{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T20:12:29Z","timestamp":1790626349738,"version":"4.1.0"},"reference-count":43,"publisher":"Public Library of Science (PLoS)","issue":"9","license":[{"start":{"date-parts":[[2011,9,1]],"date-time":"2011-09-01T00:00:00Z","timestamp":1314835200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0024216","type":"journal-article","created":{"date-parts":[[2011,9,1]],"date-time":"2011-09-01T21:47:51Z","timestamp":1314913671000},"page":"e24216","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":26,"title":["Cleavage of von Willebrand Factor by Granzyme M Destroys Its Factor VIII Binding Capacity"],"prefix":"10.1371","volume":"6","author":[{"given":"Martine J.","family":"Hollestelle","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ka Wai","family":"Lai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcel","family":"van Deuren","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter J.","family":"Lenting","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip G.","family":"de Groot","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tom","family":"Sprong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Niels","family":"Bovenschen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2011,9,1]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"576","DOI":"10.1055\/s-0037-1615883","article-title":"Structure and function of von Willebrand factor.","volume":"82","author":"ZM Ruggeri","year":"1999","journal-title":"Thromb Haemost"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"390","DOI":"10.1172\/JCI108788","article-title":"Stabilization of factor VIII in plasma by the von Willebrand factor. Studies on posttransfusion and dissociated factor VIII and in patients with von Willebrand&apos;s disease.","volume":"60","author":"HJ Weiss","year":"1977","journal-title":"J Clin Invest"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"1335","DOI":"10.1046\/j.1538-7836.2003.00260.x","article-title":"Von Willebrand factor, platelets and endothelial cell interactions.","volume":"1","author":"ZM Ruggeri","year":"2003","journal-title":"J Thromb Haemost"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"1713","DOI":"10.1182\/blood-2008-04-148759","article-title":"Extensive contacts between ADAMTS13 exosites and von Willebrand factor domain A2 contribute to substrate specificity.","volume":"112","author":"W Gao","year":"2008","journal-title":"Blood"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"488","DOI":"10.1038\/35097008","article-title":"Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura.","volume":"413","author":"GG Levy","year":"2001","journal-title":"Nature"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"1578","DOI":"10.1056\/NEJM199811263392202","article-title":"von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome.","volume":"339","author":"M Furlan","year":"1998","journal-title":"N Engl J Med"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"962","DOI":"10.1111\/j.1538-7836.2009.03433.x","article-title":"The active conformation of von Willebrand factor in patients with thrombotic thrombocytopenic purpura in remission.","volume":"7","author":"E Groot","year":"2009","journal-title":"J Thromb Haemost"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"1901","DOI":"10.1161\/ATVBAHA.107.145797","article-title":"Thrombotic thrombocytopenic purpura in humans and mice.","volume":"27","author":"KC Desch","year":"2007","journal-title":"Arterioscler Thromb Vasc Biol"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"978","DOI":"10.1182\/blood.V74.3.978.bloodjournal743978","article-title":"Enhanced proteolysis of plasma von Willebrand factor in thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome.","volume":"74","author":"PM Mannucci","year":"1989","journal-title":"Blood"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"1666","DOI":"10.1182\/blood-2009-01-195461","article-title":"Leukocyte proteases cleave von Willebrand factor at or near the ADAMTS13 cleavage site.","volume":"114","author":"TJ Raife","year":"2009","journal-title":"Blood"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"980","DOI":"10.1016\/0006-291X(89)92818-0","article-title":"Endothelial cell-derived high molecular weight von Willebrand factor is converted into the plasma multimer pattern by granulocyte proteases.","volume":"158","author":"HM Tsai","year":"1989","journal-title":"Biochem Biophys Res Commun"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"22498","DOI":"10.1074\/jbc.M709080200","article-title":"Antihemostatic activity of human granzyme B mediated by cleavage of von Willebrand factor.","volume":"283","author":"MS Buzza","year":"2008","journal-title":"J Biol Chem"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1146\/annurev.immunol.26.021607.090404","article-title":"Death by a thousand cuts: granzyme pathways of programmed cell death.","volume":"26:389\u2013420.","author":"D Chowdhury","year":"2008","journal-title":"Annu Rev Immunol"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"827","DOI":"10.1515\/BC.2006.106","article-title":"Extracellular granzymes: current perspectives.","volume":"387","author":"MS Buzza","year":"2006","journal-title":"Biol Chem"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"2940","DOI":"10.1002\/eji.200526249","article-title":"Detection of soluble human granzyme K in vitro and in vivo.","volume":"35","author":"B Bade","year":"2005","journal-title":"Eur J Immunol"},{"key":"ref16","article-title":"Von Willebrand factor activation, granzyme-B and thrombocytopenia in meningococcal disease.","author":"MJ Hollestelle","year":"2010","journal-title":"J Thromb Haemostasis"},{"key":"ref17","first-page":"89","article-title":"The role of Granzyme B in atheromatous diseases.","volume":"85","author":"CM Chamberlain","year":"2007","journal-title":"Can J Physiol Pharmacol"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"460","DOI":"10.1097\/01.MP.0000067424.12280.BC","article-title":"Granzyme B in atherosclerosis and transplant vascular disease: association with cell death and atherosclerotic disease severity.","volume":"16","author":"JC Choy","year":"2003","journal-title":"Mod Pathol"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"5538","DOI":"10.1016\/S0021-9258(19)63384-3","article-title":"Relationship of sialic acid to function and in vivo survival of human factor VIII\/von Willebrand factor protein.","volume":"252","author":"JM Sodetz","year":"1977","journal-title":"J Biol Chem"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"8184","DOI":"10.4049\/jimmunol.180.12.8184","article-title":"NK cell protease granzyme M targets alpha-tubulin and disorganizes the microtubule network.","volume":"180","author":"N Bovenschen","year":"2008","journal-title":"J Immunol"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"3504","DOI":"10.1074\/jbc.M806716200","article-title":"Granzyme K displays highly restricted substrate specificity that only partially overlaps with granzyme A.","volume":"284","author":"N Bovenschen","year":"2009","journal-title":"J Biol Chem"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"3035","DOI":"10.1182\/blood-2005-03-1153","article-title":"A novel nanobody that detects the gain-of-function phenotype of von Willebrand factor in ADAMTS13 deficiency and von Willebrand disease type 2B.","volume":"106","author":"JJ Hulstein","year":"2005","journal-title":"Blood"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"3227","DOI":"10.1182\/blood-2008-02-139113","article-title":"The glycoprotein Ib-IX-V complex contributes to tissue factor-independent thrombin generation by recombinant factor VIIa on the activated platelet surface.","volume":"112","author":"C Weeterings","year":"2008","journal-title":"Blood"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"1005","DOI":"10.1055\/s-0037-1614163","article-title":"Binding of von Willebrand factor to collagen type III: role of specific amino acids in the collagen binding domain of vWF and effects of neighboring domains.","volume":"84","author":"RM van der Plas","year":"2000","journal-title":"Thromb Haemost"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1097\/01.PCC.0000149131.72248.E6","article-title":"International pediatric sepsis consensus conference: definitions for sepsis and organ dysfunction in pediatrics.","volume":"6","author":"B Goldstein","year":"2005","journal-title":"Pediatr Crit Care Med"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"903","DOI":"10.1016\/j.molimm.2009.10.001","article-title":"The cytotoxic protease granzyme M is expressed by lymphocytes of both the innate and adaptive immune system.","volume":"47","author":"PJ de Koning","year":"2010","journal-title":"Mol Immunol"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"1710","DOI":"10.1111\/j.1538-7836.2005.01360.x","article-title":"Cleavage of ultra-large von Willebrand factor by ADAMTS-13 under flow conditions.","volume":"3","author":"JF Dong","year":"2005","journal-title":"J Thromb Haemost"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"4223","DOI":"10.1182\/blood.V87.10.4223.bloodjournal87104223","article-title":"Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis.","volume":"87","author":"M Furlan","year":"1996","journal-title":"Blood"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"4235","DOI":"10.1182\/blood.V87.10.4235.bloodjournal87104235","article-title":"Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion.","volume":"87","author":"HM Tsai","year":"1996","journal-title":"Blood"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"23295","DOI":"10.1074\/jbc.M501143200","article-title":"Role of chloride ions in modulation of the interaction between von Willebrand factor and ADAMTS-13.","volume":"280","author":"CR De","year":"2005","journal-title":"J Biol Chem"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1097\/MOH.0b013e3280dce531","article-title":"The presence of active von Willebrand factor under various pathological conditions.","volume":"14","author":"E Groot","year":"2007","journal-title":"Curr Opin Hematol"},{"key":"ref32","doi-asserted-by":"crossref","first-page":"3610","DOI":"10.4049\/jimmunol.160.7.3610","article-title":"Extracellular granzymes A and B in humans: detection of native species during CTL responses in vitro and in vivo.","volume":"160","author":"EH Spaeny-Dekking","year":"1998","journal-title":"J Immunol"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1016\/j.it.2009.01.002","article-title":"Granule-associated serine proteases: granzymes might not just be killer proteases.","volume":"30","author":"CJ Froelich","year":"2009","journal-title":"Trends Immunol"},{"key":"ref34","doi-asserted-by":"crossref","first-page":"720","DOI":"10.1016\/j.immuni.2008.08.014","article-title":"Human and mouse granzyme A induce a proinflammatory cytokine response.","volume":"29","author":"SS Metkar","year":"2008","journal-title":"Immunity"},{"key":"ref35","doi-asserted-by":"crossref","first-page":"11552","DOI":"10.1073\/pnas.192448999","article-title":"A new name in thrombosis, ADAMTS13.","volume":"99","author":"JE Sadler","year":"2002","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref36","doi-asserted-by":"crossref","first-page":"54275","DOI":"10.1074\/jbc.M411482200","article-title":"Granzyme M is a regulatory protease that inactivates proteinase inhibitor 9, an endogenous inhibitor of granzyme B.","volume":"279","author":"S Mahrus","year":"2004","journal-title":"J Biol Chem"},{"key":"ref37","doi-asserted-by":"crossref","first-page":"4699","DOI":"10.1074\/jbc.M513314200","article-title":"Shielding of the A1 domain by the D&apos;D3 domains of von Willebrand factor modulates its interaction with platelet glycoprotein Ib-IX-V.","volume":"281","author":"H Ulrichts","year":"2006","journal-title":"J Biol Chem"},{"key":"ref38","doi-asserted-by":"crossref","first-page":"4663","DOI":"10.1182\/blood.V92.12.4663","article-title":"A novel mutation in the D3 domain of von Willebrand factor markedly decreases its ability to bind factor VIII and affects its multimerization.","volume":"92","author":"S Jorieux","year":"1998","journal-title":"Blood"},{"key":"ref39","doi-asserted-by":"crossref","first-page":"627","DOI":"10.1046\/j.1365-2141.2003.04163.x","article-title":"Two novel mutations, Q1053H and C1060R, located in the D3 domain of von Willebrand factor, are responsible for decreased FVIII-binding capacity.","volume":"120","author":"L Hilbert","year":"2003","journal-title":"Br J Haematol"},{"key":"ref40","doi-asserted-by":"crossref","first-page":"3161","DOI":"10.1182\/blood-2005-07-2765","article-title":"Complete deficiency in ADAMTS13 is prothrombotic, but it alone is not sufficient to cause thrombotic thrombocytopenic purpura.","volume":"107","author":"F Banno","year":"2006","journal-title":"Blood"},{"key":"ref41","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1083\/jcb.200606073","article-title":"The major human and mouse granzymes are structurally and functionally divergent.","volume":"175","author":"D Kaiserman","year":"2006","journal-title":"J Cell Biol %20"},{"key":"ref42","doi-asserted-by":"crossref","first-page":"435","DOI":"10.1083\/jcb.200612025","article-title":"Human and murine granzyme B exhibit divergent substrate preferences.","volume":"176","author":"SP Cullen","year":"2007","journal-title":"J Cell Biol"},{"key":"ref43","doi-asserted-by":"crossref","first-page":"1239","DOI":"10.1007\/s00134-005-2720-3","article-title":"Elastase and granzymes during meningococcal disease in children: correlation to disease severity.","volume":"31","author":"JB van Woensel","year":"2005","journal-title":"Intensive Care Med"}],"container-title":["PLoS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0024216","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,4,10]],"date-time":"2024-04-10T22:01:58Z","timestamp":1712786518000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0024216"}},"subtitle":[],"editor":[{"given":"Christopher B.","family":"Doering","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2011,9,1]]},"references-count":43,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2011,9,1]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0024216","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2011,9,1]]}}}