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Sequence of ATS13_HUMAN

EC Number:3.4.24.87

EC Number
Recommended Name
Accession Code
Organism
No of amino acids
Molecular Weight [Da]
Source
ADAMTS13 endopeptidase
Q76LX8
Homo sapiens
1427
153604
Reaction
The enzyme cleaves the von Willebrand factor at bond Tyr842-/-Met843 within the A2 domain
Other sequences found for EC No. 3.4.24.87

General information:

Sequence
show sequence in fasta format
   0 MHQRHPRARC PPLCVAGILA CGFLLGCWGP SHFQQSCLQA LEPQAVSSYL SPGAPLKGRP
  60 PSPGFQRQRQ RQRRAAGGIL HLELLVAVGP DVFQAHQEDT ERYVLTNLNI GAELLRDPSL
 120 GAQFRVHLVK MVILTEPEGA PNITANLTSS LLSVCGWSQT INPEDDTDPG HADLVLYITR
 180 FDLELPDGNR QVRGVTQLGG ACSPTWSCLI TEDTGFDLGV TIAHEIGHSF GLEHDGAPGS
 240 GCGPSGHVMA SDGAAPRAGL AWSPCSRRQL LSLLSAGRAR CVWDPPRPQP GSAGHPPDAQ
 300 PGLYYSANEQ CRVAFGPKAV ACTFAREHLD MCQALSCHTD PLDQSSCSRL LVPLLDGTEC
 360 GVEKWCSKGR CRSLVELTPI AAVHGRWSSW GPRSPCSRSC GGGVVTRRRQ CNNPRPAFGG
 420 RACVGADLQA EMCNTQACEK TQLEFMSQQC ARTDGQPLRS SPGGASFYHW GAAVPHSQGD
 480 ALCRHMCRAI GESFIMKRGD SFLDGTRCMP SGPREDGTLS LCVSGSCRTF GCDGRMDSQQ
 540 VWDRCQVCGG DNSTCSPRKG SFTAGRAREY VTFLTVTPNL TSVYIANHRP LFTHLAVRIG
 600 GRYVVAGKMS ISPNTTYPSL LEDGRVEYRV ALTEDRLPRL EEIRIWGPLQ EDADIQVYRR
 660 YGEEYGNLTR PDITFTYFQP KPRQAWVWAA VRGPCSVSCG AGLRWVNYSC LDQARKELVE
 720 TVQCQGSQQP PAWPEACVLE PCPPYWAVGD FGPCSASCGG GLRERPVRCV EAQGSLLKTL
 780 PPARCRAGAQ QPAVALETCN PQPCPARWEV SEPSSCTSAG GAGLALENET CVPGADGLEA
 840 PVTEGPGSVD EKLPAPEPCV GMSCPPGWGH LDATSAGEKA PSPWGSIRTG AQAAHVWTPA
 900 AGSCSVSCGR GLMELRFLCM DSALRVPVQE ELCGLASKPG SRREVCQAVP CPARWQYKLA
 960 ACSVSCGRGV VRRILYCARA HGEDDGEEIL LDTQCQGLPR PEPQEACSLE PCPPRWKVMS
1020 LGPCSASCGL GTARRSVACV QLDQGQDVEV DEAACAALVR PEASVPCLIA DCTYRWHVGT
1080 WMECSVSCGD GIQRRRDTCL GPQAQAPVPA DFCQHLPKPV TVRGCWAGPC VGQGTPSLVP
1140 HEEAAAPGRT TATPAGASLE WSQARGLLFS PAPQPRRLLP GPQENSVQSS ACGRQHLEPT
1200 GTIDMRGPGQ ADCAVAIGRP LGEVVTLRVL ESSLNCSAGD MLLLWGRLTW RKMCRKLLDM
1260 TFSSKTNTLV VRQRCGRPGG GVLLRYGSQL APETFYRECD MQLFGPWGEI VSPSLSPATS
1320 NAGGCRLFIN VAPHARIAIH ALATNMGAGT EGANASYILI RDTHSLRTTA FHGQQVLYWE
1380 SESSQAEMEF SEGFLKAQAS LRGQYWTLQS WVPEMQDPQS WKGKEGT
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Sequence related references
Sequence Reference
Authors
Title
Journal
Volume
Pages
Year
PubMed ID
9241
Soejima K.,Mimura N.,Hirashima M.,Maeda H.,Hamamoto T.,Nakagaki T.,Nozaki C.
A novel human metalloprotease synthesized in the liver and secreted into the blood: possibly, the von Willebrand factor-cleaving protease?
J. Biochem.
130
475-480
2001
9242
Zheng X.,Chung D.,Takayama T.K.,Majerus E.M.,Sadler J.E.,Fujikawa K.
Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura.
J. Biol. Chem.
276
41059-41063
2001
9243
Levy G.G.,Nichols W.C.,Lian E.C.,Foroud T.,McClintick J.N.,McGee B.M.,Yang A.Y.,Siemieniak D.R.,Stark K.R.,Gruppo R.,Sarode R.,Shurin S.B.,Chandrasekaran V.,Stabler S.P.,Sabio H.,Bouhassira E.E.,Upshaw J.D. Jr.,Ginsburg D.,Tsai H.-M.
Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura.
Nature
413
488-494
2001
9244
Cal S.,Obaya A.J.,Llamazares M.,Garabaya C.,Quesada V.,Lopez-Otin C.
Cloning, expression analysis, and structural characterization of seven novel human ADAMTSs, a family of metalloproteinases with disintegrin and thrombospondin-1 domains.
Gene
283
49-62
2002
9247
Humphray S.J.,Oliver K.,Hunt A.R.,Plumb R.W.,Loveland J.E.,Howe K.L.,Andrews T.D.,Searle S.,Hunt S.E.,Scott C.E.,Jones M.C.,Ainscough R.,Almeida J.P.,Ambrose K.D.,Ashwell R.I.S.,Babbage A.K.,Babbage S.,Bagguley C.L.,Bailey J.,Banerjee R.,Barker D.J.,Barlow K.F.,Bates K.,Beasley H.,Beasley O.,Bird C.P.,Bray-Allen S.,Brown A.J.,Brown J.Y.,Burford D.,Burrill W.,Burton J.,Carder C.,Carter N.P.,Chapman J.C.,Chen Y.,Clarke G.,Clark S.Y.,Clee C.M.,Clegg S.,Collier R.E.,Corby N.,Crosier M.,Cummings A.T.,Davies J.,Dhami P.,Dunn M.,Dutta I.,Dyer L.W.,Earthrowl M.E.,Faulkner L.,Fleming C.J.,Frankish A.,Frankland J.A.,French L.,Fricker D.G.,Garner P.,Garnett J.,Ghori J.,Gilbert J.G.R.,Glison C.,Grafham D.V.,Gribble S.,Griffiths C.,Griffiths-Jones S.,Grocock R.,Guy J.,Hall R.E.,Hammond S.,Harley J.L.,Harrison E.S.I.,Hart E.A.,Heath P.D.,Henderson C.D.,Hopkins B.L.,Howard P.J.,Howden P.J.,Huckle E.,Johnson C.,Johnson D.,Joy A.A.,Kay M.,Keenan S.,Kershaw J.K.,Kimberley A.M.,King A.,Knights A.,Laird G.K.,Langford C.,Lawlor S.,Leongamornlert D.A.,Leversha M.,Lloyd C.,Lloyd D.M.,Lovell J.,Martin S.,Mashreghi-Mohammadi M.,Matthews L.,McLaren S.,McLay K.E.,McMurray A.,Milne S.,Nickerson T.,Nisbett J.,Nordsiek G.,Pearce A.V.,Peck A.I.,Porter K.M.,Pandian R.,Pelan S.,Phillimore B.,Povey S.,Ramsey Y.,Rand V.,Scharfe M.,Sehra H.K.,Shownkeen R.,Sims S.K.,Skuce C.D.,Smith M.,Steward C.A.,Swarbreck D.,Sycamore N.,Tester J.,Thorpe A.,Tracey A.,Tromans A.,Thomas D.W.,Wall M.,Wallis J.M.,West A.P.,Whitehead S.L.,Willey D.L.,Williams S.A.,Wilming L.,Wray P.W.,Young L.,Ashurst J.L.,Coulson A.,Blocker H.,Durbin R.M.,Sulston J.E.,Hubbard T.,Jackson M.J.,Bentley D.R.,Beck S.,Rogers J.,Dunham I.
DNA sequence and analysis of human chromosome 9.
Nature
429
369-374
2004
9249
Clark H.F.,Gurney A.L.,Abaya E.,Baker K.,Baldwin D.T.,Brush J.,Chen J.,Chow B.,Chui C.,Crowley C.,Currell B.,Deuel B.,Dowd P.,Eaton D.,Foster J.S.,Grimaldi C.,Gu Q.,Hass P.E.,Heldens S.,Huang A.,Kim H.S.,Klimowski L.,Jin Y.,Johnson S.,Lee J.,Lewis L.,Liao D.,Mark M.R.,Robbie E.,Sanchez C.,Schoenfeld J.,Seshagiri S.,Simmons L.,Singh J.,Smith V.,Stinson J.,Vagts A.,Vandlen R.L.,Watanabe C.,Wieand D.,Woods K.,Xie M.-H.,Yansura D.G.,Yi S.,Yu G.,Yuan J.,Zhang M.,Zhang Z.,Goddard A.D.,Wood W.I.,Godowski P.J.,Gray A.M.
The secreted protein discovery initiative (SPDI), a large-scale effort to identify novel human secreted and transmembrane proteins: a bioinformatics assessment.
Genome Res.
13
2265-2270
2003
9250
Wiemann S.,Weil B.,Wellenreuther R.,Gassenhuber J.,Glassl S.,Ansorge W.,Boecher M.,Bloecker H.,Bauersachs S.,Blum H.,Lauber J.,Duesterhoeft A.,Beyer A.,Koehrer K.,Strack N.,Mewes H.-W.,Ottenwaelder B.,Obermaier B.,Tampe J.,Heubner D.,Wambutt R.,Korn B.,Klein M.,Poustka A.
Towards a catalog of human genes and proteins: sequencing and analysis of 500 novel complete protein coding human cDNAs.
Genome Res.
11
422-435
2001
9251
Gerritsen H.E.,Robles R.,Laemmle B.,Furlan M.
Partial amino acid sequence of purified von Willebrand factor-cleaving protease.
Blood
98
1654-1661
2001
9252
Fujikawa K.,Suzuki H.,McMullen B.,Chung D.
Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family.
Blood
98
1662-1666
2001
9253
Zheng X.,Nishio K.,Majerus E.M.,Sadler J.E.
Cleavage of von Willebrand factor requires the spacer domain of the metalloprotease ADAMTS13.
J. Biol. Chem.
278
30136-30141
2003
9254
Majerus E.M.,Zheng X.,Tuley E.A.,Sadler J.E.
Cleavage of the ADAMTS13 propeptide is not required for protease activity.
J. Biol. Chem.
278
46643-46648
2003
9255
Liu T.,Qian W.-J.,Gritsenko M.A.,Camp D.G. II,Monroe M.E.,Moore R.J.,Smith R.D.
Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry.
J. Proteome Res.
4
2070-2080
2005
9256
Anderson P.J.,Kokame K.,Sadler J.E.
Zinc and calcium ions cooperatively modulate ADAMTS13 activity.
J. Biol. Chem.
281
850-857
2006
9257
Ricketts L.M.,Dlugosz M.,Luther K.B.,Haltiwanger R.S.,Majerus E.M.
O-fucosylation is required for ADAMTS13 secretion.
J. Biol. Chem.
282
17014-17023
2007
9258
Gardner M.D.,Chion C.K.,de Groot R.,Shah A.,Crawley J.T.,Lane D.A.
A functional calcium-binding site in the metalloprotease domain of ADAMTS13.
Blood
113
1149-1157
2009
9259
Jia W.,Lu Z.,Fu Y.,Wang H.P.,Wang L.H.,Chi H.,Yuan Z.F.,Zheng Z.B.,Song L.N.,Han H.H.,Liang Y.M.,Wang J.L.,Cai Y.,Zhang Y.K.,Deng Y.L.,Ying W.T.,He S.M.,Qian X.H.
A strategy for precise and large scale identification of core fucosylated glycoproteins.
Mol. Cell. Proteomics
8
913-923
2009
9260
Lotta L.A.,Garagiola I.,Palla R.,Cairo A.,Peyvandi F.
ADAMTS13 mutations and polymorphisms in congenital thrombotic thrombocytopenic purpura.
Hum. Mutat.
31
11-19
2010
9261
Akiyama M.,Takeda S.,Kokame K.,Takagi J.,Miyata T.
Crystal structures of the noncatalytic domains of ADAMTS13 reveal multiple discontinuous exosites for von Willebrand factor.
Proc. Natl. Acad. Sci. U.S.A.
106
19274-19279
2009
9262
Kokame K.,Matsumoto M.,Soejima K.,Yagi H.,Ishizashi H.,Funato M.,Tamai H.,Konno M.,Kamide K.,Kawano Y.,Miyata T.,Fujimura Y.
Mutations and common polymorphisms in ADAMTS13 gene responsible for von Willebrand factor-cleaving protease activity.
Proc. Natl. Acad. Sci. U.S.A.
99
11902-11907
2002
9263
Schneppenheim R.,Budde U.,Oyen F.,Angerhaus D.,Aumann V.,Drewke E.,Hassenpflug W.,Haberle J.,Kentouche K.,Kohne E.,Kurnik K.,Mueller-Wiefel D.,Obser T.,Santer R.,Sykora K.W.
von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP.
Blood
101
1845-1850
2003
9264
Antoine G.,Zimmermann K.,Plaimauer B.,Grillowitzer M.,Studt J.D.,Lammle B.,Scheiflinger F.
ADAMTS13 gene defects in two brothers with constitutional thrombotic thrombocytopenic purpura and normalization of von Willebrand factor-cleaving protease activity by recombinant human ADAMTS13.
Br. J. Haematol.
120
821-824
2003
9265
Assink K.,Schiphorst R.,Allford S.,Karpman D.,Etzioni A.,Brichard B.,van de Kar N.,Monnens L.,van den Heuvel L.
Mutation analysis and clinical implications of von Willebrand factor-cleaving protease deficiency.
Kidney Int.
63
1995-1999
2003
9266
Pimanda J.E.,Maekawa A.,Wind T.,Paxton J.,Chesterman C.N.,Hogg P.J.
Congenital thrombotic thrombocytopenic purpura in association with a mutation in the second CUB domain of ADAMTS13.
Blood
103
627-629
2004
9267
Matsumoto M.,Kokame K.,Soejima K.,Miura M.,Hayashi S.,Fujii Y.,Iwai A.,Ito E.,Tsuji Y.,Takeda-Shitaka M.,Iwadate M.,Umeyama H.,Yagi H.,Ishizashi H.,Banno F.,Nakagaki T.,Miyata T.,Fujimura Y.
Molecular characterization of ADAMTS13 gene mutations in Japanese patients with Upshaw-Schulman syndrome.
Blood
103
1305-1310
2004
9268
Uchida T.,Wada H.,Mizutani M.,Iwashita M.,Ishihara H.,Shibano T.,Suzuki M.,Matsubara Y.,Soejima K.,Matsumoto M.,Fujimura Y.,Ikeda Y.,Murata M.
Identification of novel mutations in ADAMTS13 in an adult patient with congenital thrombotic thrombocytopenic purpura.
Blood
104
2081-2083
2004
9269
Veyradier A.,Lavergne J.M.,Ribba A.S.,Obert B.,Loirat C.,Meyer D.,Girma J.P.
Ten candidate ADAMTS13 mutations in six French families with congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome).
J. Thromb. Haemost.
2
424-429
2004
9270
Licht C.,Stapenhorst L.,Simon T.,Budde U.,Schneppenheim R.,Hoppe B.
Two novel ADAMTS13 gene mutations in thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome (TTP/HUS).
Kidney Int.
66
955-958
2004
9271
Liu F.,Jin J.,Dong N.Z.,Wang Y.G.,Ruan C.G.
Identification of two novel mutations in ADAMTS13 gene in a patient with hereditary thrombotic thrombocytopenic purpura.
Zhonghua Xue Ye Xue Za Zhi
26
521-524
2005
9272
Plaimauer B.,Fuhrmann J.,Mohr G.,Wernhart W.,Bruno K.,Ferrari S.,Konetschny C.,Antoine G.,Rieger M.,Scheiflinger F.
Modulation of ADAMTS13 secretion and specific activity by a combination of common amino acid polymorphisms and a missense mutation.
Blood
107
118-125
2006
9273
Peyvandi F.,Lavoretano S.,Palla R.,Valsecchi C.,Merati G.,De Cristofaro R.,Rossi E.,Mannuccio Mannucci P.
Mechanisms of the interaction between two ADAMTS13 gene mutations leading to severe deficiency of enzymatic activity.
Hum. Mutat.
27
330-336
2006
9274
Tao Z.,Anthony K.,Peng Y.,Choi H.,Nolasco L.,Rice L.,Moake J.L.,Dong J.F.
Novel ADAMTS-13 mutations in an adult with delayed onset thrombotic thrombocytopenic purpura.
J. Thromb. Haemost.
4
1931-1935
2006
9275
Shibagaki Y.,Matsumoto M.,Kokame K.,Ohba S.,Miyata T.,Fujimura Y.,Fujita T.
Novel compound heterozygote mutations (H234Q/R1206X) of the ADAMTS13 gene in an adult patient with Upshaw-Schulman syndrome showing predominant episodes of repeated acute renal failure.
Nephrol. Dial. Transplant.
21
1289-1292
2006
9276
Schneppenheim R.,Kremer Hovinga J.A.,Becker T.,Budde U.,Karpman D.,Brockhaus W.,Hrachovinova I.,Korczowski B.,Oyen F.,Rittich S.,von Rosen J.,Tjonnfjord G.E.,Pimanda J.E.,Wienker T.F.,Lammle B.
A common origin of the 4143insA ADAMTS13 mutation.
Thromb. Haemost.
96
3-6
2006
9277
Donadelli R.,Banterla F.,Galbusera M.,Capoferri C.,Bucchioni S.,Gastoldi S.,Nosari S.,Monteferrante G.,Ruggeri Z.M.,Bresin E.,Scheiflinger F.,Rossi E.,Martinez C.,Coppo R.,Remuzzi G.,Noris M.
In-vitro and in-vivo consequences of mutations in the von Willebrand factor cleaving protease ADAMTS13 in thrombotic thrombocytopenic purpura.
Thromb. Haemost.
96
454-464
2006
9278
Meyer S.C.,Jeddi R.,Meddeb B.,Gouider E.,Lammle B.,Kremer Hovinga J.A.
A first case of congenital TTP on the African continent due to a new homozygous mutation in the catalytic domain of ADAMTS13.
Ann. Hematol.
87
663-666
2008
9279
Fujimura Y.,Matsumoto M.,Kokame K.,Isonishi A.,Soejima K.,Akiyama N.,Tomiyama J.,Natori K.,Kuranishi Y.,Imamura Y.,Inoue N.,Higasa S.,Seike M.,Kozuka T.,Hara M.,Wada H.,Murata M.,Ikeda Y.,Miyata T.,George J.N.
Pregnancy-induced thrombocytopenia and TTP, and the risk of fetal death, in Upshaw-Schulman syndrome: a series of 15 pregnancies in 9 genotyped patients.
Br. J. Haematol.
144
742-754
2009
9280
Palla R.,Lavoretano S.,Lombardi R.,Garagiola I.,Karimi M.,Afrasiabi A.,Ramzi M.,De Cristofaro R.,Peyvandi F.
The first deletion mutation in the TSP1-6 repeat domain of ADAMTS13 in a family with inherited thrombotic thrombocytopenic purpura.
Haematologica
94
289-293
2009
9281
Lee S.H.,Park J.H.,Park S.K.,Lee E.H.,Choi J.I.,Visentin G.P.,Park T.S.,Oh S.H.,Kim S.R.
A novel homozygous missense ADAMTS13 mutation Y658C in a patient with recurrent thrombotic thrombocytopenic purpura.
Ann. Clin. Lab. Sci.
41
273-276
2011
9282
Choi H.S.,Cheong H.I.,Kim N.K.,Oh D.,Park H.W.
ADAMTS13 gene mutations in children with hemolytic uremic syndrome.
Yonsei Med. J.
52
530-534
2011