A disintegrin and metalloproteinase with thrombospondin motifs 13
Details
- Name
- A disintegrin and metalloproteinase with thrombospondin motifs 13
- Kind
- protein
- Synonyms
- 3.4.24.87
- ADAM-TS 13
- ADAM-TS13
- ADAMTS-13
- C9orf8
- von Willebrand factor-cleaving protease
- vWF-cleaving protease
- vWF-CP
- Gene Name
- ADAMTS13
- UniProtKB Entry
- Q76LX8Swiss-Prot
- Organism
- Humans
- NCBI Taxonomy ID
- 9606
- Amino acid sequence
>lcl|BSEQ0051592|A disintegrin and metalloproteinase with thrombospondin motifs 13 MHQRHPRARCPPLCVAGILACGFLLGCWGPSHFQQSCLQALEPQAVSSYLSPGAPLKGRP PSPGFQRQRQRQRRAAGGILHLELLVAVGPDVFQAHQEDTERYVLTNLNIGAELLRDPSL GAQFRVHLVKMVILTEPEGAPNITANLTSSLLSVCGWSQTINPEDDTDPGHADLVLYITR FDLELPDGNRQVRGVTQLGGACSPTWSCLITEDTGFDLGVTIAHEIGHSFGLEHDGAPGS GCGPSGHVMASDGAAPRAGLAWSPCSRRQLLSLLSAGRARCVWDPPRPQPGSAGHPPDAQ PGLYYSANEQCRVAFGPKAVACTFAREHLDMCQALSCHTDPLDQSSCSRLLVPLLDGTEC GVEKWCSKGRCRSLVELTPIAAVHGRWSSWGPRSPCSRSCGGGVVTRRRQCNNPRPAFGG RACVGADLQAEMCNTQACEKTQLEFMSQQCARTDGQPLRSSPGGASFYHWGAAVPHSQGD ALCRHMCRAIGESFIMKRGDSFLDGTRCMPSGPREDGTLSLCVSGSCRTFGCDGRMDSQQ VWDRCQVCGGDNSTCSPRKGSFTAGRAREYVTFLTVTPNLTSVYIANHRPLFTHLAVRIG GRYVVAGKMSISPNTTYPSLLEDGRVEYRVALTEDRLPRLEEIRIWGPLQEDADIQVYRR YGEEYGNLTRPDITFTYFQPKPRQAWVWAAVRGPCSVSCGAGLRWVNYSCLDQARKELVE TVQCQGSQQPPAWPEACVLEPCPPYWAVGDFGPCSASCGGGLRERPVRCVEAQGSLLKTL PPARCRAGAQQPAVALETCNPQPCPARWEVSEPSSCTSAGGAGLALENETCVPGADGLEA PVTEGPGSVDEKLPAPEPCVGMSCPPGWGHLDATSAGEKAPSPWGSIRTGAQAAHVWTPA AGSCSVSCGRGLMELRFLCMDSALRVPVQEELCGLASKPGSRREVCQAVPCPARWQYKLA ACSVSCGRGVVRRILYCARAHGEDDGEEILLDTQCQGLPRPEPQEACSLEPCPPRWKVMS LGPCSASCGLGTARRSVACVQLDQGQDVEVDEAACAALVRPEASVPCLIADCTYRWHVGT WMECSVSCGDGIQRRRDTCLGPQAQAPVPADFCQHLPKPVTVRGCWAGPCVGQGTPSLVP HEEAAAPGRTTATPAGASLEWSQARGLLFSPAPQPRRLLPGPQENSVQSSACGRQHLEPT GTIDMRGPGQADCAVAIGRPLGEVVTLRVLESSLNCSAGDMLLLWGRLTWRKMCRKLLDM TFSSKTNTLVVRQRCGRPGGGVLLRYGSQLAPETFYRECDMQLFGPWGEIVSPSLSPATS NAGGCRLFINVAPHARIAIHALATNMGAGTEGANASYILIRDTHSLRTTAFHGQQVLYWE SESSQAEMEFSEGFLKAQASLRGQYWTLQSWVPEMQDPQSWKGKEGT
- Number of residues
- 1427
- Molecular Weight
- 153603.05
- Theoretical pI
- Not Available
- GO Classification
- Processesblood coagulation / cellular response to type II interferon / extracellular matrix organization / protein catabolic processComponentsextracellular matrix / extracellular region
- General Function
- Cleaves the vWF multimers in plasma into smaller forms thereby controlling vWF-mediated platelet thrombus formation
- Specific Function
- calcium ion binding
- Pfam Domain Function
- Signal Regions
- 1-29
- Transmembrane Regions
- Not Available
- Cellular Location
- Secreted
- Gene sequence
>lcl|BSEQ0051593|A disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAMTS13) ATGCACCAGCGTCACCCCCGGGCAAGATGCCCTCCCCTCTGTGTGGCCGGAATCCTTGCC TGTGGCTTTCTCCTGGGCTGCTGGGGACCCTCCCATTTCCAGCAGAGTTGTCTTCAGGCT TTGGAGCCACAGGCCGTGTCTTCTTACTTGAGCCCTGGTGCTCCCTTAAAAGGCCGCCCT CCTTCCCCTGGCTTCCAGAGGCAGAGGCAGAGGCAGAGGCGGGCTGCAGGCGGCATCCTA CACCTGGAGCTGCTGGTGGCCGTGGGCCCCGATGTCTTCCAGGCTCACCAGGAGGACACA GAGCGCTATGTGCTCACCAACCTCAACATCGGGGCAGAACTGCTTCGGGACCCGTCCCTG GGGGCTCAGTTTCGGGTGCACCTGGTGAAGATGGTCATTCTGACAGAGCCTGAGGGTGCT CCAAATATCACAGCCAACCTCACCTCGTCCCTGCTGAGCGTCTGTGGGTGGAGCCAGACC ATCAACCCTGAGGACGACACGGATCCTGGCCATGCTGACCTGGTCCTCTATATCACTAGG TTTGACCTGGAGTTGCCTGATGGTAACCGGCAGGTGCGGGGCGTCACCCAGCTGGGCGGT GCCTGCTCCCCAACCTGGAGCTGCCTCATTACCGAGGACACTGGCTTCGACCTGGGAGTC ACCATTGCCCATGAGATTGGGCACAGCTTCGGCCTGGAGCACGACGGCGCGCCCGGCAGC GGCTGCGGCCCCAGCGGACACGTGATGGCTTCGGACGGCGCCGCGCCCCGCGCCGGCCTC GCCTGGTCCCCCTGCAGCCGCCGGCAGCTGCTGAGCCTGCTCAGCGCAGGACGGGCGCGC TGCGTGTGGGACCCGCCGCGGCCTCAACCCGGGTCCGCGGGGCACCCGCCGGATGCGCAG CCTGGCCTCTACTACAGCGCCAACGAGCAGTGCCGCGTGGCCTTCGGCCCCAAGGCTGTC GCCTGCACCTTCGCCAGGGAGCACCTGGATATGTGCCAGGCCCTCTCCTGCCACACAGAC CCGCTGGACCAAAGCAGCTGCAGCCGCCTCCTCGTTCCTCTCCTGGATGGGACAGAATGT GGCGTGGAGAAGTGGTGCTCCAAGGGTCGCTGCCGCTCCCTGGTGGAGCTGACCCCCATA GCAGCAGTGCATGGGCGCTGGTCTAGCTGGGGTCCCCGAAGTCCTTGCTCCCGCTCCTGC GGAGGAGGTGTGGTCACCAGGAGGCGGCAGTGCAACAACCCCAGACCTGCCTTTGGGGGG CGTGCATGTGTTGGTGCTGACCTCCAGGCCGAGATGTGCAACACTCAGGCCTGCGAGAAG ACCCAGCTGGAGTTCATGTCGCAACAGTGCGCCAGGACCGACGGCCAGCCGCTGCGCTCC TCCCCTGGCGGCGCCTCCTTCTACCACTGGGGTGCTGCTGTACCACACAGCCAAGGGGAT GCTCTGTGCAGACACATGTGCCGGGCCATTGGCGAGAGCTTCATCATGAAGCGTGGAGAC AGCTTCCTCGATGGGACCCGGTGTATGCCAAGTGGCCCCCGGGAGGACGGGACCCTGAGC CTGTGTGTGTCGGGCAGCTGCAGGACATTTGGCTGTGATGGTAGGATGGACTCCCAGCAG GTATGGGACAGGTGCCAGGTGTGTGGTGGGGACAACAGCACGTGCAGCCCACGGAAGGGC TCTTTCACAGCTGGCAGAGCGAGAGAATATGTCACGTTTCTGACAGTTACCCCCAACCTG ACCAGTGTCTACATTGCCAACCACAGGCCTCTCTTCACACACTTGGCGGTGAGGATCGGA GGGCGCTATGTCGTGGCTGGGAAGATGAGCATCTCCCCTAACACCACCTACCCCTCCCTC CTGGAGGATGGTCGTGTCGAGTACAGAGTGGCCCTCACCGAGGACCGGCTGCCCCGCCTG GAGGAGATCCGCATCTGGGGACCCCTCCAGGAAGATGCTGACATCCAGGTTTACAGGCGG TATGGCGAGGAGTATGGCAACCTCACCCGCCCAGACATCACCTTCACCTACTTCCAGCCT AAGCCACGGCAGGCCTGGGTGTGGGCCGCTGTGCGTGGGCCCTGCTCGGTGAGCTGTGGG GCAGGGCTGCGCTGGGTAAACTACAGCTGCCTGGACCAGGCCAGGAAGGAGTTGGTGGAG ACTGTCCAGTGCCAAGGGAGCCAGCAGCCACCAGCGTGGCCAGAGGCCTGCGTGCTCGAA CCCTGCCCTCCCTACTGGGCGGTGGGAGACTTCGGCCCATGCAGCGCCTCCTGTGGGGGT GGCCTGCGGGAGCGGCCAGTGCGCTGCGTGGAGGCCCAGGGCAGCCTCCTGAAGACATTG CCCCCAGCCCGGTGCAGAGCAGGGGCCCAGCAGCCAGCTGTGGCGCTGGAAACCTGCAAC CCCCAGCCCTGCCCTGCCAGGTGGGAGGTGTCAGAGCCCAGCTCATGCACATCAGCTGGT GGAGCAGGCCTGGCCTTGGAGAACGAGACCTGTGTGCCAGGGGCAGATGGCCTGGAGGCT CCAGTGACTGAGGGGCCTGGCTCCGTAGATGAGAAGCTGCCTGCCCCTGAGCCCTGTGTC GGGATGTCATGTCCTCCAGGCTGGGGCCATCTGGATGCCACCTCTGCAGGGGAGAAGGCT CCCTCCCCATGGGGCAGCATCAGGACGGGGGCTCAAGCTGCACACGTGTGGACCCCTGCG GCAGGGTCGTGCTCCGTCTCCTGCGGGCGAGGTCTGATGGAGCTGCGTTTCCTGTGCATG GACTCTGCCCTCAGGGTGCCTGTCCAGGAAGAGCTGTGTGGCCTGGCAAGCAAGCCTGGG AGCCGGCGGGAGGTCTGCCAGGCTGTCCCGTGCCCTGCTCGGTGGCAGTACAAGCTGGCG GCCTGCAGCGTGAGCTGTGGGAGAGGGGTCGTGCGGAGGATCCTGTATTGTGCCCGGGCC CATGGGGAGGACGATGGTGAGGAGATCCTGTTGGACACCCAGTGCCAGGGGCTGCCTCGC CCGGAACCCCAGGAGGCCTGCAGCCTGGAGCCCTGCCCACCTAGGTGGAAAGTCATGTCC CTTGGCCCATGTTCGGCCAGCTGTGGCCTTGGCACTGCTAGACGCTCGGTGGCCTGTGTG CAGCTCGACCAAGGCCAGGACGTGGAGGTGGACGAGGCGGCCTGTGCGGCGCTGGTGCGG CCCGAGGCCAGTGTCCCCTGTCTCATTGCCGACTGCACCTACCGCTGGCATGTTGGCACC TGGATGGAGTGCTCTGTTTCCTGTGGGGATGGCATCCAGCGCCGGCGTGACACCTGCCTC GGACCCCAGGCCCAGGCGCCTGTGCCAGCTGATTTCTGCCAGCACTTGCCCAAGCCGGTG ACTGTGCGTGGCTGCTGGGCTGGGCCCTGTGTGGGACAGGGTACGCCCAGCCTGGTGCCC CACGAAGAAGCCGCTGCTCCAGGACGGACCACAGCCACCCCTGCTGGTGCCTCCCTGGAG TGGTCCCAGGCCCGGGGCCTGCTCTTCTCCCCGGCTCCCCAGCCTCGGCGGCTCCTGCCC GGGCCCCAGGAAAACTCAGTGCAGTCCAGTGCCTGTGGCAGGCAGCACCTTGAGCCAACA GGAACCATTGACATGCGAGGCCCAGGGCAGGCAGACTGTGCAGTGGCCATTGGGCGGCCC CTCGGGGAGGTGGTGACCCTCCGCGTCCTTGAGAGTTCTCTCAACTGCAGTGCGGGGGAC ATGTTGCTGCTTTGGGGCCGGCTCACCTGGAGGAAGATGTGCAGGAAGCTGTTGGACATG ACTTTCAGCTCCAAGACCAACACGCTGGTGGTGAGGCAGCGCTGCGGGCGGCCAGGAGGT GGGGTGCTGCTGCGGTATGGGAGCCAGCTTGCTCCTGAAACCTTCTACAGAGAATGTGAC ATGCAGCTCTTTGGGCCCTGGGGTGAAATCGTGAGCCCCTCGCTGAGTCCAGCCACGAGT AATGCAGGGGGCTGCCGGCTCTTCATTAATGTGGCTCCGCACGCACGGATTGCCATCCAT GCCCTGGCCACCAACATGGGCGCTGGGACCGAGGGAGCCAATGCCAGCTACATCTTGATC CGGGACACCCACAGCTTGAGGACCACAGCGTTCCATGGGCAGCAGGTGCTCTACTGGGAG TCAGAGAGCAGCCAGGCTGAGATGGAGTTCAGCGAGGGCTTCCTGAAGGCTCAGGCCAGC CTGCGGGGCCAGTACTGGACCCTCCAATCATGGGTACCGGAGATGCAGGACCCTCAGTCC TGGAAGGGAAAGGAAGGAACCTGA
- Chromosome Location
- 9
- Locus
- 9q34.2
- External Identifiers
Resource Link UniProtKB ID Q76LX8 UniProtKB Entry Name ATS13_HUMAN GeneCard ID ADAMTS13 HGNC ID HGNC:1366 PDB ID(s) 3GHM, 3GHN, 3VN4, 6QIG, 7B01 KEGG ID hsa:11093 NCBI Gene ID 11093 - General References
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- Meyer SC, Jeddi R, Meddeb B, Gouider E, Lammle B, Kremer Hovinga JA: A first case of congenital TTP on the African continent due to a new homozygous mutation in the catalytic domain of ADAMTS13. Ann Hematol. 2008 Aug;87(8):663-6. doi: 10.1007/s00277-008-0496-6. Epub 2008 Apr 29. [Article]
- 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 JN: 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. 2009 Mar;144(5):742-54. doi: 10.1111/j.1365-2141.2008.07515.x. Epub 2008 Nov 26. [Article]
- 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. 2009 Feb;94(2):289-93. doi: 10.3324/haematol.13524. Epub 2008 Dec 30. [Article]
- Lee SH, Park JH, Park SK, Lee EH, Choi JI, Visentin GP, Park TS, Oh SH, Kim SR: A novel homozygous missense ADAMTS13 mutation Y658C in a patient with recurrent thrombotic thrombocytopenic purpura. Ann Clin Lab Sci. 2011 Summer;41(3):273-6. [Article]
- Choi HS, Cheong HI, Kim NK, Oh D, Park HW: ADAMTS13 gene mutations in children with hemolytic uremic syndrome. Yonsei Med J. 2011 May;52(3):530-4. doi: 10.3349/ymj.2011.52.3.530. [Article]
Associated Data
- Drug Relations
Drug Drug group Pharmacological action? Type Actions Details Vonicog alfa approved, investigational no enzyme substrate Details Von Willebrand factor human approved, investigational no enzyme substrate Details