ADAMTS13 Protein

An ADAMTS protease that contains eight thrombospondin (TS) motifs. It cleaves VON WILLEBRAND FACTOR to control vWF-mediated THROMBOSIS. Mutations in the ADAMTS13 gene have been identified in familial cases of PURPURA, THROMBOTIC THROMBOCYTOPENIC and defects in ADAMTS13 activity are associated with MYOCARDIAL INFARCTION; BRAIN ISCHEMIA; PRE-ECLAMPSIA; and MALARIA.

Also Known As:

Networked: 252 relevant articles (3 outcomes, 11 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Matsumoto, Masanori: 10 articles (01/2017 - 01/2002)
2.	Fujimura, Yoshihiro: 10 articles (02/2007 - 01/2002)
3.	Lämmle, Bernhard: 9 articles (08/2015 - 04/2002)
4.	Veyradier, Agnès: 8 articles (01/2021 - 04/2002)
5.	Scheiflinger, Friedrich: 8 articles (06/2012 - 03/2003)
6.	Furlan, M: 8 articles (01/2002 - 01/2000)
7.	Su, Jian: 7 articles (12/2014 - 06/2004)
8.	Soejima, Kenji: 7 articles (03/2010 - 11/2003)
9.	Lämmle, B: 7 articles (08/2003 - 04/2001)
10.	Fujimura, Y: 7 articles (05/2002 - 07/2001)

Related Diseases

1.	Thrombotic Thrombocytopenic Purpura 11/30/2005 - "[A study on the significance of plasma thrombospondin1 in thrombotic thrombocytopenic purpura and the relationship between thrombospondin1 and von Willebrand factor cleaving protease (ADAMTS13)]." 01/01/2017 - "Inherited ADMATS13 or Upshaw-Schulman syndrome (USS) is caused by the deficiency of the Von Willebrand factor-cleaving protease. " 05/29/2013 - "The Upshaw Schulman syndrome (MIM #274150) is a hereditary deficiency of the von Willebrand factor cleaving protease (ADAMTS13) due to homozygous or compound heterozygous mutations in the ADAMTS13 gene. " 05/01/2012 - "Thrombotic thrombocytopenic purpura associated with human immunodeficiency virus is typically characterized by a sudden onset in a patient with a moderate immune deficiency and a few events of opportunistic diseases, and a profound acquired deficiency in the von Willebrand factor cleaving protease ADAMTS13. " 03/01/2010 - "The diagnosis of Upshaw Schulman Syndrome was confirmed by molecular testing of the gene encoding the von Willebrand factor cleaving protease ADAMTS13 which revealed compound heterozygosity for 1045C>T (R349C) and 3107C>A (S1036X) in the patients of family 1 and homozygosity for the common mutation 4143insA in the patients of family 2. Regular fresh-frozen plasma transfusions were sufficient to control the disease."
2.	acquired Thrombotic thrombocytopenic purpura 04/01/2002 - "Assays of von Willebrand factor-cleaving protease: a test for diagnosis of familial and acquired thrombotic thrombocytopenic purpura." 06/01/1998 - "Deficiency of von Willebrand factor-cleaving protease in familial and acquired thrombotic thrombocytopenic purpura." 09/01/2005 - "In the future, a better understanding of the relation between the von Willebrand factor-cleaving protease activity, the nature and titer of von Willebrand factor-cleaving protease inhibitors, and the risk of relapse of thrombotic thrombocytopenic purpura will be important to further validate the strategy of targeting the von Willebrand factor-cleaving protease inhibitory antibody as an effective means of treating acquired thrombotic thrombocytopenic purpura. " 04/01/2021 - "Acquired thrombotic thrombocytopenic purpura is a rare disease associated with the production of autoantibodies against the VWF-cleaving protease ADAMTS13. " 11/01/2020 - "Introduction: Acquired thrombotic thrombocytopenic purpura (aTTP) is a thrombotic microangiopathy caused by inhibitory autoantibodies against ADAMTS13 protein. "
3.	Glycogen Storage Disease Type VI (Glycogenosis Type VI) 12/01/2004 - "The level of VWF-cleaving protease activity in the patient was remarkably low (<5%) throughout her disease, even after she entered complete remission. "
4.	Thrombosis (Thrombus) 04/01/2019 - "We developed an mCherry-tagged murine ADAMTS13 protein and utilized an ex vivo flow chamber system to visualize the localization of ADAMTS13 within the platelet thrombus under different conditions of shear. " 10/01/2007 - "A specific VWF-cleaving protease (ADAMTS-13) physiologically down regulates the multimeric size of newly released and circulating VWF in order to prevent unwanted platelet thrombus formation. " 01/01/2019 - "Conclusion: These findings suggest that even in the setting of increased ADAMTS13 protease activity, VWF in HIV-1 infection is hyperadhesive, which may favor development of microvascular and arterial thromboses and thereby contribute to increased cardiovascular risk in HIV-1-infected individuals." 01/01/2022 - "Thrombus formation in iTTP is related to a severe deficiency of the specific von Willebrand-factor-cleaving protease ADAMTS13 due to ADAMTS13 autoantibodies. " 06/01/2016 - "ADAMTS13, as a specific von Willebrand factor (VWF)-cleaving protease, prevents microvascular thrombosis of VWF/platelet thrombi. "
5.	Coronary Disease (Coronary Heart Disease) 08/01/2001 - "Are increased levels of von Willebrand factor in chronic coronary heart disease caused by decrease in von Willebrand factor cleaving protease activity? " 04/01/2003 - "Decreased levels of von Willebrand factor-cleaving protease in coronary heart disease and thrombotic thrombocytopenic purpura: study of a simplified method for assaying the enzyme activity based on ristocetin-induced platelet aggregation." 08/01/2001 - "In the present study, we designed an enzyme-liked immunosorbent assay of VWF cleaving protease activity to be used to assess whether the high levels of VWF in coronary heart disease (CHD) relate to a deficiency of this protease. "

Related Drugs and Biologics

1.	Peptide Hydrolases (Proteases)
2.	Autoantibodies
3.	Solvents
4.	Detergents (Detergent)
5.	von Willebrand Factor
6.	Metalloproteases (Metalloproteinases)
7.	Thrombospondins
8.	Disintegrins
9.	Enzymes
10.	thiamine triphosphorate (TTP)

Related Therapies and Procedures

1.	Aftercare (After-Treatment)
2.	Thrombectomy
3.	Plasma Exchange
4.	Therapeutics
5.	Stem Cell Transplantation