LP32458-9
von Willebrand factor cleaving protease
Active
Description
von Willebrand factor (vWF) cleaving protease, or ADAMTS13, is a metalloprotease that cleaves vWF and thereby limits platelet aggregation and formation of microthrombi. vWF cleaving protease inhibitors can decrease the protease activity and/or more rapidly remove the enzyme from the circulation. Such inhibitors are one cause of thrombotic thrombocytopenic purpura (TTP), a disorder characterized by thrombocytopenia, anemia, fever, and neurologic and renal abnormalities. Idiopathic TTP is often due to anti-ADAMTS13 autoantibodies, while inherited TTP is an autosomal recessive disorder due to mutations in the ADAMTS13 gene that lead to impaired protease function. Work-up of patients with TTP can include vWF cleaving protease activity and, if that activity is low, inhibitor screen followed by quantification. PMID: 18756543 Source: Regenstrief LOINC
Reference Information
| Type | Source | Reference |
|---|---|---|
| Article | New England Journal of MedicineCopyright Copyright © 2008 Massachusetts Medical Society | Furlan, M, Robles, R, et al. von Willebrand Factor-Cleaving Protease in Thrombotic Thrombocytopenic Purpura and the Hemolytic-Uremic Syndrome. New England Journal Medicine. 1998 339:1578-1584. Link to New England Journal of Medicine |
| Article | BloodCopyright Copyright © 2008 American Society of Hematology. | M Furlan, M, Robles, Lamie, B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood. 1996;87:4223-4234. Link to Blood |
Basic Part Properties
- Part Name
- von Willebrand factor cleaving protease
- Part Display Name
- von Willebrand factor (vWf) cleaving protease
- Part Type
- Component (Describes the core component or analyte measured)
- Created On
- 2003-09-12
- Construct for LOINC Short Name
- vWF Cp
LOINC Terminology Service (API) using HL7® FHIR® Get Info
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- CodeSystem lookup
- https:
//fhir.loinc.org/CodeSystem/$lookup?system=http: //loinc.org&code=LP32458-9
Language Variants Get Info
| Tag | Language | Translation |
|---|---|---|
| zh-CN | Chinese (China) | von Willebrand 因子裂解蛋白酶 Synonyms: von Willebrand 因子（vWf 因子、 |
| es-ES | Spanish (Spain) | Proteasa divisora del Factor de von Willebrand |
| it-IT | Italian (Italy) | von Willebrand, proteasi di taglio fattore Synonyms: Proteasi di taglio del fattore di von Willebrand |
| tr-TR | Turkish (Turkey) | von Willebrand faktörü parçalıyıcı proteaz |
| ru-RU | Russian (Russian Federation) | von Willebrand фактор (vWf) расщепляющая протеаза |
| fr-BE | French (Belgium) | Protéase de clivage du Facteur von Willebrand |
| fr-CA | French (Canada) | Protéase clivage du Facteur von Willebrand |
| nl-NL | Dutch (Netherlands) | von Willebrand-factor cleaving protease |
| el-GR | Greek (Greece) | πρωτεάση διάσπασης παράγοντα Von Willebrand Synonyms: πρωτεάση διάσπασης παράγοντα Von Willebrand |
LOINC Copyright
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