Plasma levels of von Willebrand factor regulate ADAMTS‐13, its major cleaving protease

Summary ADAMTS‐13, the metalloprotease that disposes physiologically of the most thrombogenic multimers of von Willebrand factor (VWF), tends to be low in plasma when VWF is high. We evaluated the behaviour of these two proteins in naturally occurring, experimental and clinical situations associated...

Ausführliche Beschreibung

Gespeichert in:
Bibliographische Detailangaben
Veröffentlicht in:British journal of haematology 2004-07, Vol.126 (2), p.213-218
Hauptverfasser: Mannucci, Pier Mannuccio, Capoferri, Cristina, Canciani, Maria Teresa
Format: Artikel
Sprache:eng
Schlagworte:
Online-Zugang:Volltext
Tags: Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
container_end_page 218
container_issue 2
container_start_page 213
container_title British journal of haematology
container_volume 126
creator Mannucci, Pier Mannuccio
Capoferri, Cristina
Canciani, Maria Teresa
description Summary ADAMTS‐13, the metalloprotease that disposes physiologically of the most thrombogenic multimers of von Willebrand factor (VWF), tends to be low in plasma when VWF is high. We evaluated the behaviour of these two proteins in naturally occurring, experimental and clinical situations associated with VWF levels spanning from undetectable to supranormal. ADAMTS‐13 was approximately 10% higher (and VWF 35% lower) in 65 healthy individuals of blood group O than in 65 individuals of groups A, B and AB. Thirty‐three patients with type 3 von Willebrand disease (VWD) with undetectable plasma VWF had approximately 35% higher levels of ADAMTS‐13 than a comparable group of healthy individuals with normal VWF. When VWF was raised to supranormal levels by desmopressin (DDAVP) in 10 healthy volunteers, ADAMTS‐13 decreased by approximately 20%, with no change of the protease in three patients with severe VWD who had no post‐DDAVP VWF rise. When VWF was raised from very low to normal levels by the infusion of VWF‐containing plasma concentrates in four patients with type 3 VWD and one with type 1 VWD plasma, ADAMTS‐13 decreased in parallel. These data show that throughout a large spectrum of plasma VWF levels there is a negative association between this protein and the activity of its major cleaving protease.
doi_str_mv 10.1111/j.1365-2141.2004.05009.x
format Article
fullrecord <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_66685450</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>668185781</sourcerecordid><originalsourceid>FETCH-LOGICAL-c4889-2fd4850c22361d2a36ffc62c17999b2f5885180868cc0bd249aa1c6e5407a1033</originalsourceid><addsrcrecordid>eNqNkM1u1DAQgC0EokvhFZCFRE8kzDh24hw4LC1QUBFIFMHN8jp2lchJip0s7Y1H6DP2SXDYFSBOWCN5pPnmRx8hFCHH9J53ORalyBhyzBkAz0EA1PnVHbL6XbhLVgBQZQhcHpAHMXYAWIDA--QABSskcrYiXz96HXtNvd1aH-no6HYc6JfWe7sJemio02YaAw32YvZ6snR9sn5__un2xw0Wz2g7RdrrLtWNt3rbDhf0MoyT1dE-JPec9tE-2v-H5PPrV-fHp9nZhzdvj9dnmeFS1hlzDZcCDGNFiQ3TRemcKZnBqq7rDXNCSoESZCmNgU3DeK01mtIKDpVGKIpDcrSbmxZ_m22cVN9GY73Xgx3nqMqylIILSOCTf8BunMOQblNYJ4RXVZUguYNMGGMM1qnL0PY6XCsEtahXnVoMq8WwWtSrX-rVVWp9vJ8_b3rb_Gncu07A0z2go9HeJb2mjX9xdYoKE_dix31vvb3-7wPUy3enS1b8BHYLnRI</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>198544777</pqid></control><display><type>article</type><title>Plasma levels of von Willebrand factor regulate ADAMTS‐13, its major cleaving protease</title><source>MEDLINE</source><source>Wiley Online Library Journals Frontfile Complete</source><source>Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals</source><source>Wiley Free Content</source><creator>Mannucci, Pier Mannuccio ; Capoferri, Cristina ; Canciani, Maria Teresa</creator><creatorcontrib>Mannucci, Pier Mannuccio ; Capoferri, Cristina ; Canciani, Maria Teresa</creatorcontrib><description>Summary ADAMTS‐13, the metalloprotease that disposes physiologically of the most thrombogenic multimers of von Willebrand factor (VWF), tends to be low in plasma when VWF is high. We evaluated the behaviour of these two proteins in naturally occurring, experimental and clinical situations associated with VWF levels spanning from undetectable to supranormal. ADAMTS‐13 was approximately 10% higher (and VWF 35% lower) in 65 healthy individuals of blood group O than in 65 individuals of groups A, B and AB. Thirty‐three patients with type 3 von Willebrand disease (VWD) with undetectable plasma VWF had approximately 35% higher levels of ADAMTS‐13 than a comparable group of healthy individuals with normal VWF. When VWF was raised to supranormal levels by desmopressin (DDAVP) in 10 healthy volunteers, ADAMTS‐13 decreased by approximately 20%, with no change of the protease in three patients with severe VWD who had no post‐DDAVP VWF rise. When VWF was raised from very low to normal levels by the infusion of VWF‐containing plasma concentrates in four patients with type 3 VWD and one with type 1 VWD plasma, ADAMTS‐13 decreased in parallel. These data show that throughout a large spectrum of plasma VWF levels there is a negative association between this protein and the activity of its major cleaving protease.</description><identifier>ISSN: 0007-1048</identifier><identifier>EISSN: 1365-2141</identifier><identifier>DOI: 10.1111/j.1365-2141.2004.05009.x</identifier><identifier>PMID: 15238142</identifier><identifier>CODEN: BJHEAL</identifier><language>eng</language><publisher>Oxford, UK: Blackwell Science Ltd</publisher><subject>ADAM Proteins ; ADAMTS13 Protein ; ADAMTS‐13 ; Adult ; Biological and medical sciences ; Blood Group Antigens ; Case-Control Studies ; Deamino Arginine Vasopressin ; desmopressin ; Factor VIII - therapeutic use ; factor VIII concentrate ; Female ; Hematologic and hematopoietic diseases ; Hematology ; Hemostatics ; Humans ; Male ; Medical sciences ; Metalloendopeptidases - blood ; Middle Aged ; Platelet diseases and coagulopathies ; Statistics, Nonparametric ; von Willebrand disease ; von Willebrand Diseases - blood ; von Willebrand Diseases - drug therapy ; von Willebrand factor ; von Willebrand Factor - analysis ; von Willebrand Factor - therapeutic use</subject><ispartof>British journal of haematology, 2004-07, Vol.126 (2), p.213-218</ispartof><rights>2004 INIST-CNRS</rights><rights>Copyright Blackwell Scientific Publications Ltd. Jul 2004</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4889-2fd4850c22361d2a36ffc62c17999b2f5885180868cc0bd249aa1c6e5407a1033</citedby><cites>FETCH-LOGICAL-c4889-2fd4850c22361d2a36ffc62c17999b2f5885180868cc0bd249aa1c6e5407a1033</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fj.1365-2141.2004.05009.x$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fj.1365-2141.2004.05009.x$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,1427,27903,27904,45553,45554,46387,46811</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&amp;idt=15915971$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/15238142$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Mannucci, Pier Mannuccio</creatorcontrib><creatorcontrib>Capoferri, Cristina</creatorcontrib><creatorcontrib>Canciani, Maria Teresa</creatorcontrib><title>Plasma levels of von Willebrand factor regulate ADAMTS‐13, its major cleaving protease</title><title>British journal of haematology</title><addtitle>Br J Haematol</addtitle><description>Summary ADAMTS‐13, the metalloprotease that disposes physiologically of the most thrombogenic multimers of von Willebrand factor (VWF), tends to be low in plasma when VWF is high. We evaluated the behaviour of these two proteins in naturally occurring, experimental and clinical situations associated with VWF levels spanning from undetectable to supranormal. ADAMTS‐13 was approximately 10% higher (and VWF 35% lower) in 65 healthy individuals of blood group O than in 65 individuals of groups A, B and AB. Thirty‐three patients with type 3 von Willebrand disease (VWD) with undetectable plasma VWF had approximately 35% higher levels of ADAMTS‐13 than a comparable group of healthy individuals with normal VWF. When VWF was raised to supranormal levels by desmopressin (DDAVP) in 10 healthy volunteers, ADAMTS‐13 decreased by approximately 20%, with no change of the protease in three patients with severe VWD who had no post‐DDAVP VWF rise. When VWF was raised from very low to normal levels by the infusion of VWF‐containing plasma concentrates in four patients with type 3 VWD and one with type 1 VWD plasma, ADAMTS‐13 decreased in parallel. These data show that throughout a large spectrum of plasma VWF levels there is a negative association between this protein and the activity of its major cleaving protease.</description><subject>ADAM Proteins</subject><subject>ADAMTS13 Protein</subject><subject>ADAMTS‐13</subject><subject>Adult</subject><subject>Biological and medical sciences</subject><subject>Blood Group Antigens</subject><subject>Case-Control Studies</subject><subject>Deamino Arginine Vasopressin</subject><subject>desmopressin</subject><subject>Factor VIII - therapeutic use</subject><subject>factor VIII concentrate</subject><subject>Female</subject><subject>Hematologic and hematopoietic diseases</subject><subject>Hematology</subject><subject>Hemostatics</subject><subject>Humans</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Metalloendopeptidases - blood</subject><subject>Middle Aged</subject><subject>Platelet diseases and coagulopathies</subject><subject>Statistics, Nonparametric</subject><subject>von Willebrand disease</subject><subject>von Willebrand Diseases - blood</subject><subject>von Willebrand Diseases - drug therapy</subject><subject>von Willebrand factor</subject><subject>von Willebrand Factor - analysis</subject><subject>von Willebrand Factor - therapeutic use</subject><issn>0007-1048</issn><issn>1365-2141</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2004</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkM1u1DAQgC0EokvhFZCFRE8kzDh24hw4LC1QUBFIFMHN8jp2lchJip0s7Y1H6DP2SXDYFSBOWCN5pPnmRx8hFCHH9J53ORalyBhyzBkAz0EA1PnVHbL6XbhLVgBQZQhcHpAHMXYAWIDA--QABSskcrYiXz96HXtNvd1aH-no6HYc6JfWe7sJemio02YaAw32YvZ6snR9sn5__un2xw0Wz2g7RdrrLtWNt3rbDhf0MoyT1dE-JPec9tE-2v-H5PPrV-fHp9nZhzdvj9dnmeFS1hlzDZcCDGNFiQ3TRemcKZnBqq7rDXNCSoESZCmNgU3DeK01mtIKDpVGKIpDcrSbmxZ_m22cVN9GY73Xgx3nqMqylIILSOCTf8BunMOQblNYJ4RXVZUguYNMGGMM1qnL0PY6XCsEtahXnVoMq8WwWtSrX-rVVWp9vJ8_b3rb_Gncu07A0z2go9HeJb2mjX9xdYoKE_dix31vvb3-7wPUy3enS1b8BHYLnRI</recordid><startdate>200407</startdate><enddate>200407</enddate><creator>Mannucci, Pier Mannuccio</creator><creator>Capoferri, Cristina</creator><creator>Canciani, Maria Teresa</creator><general>Blackwell Science Ltd</general><general>Blackwell</general><general>Blackwell Publishing Ltd</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>H94</scope><scope>7X8</scope></search><sort><creationdate>200407</creationdate><title>Plasma levels of von Willebrand factor regulate ADAMTS‐13, its major cleaving protease</title><author>Mannucci, Pier Mannuccio ; Capoferri, Cristina ; Canciani, Maria Teresa</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4889-2fd4850c22361d2a36ffc62c17999b2f5885180868cc0bd249aa1c6e5407a1033</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2004</creationdate><topic>ADAM Proteins</topic><topic>ADAMTS13 Protein</topic><topic>ADAMTS‐13</topic><topic>Adult</topic><topic>Biological and medical sciences</topic><topic>Blood Group Antigens</topic><topic>Case-Control Studies</topic><topic>Deamino Arginine Vasopressin</topic><topic>desmopressin</topic><topic>Factor VIII - therapeutic use</topic><topic>factor VIII concentrate</topic><topic>Female</topic><topic>Hematologic and hematopoietic diseases</topic><topic>Hematology</topic><topic>Hemostatics</topic><topic>Humans</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Metalloendopeptidases - blood</topic><topic>Middle Aged</topic><topic>Platelet diseases and coagulopathies</topic><topic>Statistics, Nonparametric</topic><topic>von Willebrand disease</topic><topic>von Willebrand Diseases - blood</topic><topic>von Willebrand Diseases - drug therapy</topic><topic>von Willebrand factor</topic><topic>von Willebrand Factor - analysis</topic><topic>von Willebrand Factor - therapeutic use</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Mannucci, Pier Mannuccio</creatorcontrib><creatorcontrib>Capoferri, Cristina</creatorcontrib><creatorcontrib>Canciani, Maria Teresa</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>British journal of haematology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Mannucci, Pier Mannuccio</au><au>Capoferri, Cristina</au><au>Canciani, Maria Teresa</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Plasma levels of von Willebrand factor regulate ADAMTS‐13, its major cleaving protease</atitle><jtitle>British journal of haematology</jtitle><addtitle>Br J Haematol</addtitle><date>2004-07</date><risdate>2004</risdate><volume>126</volume><issue>2</issue><spage>213</spage><epage>218</epage><pages>213-218</pages><issn>0007-1048</issn><eissn>1365-2141</eissn><coden>BJHEAL</coden><abstract>Summary ADAMTS‐13, the metalloprotease that disposes physiologically of the most thrombogenic multimers of von Willebrand factor (VWF), tends to be low in plasma when VWF is high. We evaluated the behaviour of these two proteins in naturally occurring, experimental and clinical situations associated with VWF levels spanning from undetectable to supranormal. ADAMTS‐13 was approximately 10% higher (and VWF 35% lower) in 65 healthy individuals of blood group O than in 65 individuals of groups A, B and AB. Thirty‐three patients with type 3 von Willebrand disease (VWD) with undetectable plasma VWF had approximately 35% higher levels of ADAMTS‐13 than a comparable group of healthy individuals with normal VWF. When VWF was raised to supranormal levels by desmopressin (DDAVP) in 10 healthy volunteers, ADAMTS‐13 decreased by approximately 20%, with no change of the protease in three patients with severe VWD who had no post‐DDAVP VWF rise. When VWF was raised from very low to normal levels by the infusion of VWF‐containing plasma concentrates in four patients with type 3 VWD and one with type 1 VWD plasma, ADAMTS‐13 decreased in parallel. These data show that throughout a large spectrum of plasma VWF levels there is a negative association between this protein and the activity of its major cleaving protease.</abstract><cop>Oxford, UK</cop><pub>Blackwell Science Ltd</pub><pmid>15238142</pmid><doi>10.1111/j.1365-2141.2004.05009.x</doi><tpages>6</tpages></addata></record>
fulltext fulltext
identifier ISSN: 0007-1048
ispartof British journal of haematology, 2004-07, Vol.126 (2), p.213-218
issn 0007-1048
1365-2141
language eng
recordid cdi_proquest_miscellaneous_66685450
source MEDLINE; Wiley Online Library Journals Frontfile Complete; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; Wiley Free Content
subjects ADAM Proteins
ADAMTS13 Protein
ADAMTS‐13
Adult
Biological and medical sciences
Blood Group Antigens
Case-Control Studies
Deamino Arginine Vasopressin
desmopressin
Factor VIII - therapeutic use
factor VIII concentrate
Female
Hematologic and hematopoietic diseases
Hematology
Hemostatics
Humans
Male
Medical sciences
Metalloendopeptidases - blood
Middle Aged
Platelet diseases and coagulopathies
Statistics, Nonparametric
von Willebrand disease
von Willebrand Diseases - blood
von Willebrand Diseases - drug therapy
von Willebrand factor
von Willebrand Factor - analysis
von Willebrand Factor - therapeutic use
title Plasma levels of von Willebrand factor regulate ADAMTS‐13, its major cleaving protease
url https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-23T20%3A56%3A59IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_cross&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Plasma%20levels%20of%20von%20Willebrand%20factor%20regulate%20ADAMTS%E2%80%9013,%20its%20major%20cleaving%20protease&rft.jtitle=British%20journal%20of%20haematology&rft.au=Mannucci,%20Pier%20Mannuccio&rft.date=2004-07&rft.volume=126&rft.issue=2&rft.spage=213&rft.epage=218&rft.pages=213-218&rft.issn=0007-1048&rft.eissn=1365-2141&rft.coden=BJHEAL&rft_id=info:doi/10.1111/j.1365-2141.2004.05009.x&rft_dat=%3Cproquest_cross%3E668185781%3C/proquest_cross%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=198544777&rft_id=info:pmid/15238142&rfr_iscdi=true