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...
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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. |
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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&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> |
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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 |
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