMTS13)
AFOR103905329(ADAMTS13)
ACHC115335017(ADAMTS13)
HALD104323021(ADAMTS13)
HLE104833867(ADAMTS13)
AGEN126051980
GCL127023651
CCRI104163978(ADAMTS13)
CSTI104561782(ADAMTS13)
EHS104506956(ADAMTS13)
CMAC104483793(ADAMTS13)
MUI104545065(ADAMTS13)
FGA104075580(ADAMTS13)
GSTE104261947(ADAMTS13)
LDI104354039(ADAMTS13)
MNB103774154(ADAMTS13)
OHA104329094(ADAMTS13)
NNT104406931(ADAMTS13)
DPUB104296479(ADAMTS13)
PGUU104469452(ADAMTS13)
ACAR104527328(ADAMTS13)
CPEA104389819(ADAMTS13)
AVIT104278033(ADAMTS13)
CVF104292822(ADAMTS13)
CUCA104066938(ADAMTS13)
TEO104371507(ADAMTS13)
BRHI104489548(ADAMTS13)
AAM106483743 106483751
AROW112964357(ADAMTS13)
NPD112956002(ADAMTS13)
TGT104568104(ADAMTS13)
DNE112979905(ADAMTS13)
SCAM104138242(ADAMTS13)
ASN102379615(ADAMTS13)
AMJ102565959(ADAMTS13)
CPOO109313985(ADAMTS13)
GGN109293005(ADAMTS13)
PSS102455064(ADAMTS13)
CMY102937118(ADAMTS13)
CPIC101940111
TST117889553(ADAMTS13)
CABI116817469(ADAMTS13)
MRV120386668(ADAMTS13)
PVT110079591(ADAMTS13)
PBI103050431(ADAMTS13)
PMUR107300476 107302802(ADAMTS13)
CTIG120298847(ADAMTS13)
VKO123028314(ADAMTS13)
PMUA114589118(ADAMTS13)
ZVI118083798(ADAMTS13)
GJA107113557(ADAMTS13)
XLA108698851
XTR100485801(adamts13)
NPR108792836(ADAMTS13)
RTEM120913870(ADAMTS13)
BBUF120977996(ADAMTS13)
BGAR122946443(ADAMTS13)
DRE100320289(adamts13)
SRX107725310 107740991(adamts13)
SANH107691575(adamts13) 107702807
SGH107548275 107565123(adamts13)
CCAR109087872
CAUA113070702(adamts13)
PPRM120473833(adamts13)
MAMB125279476(adamts13)
IPU108265332(adamts13)
PHYP113533593(adamts13)
SMEO124399846(adamts13)
TFD113656007(adamts13)
AMEX103029987(adamts13)
EEE113578195(adamts13)
TRU101074374(adamts13)
TNGGSTEN00025091G001
LCO104923091(adamts13)
NCC104954446
CGOB115013592(adamts13)
ELY117252782(adamts13)
EFO125889721(adamts13)
PLEP121944087(adamts13)
SLUC116050540(adamts13)
ECRA117944827(adamts13)
ESP116674422 116689894
PFLV114555418(adamts13)
GAT120830829(adamts13)
PPUG119225063(adamts13)
MSAM119891655(adamts13)
CUD121509701(adamts13)
ALAT119019795(adamts13)
MZE101477146(adamts13)
ONL100696354(adamts13)
OAU116323422(adamts13)
OLA101165315(adamts13)
OML112148121(adamts13)
XMA102237546(adamts13)
XCO114154695(adamts13)
XHE116729900(adamts13)
PRET103470625(adamts13)
PFOR103147041(adamts13)
PLAI106939142(adamts13)
PMEI106917656(adamts13)
GAF122828117(adamts13)
CVG107099575(adamts13)
CTUL119785140(adamts13)
GMU124877659(adamts13)
NFU107393634(adamts13)
KMR108236553(adamts13)
ALIM106516687(adamts13)
NWH119414061(adamts13)
AOCE111586394(adamts13)
MCEP125012297(adamts13)
POV109633866(adamts13)
SSEN122769584(adamts13)
HHIP117767414(adamts13)
HSP118115158(adamts13)
LCF108878613
SDU111226862(adamts13)
SLAL111644942(adamts13)
XGL120805232(adamts13)
HCQ109511597(adamts13)
BPEC110169902(adamts13)
MALB109954821(adamts13)
BSPL114861250(adamts13)
SASA106579902(adamts13)
STRU115200048(adamts13)
OTW112262981(adamts13)
OMY110535958(adamts13)
OGO124036245(adamts13)
ONE115139975(adamts13)
SALP111957513(adamts13)
SNH120063193(adamts13)
CCLU121551928(adamts13)
ELS105025189(adamts13)
SFM108938135(adamts13)
PKI111858732(adamts13)
AANG118206510(adamts13)
LOC102695423(adamts13)
PSPA121309677(adamts13)
ARUT117396819 117422042
PSEX120536068(adamts13)
LCM102349966(ADAMTS13)
CMK103184703(adamts13)
GAE121383603
HRF124145568
HRJ124257607 124257610
CRG105336114
MYI110455596
MMER123549007 123549785
OSN115220930
 » 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

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