Von Willebrand Factor Cleaving Protease (vWFCP)

ADAMTS13; TTP; vWF-cp; vWF-cleaving protease; A Disintegrin And Metalloproteinase With A Thrombospondin Type 1 Motif Member 13

SPN	V861
HGNC	1366
MGI	2685556
PubMed	11557746
RGD	1311594
UniProt	Q76LX8
Wiki	VWFCP

ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13)—also known as von Willebrand factor-cleaving protease (VWFCP)—is a zinc-containing metalloprotease enzyme that cleaves von Willebrand factor (vWf), a large protein involved in blood clotting. It is secreted in blood and degrades large vWf multimers, decreasing their activity.Genomically, ADAMTS13 shares many properties with the 19 member ADAMTS family, all of which are characterised by a protease domain (the part that performs the protein hydrolysis), an adjacent disintegrin domain and one or more thrombospondin domains. ADAMTS13 in fact has eight thrombospondin domains. It has no hydrophobic transmembrane domain, and hence it not anchored in the cell membrane.

Reference

"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) [PubMed] [Europe PMC] [Abstract]

"Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura."
J. Biol. Chem. 276:41059-41063(2001) [PubMed] [Europe PMC] [Abstract]

"Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura."
Nature 413:488-494(2001) [PubMed] [Europe PMC] [Abstract]

"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) [PubMed] [Europe PMC] [Abstract]

"DNA sequence and analysis of human chromosome 9."
Nature 429:369-374(2004) [PubMed] [Europe PMC] [Abstract]

"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) [PubMed] [Europe PMC] [Abstract]

"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) [PubMed] [Europe PMC] [Abstract]

"Partial amino acid sequence of purified von Willebrand factor-cleaving protease."
Blood 98:1654-1661(2001) [PubMed] [Europe PMC] [Abstract]

"Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family."
Blood 98:1662-1666(2001) [PubMed] [Europe PMC] [Abstract]

"Cleavage of von Willebrand factor requires the spacer domain of the metalloprotease ADAMTS13."
J. Biol. Chem. 278:30136-30141(2003) [PubMed] [Europe PMC] [Abstract]

"Cleavage of the ADAMTS13 propeptide is not required for protease activity."
J. Biol. Chem. 278:46643-46648(2003) [PubMed] [Europe PMC] [Abstract]

"Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry."
J. Proteome Res. 4:2070-2080(2005) [PubMed] [Europe PMC] [Abstract]

"Zinc and calcium ions cooperatively modulate ADAMTS13 activity."
J. Biol. Chem. 281:850-857(2006) [PubMed] [Europe PMC] [Abstract]

"O-fucosylation is required for ADAMTS13 secretion."
J. Biol. Chem. 282:17014-17023(2007) [PubMed] [Europe PMC] [Abstract]

"A functional calcium-binding site in the metalloprotease domain of ADAMTS13."
Blood 113:1149-1157(2009) [PubMed] [Europe PMC] [Abstract]

"A strategy for precise and large scale identification of core fucosylated glycoproteins."
Mol. Cell. Proteomics 8:913-923(2009) [PubMed] [Europe PMC] [Abstract]

"ADAMTS13 mutations and polymorphisms in congenital thrombotic thrombocytopenic purpura."
Hum. Mutat. 31:11-19(2010) [PubMed] [Europe PMC] [Abstract]

"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) [PubMed] [Europe PMC] [Abstract]

"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) [PubMed] [Europe PMC] [Abstract]

"von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP."
Blood 101:1845-1850(2003) [PubMed] [Europe PMC] [Abstract]

"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) [PubMed] [Europe PMC] [Abstract]

"Mutation analysis and clinical implications of von Willebrand factor-cleaving protease deficiency."
Kidney Int. 63:1995-1999(2003) [PubMed] [Europe PMC] [Abstract]

"Congenital thrombotic thrombocytopenic purpura in association with a mutation in the second CUB domain of ADAMTS13."
Blood 103:627-629(2004) [PubMed] [Europe PMC] [Abstract]

"Molecular characterization of ADAMTS13 gene mutations in Japanese patients with Upshaw-Schulman syndrome."
Blood 103:1305-1310(2004) [PubMed] [Europe PMC] [Abstract]

"Identification of novel mutations in ADAMTS13 in an adult patient with congenital thrombotic thrombocytopenic purpura."
Blood 104:2081-2083(2004) [PubMed] [Europe PMC] [Abstract]

"Ten candidate ADAMTS13 mutations in six French families with congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome)."
J. Thromb. Haemost. 2:424-429(2004) [PubMed] [Europe PMC] [Abstract]

"Two novel ADAMTS13 gene mutations in thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome (TTP/HUS)."
Kidney Int. 66:955-958(2004) [PubMed] [Europe PMC] [Abstract]

"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) [PubMed] [Europe PMC] [Abstract]

"Modulation of ADAMTS13 secretion and specific activity by a combination of common amino acid polymorphisms and a missense mutation."
Blood 107:118-125(2006) [PubMed] [Europe PMC] [Abstract]

"Mechanisms of the interaction between two ADAMTS13 gene mutations leading to severe deficiency of enzymatic activity."
Hum. Mutat. 27:330-336(2006) [PubMed] [Europe PMC] [Abstract]

"Novel ADAMTS-13 mutations in an adult with delayed onset thrombotic thrombocytopenic purpura."
J. Thromb. Haemost. 4:1931-1935(2006) [PubMed] [Europe PMC] [Abstract]

"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) [PubMed] [Europe PMC] [Abstract]

"A common origin of the 4143insA ADAMTS13 mutation."
Thromb. Haemost. 96:3-6(2006) [PubMed] [Europe PMC] [Abstract]

"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) [PubMed] [Europe PMC] [Abstract]

"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) [PubMed] [Europe PMC] [Abstract]

"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) [PubMed] [Europe PMC] [Abstract]

"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) [PubMed] [Europe PMC] [Abstract]

"A novel homozygous missense ADAMTS13 mutation Y658C in a patient with recurrent thrombotic thrombocytopenic purpura."
Ann. Clin. Lab. Sci. 41:273-276(2011) [PubMed] [Europe PMC] [Abstract]

"ADAMTS13 gene mutations in children with hemolytic uremic syndrome."
Yonsei Med. J. 52:530-534(2011) [PubMed] [Europe PMC] [Abstract]