Details for: ADAMTS13

Gene ID: 11093

Symbol: ADAMTS13

Ensembl ID: ENSG00000160323

Description: ADAM metallopeptidase with thrombospondin type 1 motif 13

Associated with

Other Information

Genular Protein ID: 1060051062

Symbol: ATS13_HUMAN

Name: A disintegrin and metalloproteinase with thrombospondin motifs 13

UniProtKB Accession Codes:

Database IDs:

Citations:

PubMed ID: 11574066

Title: A novel human metalloprotease synthesized in the liver and secreted into the blood: possibly, the von Willebrand factor-cleaving protease?

PubMed ID: 11574066

DOI: 10.1093/oxfordjournals.jbchem.a003009

PubMed ID: 11557746

Title: Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura.

PubMed ID: 11557746

DOI: 10.1074/jbc.c100515200

PubMed ID: 11586351

Title: Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura.

PubMed ID: 11586351

DOI: 10.1038/35097008

PubMed ID: 11867212

Title: Cloning, expression analysis, and structural characterization of seven novel human ADAMTSs, a family of metalloproteinases with disintegrin and thrombospondin-1 domains.

PubMed ID: 11867212

DOI: 10.1016/s0378-1119(01)00861-7

PubMed ID: 15164053

Title: DNA sequence and analysis of human chromosome 9.

PubMed ID: 15164053

DOI: 10.1038/nature02465

PubMed ID: 12975309

Title: The secreted protein discovery initiative (SPDI), a large-scale effort to identify novel human secreted and transmembrane proteins: a bioinformatics assessment.

PubMed ID: 12975309

DOI: 10.1101/gr.1293003

PubMed ID: 11230166

Title: Towards a catalog of human genes and proteins: sequencing and analysis of 500 novel complete protein coding human cDNAs.

PubMed ID: 11230166

DOI: 10.1101/gr.gr1547r

PubMed ID: 11535494

Title: Partial amino acid sequence of purified von Willebrand factor-cleaving protease.

PubMed ID: 11535494

DOI: 10.1182/blood.v98.6.1654

PubMed ID: 11535495

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

PubMed ID: 11535495

DOI: 10.1182/blood.v98.6.1662

PubMed ID: 12791682

Title: Cleavage of von Willebrand factor requires the spacer domain of the metalloprotease ADAMTS13.

PubMed ID: 12791682

DOI: 10.1074/jbc.m305331200

PubMed ID: 12975358

Title: Cleavage of the ADAMTS13 propeptide is not required for protease activity.

PubMed ID: 12975358

DOI: 10.1074/jbc.m309872200

PubMed ID: 16335952

Title: Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry.

PubMed ID: 16335952

DOI: 10.1021/pr0502065

PubMed ID: 16286459

Title: Zinc and calcium ions cooperatively modulate ADAMTS13 activity.

PubMed ID: 16286459

DOI: 10.1074/jbc.m504540200

PubMed ID: 17395589

Title: O-fucosylation is required for ADAMTS13 secretion.

PubMed ID: 17395589

DOI: 10.1074/jbc.m700317200

PubMed ID: 19047683

Title: A functional calcium-binding site in the metalloprotease domain of ADAMTS13.

PubMed ID: 19047683

DOI: 10.1182/blood-2008-03-144683

PubMed ID: 19139490

Title: A strategy for precise and large scale identification of core fucosylated glycoproteins.

PubMed ID: 19139490

DOI: 10.1074/mcp.m800504-mcp200

PubMed ID: 19847791

Title: ADAMTS13 mutations and polymorphisms in congenital thrombotic thrombocytopenic purpura.

PubMed ID: 19847791

DOI: 10.1002/humu.21143

PubMed ID: 19880749

Title: Crystal structures of the noncatalytic domains of ADAMTS13 reveal multiple discontinuous exosites for von Willebrand factor.

PubMed ID: 19880749

DOI: 10.1073/pnas.0909755106

PubMed ID: 12181489

Title: Mutations and common polymorphisms in ADAMTS13 gene responsible for von Willebrand factor-cleaving protease activity.

PubMed ID: 12181489

DOI: 10.1073/pnas.172277399

PubMed ID: 12393505

Title: von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP.

PubMed ID: 12393505

DOI: 10.1182/blood-2002-08-2399

PubMed ID: 12614216

Title: ADAMTS13 gene defects in two brothers with constitutional thrombotic thrombocytopenic purpura and normalization of von Willebrand factor-cleaving protease activity by recombinant human ADAMTS13.

PubMed ID: 12614216

DOI: 10.1046/j.1365-2141.2003.04183.x

PubMed ID: 12753286

Title: Mutation analysis and clinical implications of von Willebrand factor-cleaving protease deficiency.

PubMed ID: 12753286

DOI: 10.1046/j.1523-1755.63.6s.1.x

PubMed ID: 14512317

Title: Congenital thrombotic thrombocytopenic purpura in association with a mutation in the second CUB domain of ADAMTS13.

PubMed ID: 14512317

DOI: 10.1182/blood-2003-04-1346

PubMed ID: 14563640

Title: Molecular characterization of ADAMTS13 gene mutations in Japanese patients with Upshaw-Schulman syndrome.

PubMed ID: 14563640

DOI: 10.1182/blood-2003-06-1796

PubMed ID: 15126318

Title: Identification of novel mutations in ADAMTS13 in an adult patient with congenital thrombotic thrombocytopenic purpura.

PubMed ID: 15126318

DOI: 10.1182/blood-2004-02-0715

PubMed ID: 15009458

Title: Ten candidate ADAMTS13 mutations in six French families with congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome).

PubMed ID: 15009458

DOI: 10.1111/j.1538-7933.2004.00623.x

PubMed ID: 15327386

Title: Two novel ADAMTS13 gene mutations in thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome (TTP/HUS).

PubMed ID: 15327386

DOI: 10.1111/j.1523-1755.2004.00841.x

PubMed ID: 16468327

Title: Identification of two novel mutations in ADAMTS13 gene in a patient with hereditary thrombotic thrombocytopenic purpura.

PubMed ID: 16468327

PubMed ID: 16160007

Title: Modulation of ADAMTS13 secretion and specific activity by a combination of common amino acid polymorphisms and a missense mutation.

PubMed ID: 16160007

DOI: 10.1182/blood-2005-06-2482

PubMed ID: 16453338

Title: Mechanisms of the interaction between two ADAMTS13 gene mutations leading to severe deficiency of enzymatic activity.

PubMed ID: 16453338

DOI: 10.1002/humu.20267

PubMed ID: 16796708

Title: Novel ADAMTS-13 mutations in an adult with delayed onset thrombotic thrombocytopenic purpura.

PubMed ID: 16796708

DOI: 10.1111/j.1538-7836.2006.02098.x

PubMed ID: 16449289

Title: 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.

PubMed ID: 16449289

DOI: 10.1093/ndt/gfk072

PubMed ID: 16807643

Title: A common origin of the 4143insA ADAMTS13 mutation.

PubMed ID: 16807643

DOI: 10.1160/th05-12-0817

PubMed ID: 17003922

Title: In-vitro and in-vivo consequences of mutations in the von Willebrand factor cleaving protease ADAMTS13 in thrombotic thrombocytopenic purpura.

PubMed ID: 17003922

DOI: 10.1160/th06-05-0236

PubMed ID: 18443791

Title: A first case of congenital TTP on the African continent due to a new homozygous mutation in the catalytic domain of ADAMTS13.

PubMed ID: 18443791

DOI: 10.1007/s00277-008-0496-6

PubMed ID: 19055667

Title: Pregnancy-induced thrombocytopenia and TTP, and the risk of fetal death, in Upshaw-Schulman syndrome: a series of 15 pregnancies in 9 genotyped patients.

PubMed ID: 19055667

DOI: 10.1111/j.1365-2141.2008.07515.x

PubMed ID: 19116307

Title: The first deletion mutation in the TSP1-6 repeat domain of ADAMTS13 in a family with inherited thrombotic thrombocytopenic purpura.

PubMed ID: 19116307

DOI: 10.3324/haematol.13524

PubMed ID: 22075512

Title: A novel homozygous missense ADAMTS13 mutation Y658C in a patient with recurrent thrombotic thrombocytopenic purpura.

PubMed ID: 22075512

PubMed ID: 21488199

Title: ADAMTS13 gene mutations in children with hemolytic uremic syndrome.

PubMed ID: 21488199

DOI: 10.3349/ymj.2011.52.3.530

Sequence Information:

  • Length: 1427
  • Mass: 153604
  • Checksum: A2103AFABC1A4445
  • Sequence:
  • MHQRHPRARC PPLCVAGILA CGFLLGCWGP SHFQQSCLQA LEPQAVSSYL SPGAPLKGRP 
    PSPGFQRQRQ RQRRAAGGIL HLELLVAVGP DVFQAHQEDT ERYVLTNLNI GAELLRDPSL 
    GAQFRVHLVK MVILTEPEGA PNITANLTSS LLSVCGWSQT INPEDDTDPG HADLVLYITR 
    FDLELPDGNR QVRGVTQLGG ACSPTWSCLI TEDTGFDLGV TIAHEIGHSF GLEHDGAPGS 
    GCGPSGHVMA SDGAAPRAGL AWSPCSRRQL LSLLSAGRAR CVWDPPRPQP GSAGHPPDAQ 
    PGLYYSANEQ CRVAFGPKAV ACTFAREHLD MCQALSCHTD PLDQSSCSRL LVPLLDGTEC 
    GVEKWCSKGR CRSLVELTPI AAVHGRWSSW GPRSPCSRSC GGGVVTRRRQ CNNPRPAFGG 
    RACVGADLQA EMCNTQACEK TQLEFMSQQC ARTDGQPLRS SPGGASFYHW GAAVPHSQGD 
    ALCRHMCRAI GESFIMKRGD SFLDGTRCMP SGPREDGTLS LCVSGSCRTF GCDGRMDSQQ 
    VWDRCQVCGG DNSTCSPRKG SFTAGRAREY VTFLTVTPNL TSVYIANHRP LFTHLAVRIG 
    GRYVVAGKMS ISPNTTYPSL LEDGRVEYRV ALTEDRLPRL EEIRIWGPLQ EDADIQVYRR 
    YGEEYGNLTR PDITFTYFQP KPRQAWVWAA VRGPCSVSCG AGLRWVNYSC LDQARKELVE 
    TVQCQGSQQP PAWPEACVLE PCPPYWAVGD FGPCSASCGG GLRERPVRCV EAQGSLLKTL 
    PPARCRAGAQ QPAVALETCN PQPCPARWEV SEPSSCTSAG GAGLALENET CVPGADGLEA 
    PVTEGPGSVD EKLPAPEPCV GMSCPPGWGH LDATSAGEKA PSPWGSIRTG AQAAHVWTPA 
    AGSCSVSCGR GLMELRFLCM DSALRVPVQE ELCGLASKPG SRREVCQAVP CPARWQYKLA 
    ACSVSCGRGV VRRILYCARA HGEDDGEEIL LDTQCQGLPR PEPQEACSLE PCPPRWKVMS 
    LGPCSASCGL GTARRSVACV QLDQGQDVEV DEAACAALVR PEASVPCLIA DCTYRWHVGT 
    WMECSVSCGD GIQRRRDTCL GPQAQAPVPA DFCQHLPKPV TVRGCWAGPC VGQGTPSLVP 
    HEEAAAPGRT TATPAGASLE WSQARGLLFS PAPQPRRLLP GPQENSVQSS ACGRQHLEPT 
    GTIDMRGPGQ ADCAVAIGRP LGEVVTLRVL ESSLNCSAGD MLLLWGRLTW RKMCRKLLDM 
    TFSSKTNTLV VRQRCGRPGG GVLLRYGSQL APETFYRECD MQLFGPWGEI VSPSLSPATS 
    NAGGCRLFIN VAPHARIAIH ALATNMGAGT EGANASYILI RDTHSLRTTA FHGQQVLYWE 
    SESSQAEMEF SEGFLKAQAS LRGQYWTLQS WVPEMQDPQS WKGKEGT

Database document:

This is a preview of the gene's schema. Only a few entries are kept for 'singleCellExpressions,' 'mRNAExpressions,' and other large data arrays for visualization purposes. You can zoom in with the mouse wheel for a closer view, and the text will adjust automatically if necessary. For the full schema, download it here.