ADAMTS13

Summary

Gene Symbol: ADAMTS13
Description: ADAM metallopeptidase with thrombospondin type 1 motif 13
Alias: ADAM-TS13, ADAMTS-13, C9orf8, VWFCP, vWF-CP, A disintegrin and metalloproteinase with thrombospondin motifs 13, a disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 13, vWF-cleaving protease, von Willebrand factor-cleaving protease
Species: human

Top Publications

  1. ncbi VWF73, a region from D1596 to R1668 of von Willebrand factor, provides a minimal substrate for ADAMTS-13
    Koichi Kokame
    National Cardiovascular Center Research Institute, 5 7 1 Fujishirodai, Suita, Osaka 565 8565, Japan
    Blood 103:607-12. 2004
  2. ncbi Comparison of von Willebrand factor antigen, von Willebrand factor-cleaving protease and protein S in blood components used for treatment of thrombotic thrombocytopenic purpura
    H Yarranton
    Haemostasis Research Unit, Department of Haematology, University College London, London, UK
    Transfus Med 14:39-44. 2004
  3. ncbi The effect of exercise on von Willebrand factor and ADAMTS-13 in individuals with type 1 and type 2B von Willebrand disease
    J Stakiw
    Department of Medicine, Queen s University, Kingston, ON, Canada
    J Thromb Haemost 6:90-6. 2008
  4. doi IL-1β down-regulates ADAMTS-13 mRNA expression in cells of the central nervous system
    G Alkistis Frentzou
    Biomedical Research Centre, Faculty of Health and Wellbeing, Sheffield Hallam University, Howard Street, Sheffield, S1 1WB, UK
    J Mol Neurosci 46:343-51. 2012
  5. ncbi Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis
    M Furlan
    Central Hematology Laboratory of the University, Inselspital, Bern, Switzerland
    Blood 87:4223-34. 1996
  6. ncbi Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion
    H M Tsai
    Division of Hematology, Montefiore Medical Center, Bronx, NY 10467, USA
    Blood 87:4235-44. 1996
  7. ncbi Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura
    M Furlan
    Central Hematology Laboratory, University Hospital, Inselspital, Bern, Switzerland
    Blood 89:3097-103. 1997
  8. ncbi Partial amino acid sequence of purified von Willebrand factor-cleaving protease
    H E Gerritsen
    Central Hematology Laboratory, University Hospital, Inselspital, Bern, Switzerland
    Blood 98:1654-61. 2001
  9. ncbi Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura
    X Zheng
    Department of Pathology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 276:41059-63. 2001
  10. ncbi A novel human metalloprotease synthesized in the liver and secreted into the blood: possibly, the von Willebrand factor-cleaving protease?
    K Soejima
    First Research Departmen, The Chemo Sero Therapeutic Research Institute, Kumamoto 869 1298, Japan
    J Biochem 130:475-80. 2001

Research Grants

  1. A platelet ligand mimetic for neurothrombotic disorders
    Catherine Woods; Fiscal Year: 2006
  2. "Development of a Laboratory Test for TTP"
    Christopher Hillyer; Fiscal Year: 2003
  3. VON WILLEBRAND FACTOR AND THE PATHOPHYSIOLOGY OF TTP
    Joel Moake; Fiscal Year: 1990
  4. X Long Zheng; Fiscal Year: 2016
  5. Ph 3 of Cyclosporine or Corticosteroids Adjunct to Plasma Exhcange in Tx of TTP
    Haifeng M Wu; Fiscal Year: 2013
  6. Timothy A Springer; Fiscal Year: 2015
  7. David Ginsburg; Fiscal Year: 2016
  8. Xiaoping Du; Fiscal Year: 2016
  9. Von Willebrand Factor Multimer Assembly and Structure
    J Evan Sadler; Fiscal Year: 2012
  10. BIOMECHANICS OF UNFOLDING THE A2 DOMAIN OF VON WILLEBRAND FACTOR
    Jin Yu Shao; Fiscal Year: 2012

Detail Information

Publications202 found, 100 shown here

  1. ncbi VWF73, a region from D1596 to R1668 of von Willebrand factor, provides a minimal substrate for ADAMTS-13
    Koichi Kokame
    National Cardiovascular Center Research Institute, 5 7 1 Fujishirodai, Suita, Osaka 565 8565, Japan
    Blood 103:607-12. 2004
    ..Thus, VWF73 will be a useful seed to develop a new rapid assay to determine ADAMTS-13 activity...
  2. ncbi Comparison of von Willebrand factor antigen, von Willebrand factor-cleaving protease and protein S in blood components used for treatment of thrombotic thrombocytopenic purpura
    H Yarranton
    Haemostasis Research Unit, Department of Haematology, University College London, London, UK
    Transfus Med 14:39-44. 2004
    Replacement of normal levels of von Willebrand factor-cleaving protease (VWF:CP, ADAMTS13) activity from infused plasma is important in plasma exchange (PEX) for the treatment of thrombotic thrombocytopenic purpura (TTP) patients...
  3. ncbi The effect of exercise on von Willebrand factor and ADAMTS-13 in individuals with type 1 and type 2B von Willebrand disease
    J Stakiw
    Department of Medicine, Queen s University, Kingston, ON, Canada
    J Thromb Haemost 6:90-6. 2008
    ..The effect of exercise on von Willebrand factor (VWF) and ADAMTS-13 levels in individuals with von Willebrand disease (VWD) has never been reported...
  4. doi IL-1β down-regulates ADAMTS-13 mRNA expression in cells of the central nervous system
    G Alkistis Frentzou
    Biomedical Research Centre, Faculty of Health and Wellbeing, Sheffield Hallam University, Howard Street, Sheffield, S1 1WB, UK
    J Mol Neurosci 46:343-51. 2012
    ..This is the first report of ADAMTS-13 expression in cells of the CNS; however, its function remains to be determined...
  5. ncbi Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis
    M Furlan
    Central Hematology Laboratory of the University, Inselspital, Bern, Switzerland
    Blood 87:4223-34. 1996
    ..The size, amino acid composition, and amino terminal sequence of the reduced fragments confirmed that the peptide bond 842Tyr-843Met had been cleaved, ie, the same bond that has been proposed to be cleaved in vivo...
  6. ncbi Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion
    H M Tsai
    Division of Hematology, Montefiore Medical Center, Bronx, NY 10467, USA
    Blood 87:4235-44. 1996
    ..This model may explain the decrease in vWF multimer sizes in various clinical conditions...
  7. ncbi Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura
    M Furlan
    Central Hematology Laboratory, University Hospital, Inselspital, Bern, Switzerland
    Blood 89:3097-103. 1997
    ..The assay of the vWF-cleaving protease activity may be used as a sensitive diagnostic tool for identification of subjects with a latent TTP tendency...
  8. ncbi Partial amino acid sequence of purified von Willebrand factor-cleaving protease
    H E Gerritsen
    Central Hematology Laboratory, University Hospital, Inselspital, Bern, Switzerland
    Blood 98:1654-61. 2001
    ..There was even a temporary increase in protease activity during the first 3 days of incubation...
  9. ncbi Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura
    X Zheng
    Department of Pathology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 276:41059-63. 2001
    ..sequence, VWFCP was identified recently as a new member of the ADAMTS family of metalloproteases and designated ADAMTS13. The 4.6-kilobase pair cDNA sequence for VWFCP has now been determined...
  10. ncbi A novel human metalloprotease synthesized in the liver and secreted into the blood: possibly, the von Willebrand factor-cleaving protease?
    K Soejima
    First Research Departmen, The Chemo Sero Therapeutic Research Institute, Kumamoto 869 1298, Japan
    J Biochem 130:475-80. 2001
    ....
  11. ncbi Von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura
    Valentina Bianchi
    Central Hematology Laboratory, University Hospital, Inselspital, Bern, Switzerland
    Blood 100:710-3. 2002
    A severe deficiency in von Willebrand factor-cleaving protease (ADAMTS13) activity (< 5% that in normal plasma) has been observed in most patients with a diagnosis of thrombotic thrombocytopenic purpura (TTP) but not in those with a ..
  12. pmc Mutations and common polymorphisms in ADAMTS13 gene responsible for von Willebrand factor-cleaving protease activity
    Koichi Kokame
    Research Institute, National Cardiovascular Center, Suita, Osaka 565 8565, Japan
    Proc Natl Acad Sci U S A 99:11902-7. 2002
    ..of the corresponding cDNA revealed that the 1,427-aa polypeptide is a member of the ADAMTS gene family, termed ADAMTS13. Twelve rare mutations in this gene have been identified in patients with congenital TTP...
  13. ncbi ADAMTS-13 rapidly cleaves newly secreted ultralarge von Willebrand factor multimers on the endothelial surface under flowing conditions
    Jing Fei Dong
    Thrombosis Research Section, Department of Medicine, Baylor College of Medicine, Houston, TX 77030, USA
    Blood 100:4033-9. 2002
    ..These results suggest that cleavage of EC-derived ULVWF multimers by ADAMTS-13 is a rapid physiologic process that occurs on endothelial cell surfaces...
  14. ncbi ADAMTS-13 metalloprotease interacts with the endothelial cell-derived ultra-large von Willebrand factor
    Jing Fei Dong
    Thrombosis Research Section, Department of Medicine, Baylor College of Medicine, Houston, Texas 77030, USA
    J Biol Chem 278:29633-9. 2003
    ..Once released, these cleaved VWF fragments become inaccessible for the metalloprotease to prevent further cleavage...
  15. ncbi ADAMTS-13 cysteine-rich/spacer domains are functionally essential for von Willebrand factor cleavage
    Kenji Soejima
    First Research Department, The Chemo Sero Therapeutic Research Institute, Kawabe, Kyokushi, Kikuchi, Kumamoto 869 1298, Japan
    Blood 102:3232-7. 2003
    ..The major epitopes of these antibodies were found to reside within the cysteine-rich/spacer domains. These results suggest that the ADAMTS-13 cysteine-rich/spacer domains are essential for VWF-CP activity...
  16. ncbi Ten candidate ADAMTS13 mutations in six French families with congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome)
    A Veyradier
    INSERM U143, Le Kremlin Bicêtre and Service de Néphrologie, Hopital Robert Debre, Paris, France
    J Thromb Haemost 2:424-9. 2004
    b>ADAMTS13, the specific von Willebrand factor (VWF)-cleaving metalloprotease, prevents the spontaneous formation of platelet thrombi in the microcirculation by degrading the highly adhesive ultralarge VWF multimers into smaller forms...
  17. pmc Binding of platelet glycoprotein Ibalpha to von Willebrand factor domain A1 stimulates the cleavage of the adjacent domain A2 by ADAMTS13
    Kenji Nishio
    Howard Hughes Medical Institute and Department of Medicine, Washington University School of Medicine, St Louis, MO 63110, USA
    Proc Natl Acad Sci U S A 101:10578-83. 2004
    ..Domain A2 contains the Tyr-1605-Met-1606 bond that is cleaved by the metalloprotease ADAMTS13, and this reaction inhibits platelet thrombus growth...
  18. ncbi Localization of ADAMTS13 to the stellate cells of human liver
    Masahito Uemura
    Third Department of Internal Medicine, Department of Anatomy, Nara Medical University, 840 Shijo Cho, Kashihara City, Nara, 634 8522, Japan
    Blood 106:922-4. 2005
    Although the chromosomal localization (9q34) of the gene encoding the human form of ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type-1 motifs 13) and its exclusive expression in the liver have been established, ..
  19. ncbi ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases
    Manfred Rieger
    Baxter BioScience, Biomedical Reserach Center, Orth, Austria
    Blood 106:1262-7. 2005
    Autoantibodies neutralizing human ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motif), the metalloprotease that physiologically cleaves von Willebrand factor, are a major cause of severe deficiency of the ..
  20. pmc The proximal carboxyl-terminal domains of ADAMTS13 determine substrate specificity and are all required for cleavage of von Willebrand factor
    Jihui Ai
    Department of Pathology and Laboratory Medicine, The Children s Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, 19104, USA
    J Biol Chem 280:29428-34. 2005
    b>ADAMTS13 limits platelet-rich thrombosis by cleaving von Willebrand factor at the Tyr(1605)-Met(1606) bond. Previous studies showed that ADAMTS13 truncated after spacer domain remains proteolytically active or hyperactive...
  21. ncbi Cleavage of ultra-large von Willebrand factor by ADAMTS-13 under flow conditions
    J F Dong
    Thrombosis Research Section, Department of Medicine, Baylor College of Medicine, Houston, TX 77030, USA
    J Thromb Haemost 3:1710-6. 2005
    ..Dissecting the process of ULVWF proteolysis is important for not only understanding the pathophysiology of thrombotic microangiopathies, but also developing more effective means to treat these deadly diseases...
  22. pmc Recombinant CUB-1 domain polypeptide inhibits the cleavage of ULVWF strings by ADAMTS13 under flow conditions
    Zhenyin Tao
    Thrombosis Research Section, Department of Medicine, BCM286, N1319, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
    Blood 106:4139-45. 2005
    The metalloprotease ADAMTS13 (a disintegrin and metalloprotease with thrombospondin motif) converts the hyperreactive unusually large (UL) forms of von Willebrand factor (VWF) that are newly released from endothelial cells into less ..
  23. ncbi Modulation of ADAMTS13 secretion and specific activity by a combination of common amino acid polymorphisms and a missense mutation
    Barbara Plaimauer
    Baxter BioScience, Department of Discovery Research, Vienna, Austria
    Blood 107:118-25. 2006
    Sequence analysis of the ADAMTS13 locus of 2 patients with hereditary thrombotic thrombocytopenic purpura (TTP) revealed the homozygous presence of 4 single nucleotide polymorphisms (SNPs) (R7W, Q448E, P618A, A732V) and a rare missense ..
  24. ncbi Impact of mutations in the von Willebrand factor A2 domain on ADAMTS13-dependent proteolysis
    Wolf Achim Hassenpflug
    Dept of Pediatric Hematology and Oncology, University Medical Center Hamburg Eppendorf, Martinistrasse 52, D 20246 Hamburg, Germany
    Blood 107:2339-45. 2006
    ..Underlying mutations cluster in the A2 domain of VWF around its cleavage site for ADAMTS13. We investigated the impact of mutations commonly found in patients with VWD type 2A on ADAMTS13-dependent ..
  25. pmc Apical sorting of ADAMTS13 in vascular endothelial cells and Madin-Darby canine kidney cells depends on the CUB domains and their association with lipid rafts
    Dezhi Shang
    Department of Pathology and Laboratory Medicine, The Children s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, 816G ARC, Philadelphia, PA 19104, USA
    Blood 108:2207-15. 2006
    b>ADAMTS13 biosynthesis appeared to occur mainly in hepatic stellate cells, but detection of ADAMTS13 mRNA in many other tissues suggests that vascular endothelium may also produce ADAMTS13...
  26. ncbi ADAMTS13 and von Willebrand factor and the risk of myocardial infarction in men
    Chan K N K Chion
    Department of Haematology, Imperial College London, Hammersmith Hospital Campus, London, United Kingdom
    Blood 109:1998-2000. 2007
    ..This function depends on its multimeric size, which is controlled by ADAMTS13. We measured plasma ADAMTS13 and VWF antigen levels by enzyme-linked immunosorbent assay (ELISA) in a large ..
  27. pmc Platelet-VWF complexes are preferred substrates of ADAMTS13 under fluid shear stress
    Kyuhwan Shim
    Department of Medicine, Howard Hughes Medical Institute, Washington University School of Medicine, St Louis, MO 63110, USA
    Blood 111:651-7. 2008
    Endothelial cells secrete prothrombotic ultralarge von Willebrand factor (VWF) multimers, and the metalloprotease ADAMTS13 cleaves them into smaller, less dangerous multimers...
  28. pmc Evidence that high von Willebrand factor and low ADAMTS-13 levels independently increase the risk of a non-fatal heart attack
    J T B Crawley
    Department of Haematology, Imperial College London, London, UK
    J Thromb Haemost 6:583-8. 2008
    ..Moreover, ADAMTS-13 influences hemostatic plug formation in mouse models. We therefore studied their associations in the Glasgow MI Study (GLAMIS)...
  29. pmc Factor VIII accelerates proteolytic cleavage of von Willebrand factor by ADAMTS13
    Wenjing Cao
    Departments of Pathology and Laboratory Medicine, Children s Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA 19104, USA
    Proc Natl Acad Sci U S A 105:7416-21. 2008
    Proteolytic processing of von Willebrand factor (VWF) by ADAMTS13 metalloproteinase is crucial for normal hemostasis...
  30. pmc Leukocyte proteases cleave von Willebrand factor at or near the ADAMTS13 cleavage site
    Thomas J Raife
    Department of Pathology, University of Iowa Carver College of Medicine, Iowa City, IA 52240, USA
    Blood 114:1666-74. 2009
    ..The importance of ADAMTS13 metalloprotease in VWF regulation is demonstrated by the association between severe deficiency of ADAMTS13 and ..
  31. pmc Endothelial cell ADAMTS-13 and VWF: production, release, and VWF string cleavage
    Nancy A Turner
    Department of Bioengineering, Rice University, 6500 Main St, Houston, TX 77030, USA
    Blood 114:5102-11. 2009
    ..HUVEC ADAMTS13 mRNA expression was approximately 1:100 of VWF monomeric subunit expression...
  32. doi ADAMTS13 mutations and polymorphisms in congenital thrombotic thrombocytopenic purpura
    Luca A Lotta
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Universita degli Studi di Milano, Department of Medicine and Medical Specialities, IRCCS Maggiore Hospital, Mangiagalli and Regina Elena Foundation, Luigi Villa Foundation, Milan, Italy
    Hum Mutat 31:11-9. 2010
    ..microangiopathic hemolytic anemia, associated with the deficiency of the von Willebrand factor-cleaving protease (ADAMTS13) due to mutations in the corresponding gene...
  33. doi Association between reduced ADAMTS13 and diabetic nephropathy
    Shotaro Taniguchi
    Laboratory and Vascular Medicine, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan
    Thromb Res 125:e310-6. 2010
    Deficiency of Von Willebrand factor (VWF)-cleaving protease (ADAMTS13) causes platelet thrombosis in the microcirculation. Intrarenal coagulation is thought to be associated with the development and progression of diabetic nephropathy...
  34. pmc Factor VIII and platelets synergistically accelerate cleavage of von Willebrand factor by ADAMTS13 under fluid shear stress
    Christopher G Skipwith
    Biochemistry and Molecular Biophysics Graduate Program, Children s Hospital of Philadelphia and the University of Pennsylvania Medical Center, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 285:28596-603. 2010
    ..have demonstrated that factor VIII (FVIII) or platelets alone increase cleavage of von Willebrand factor (VWF) by ADAMTS13 under mechanically induced shear stresses...
  35. pmc Association of ADAMTS13 polymorphism with cerebral malaria
    Sirima Kraisin
    Doctoral Program in Biomedical Sciences, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Ibaraki, Japan
    Malar J 10:366. 2011
    ..studies reported increased levels of von Willebrand factor (VWF) and reduced activity of VWF-cleaving protease, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), in patients with cerebral ..
  36. pmc Protective anti-inflammatory effect of ADAMTS13 on myocardial ischemia/reperfusion injury in mice
    Simon F De Meyer
    Immune Disease Institute, Boston, MA 02115, USA
    Blood 120:5217-23. 2012
    ..To investigate a potential protective role of ADAMTS13, a protease cleaving von Willebrand factor multimers, during myocardial ischemia/reperfusion, we used a mouse ..
  37. ncbi Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family
    K Fujikawa
    Department of Biochemistry, University of Washington, Seattle, WA 98195, USA
    Blood 98:1662-6. 2001
    ..An active site sequence of HEIGHSFGLEHE (single-letter amino acid codes) was located at 150 residues from the N terminus of the protein...
  38. ncbi Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura
    G G Levy
    Howard Hughes Medical Institute, Departments of Internal Medicine and Human Genetics, and Cellular and Molecular Biology Program, University of Michigan Medical Center, Ann Arbor, Michigan 48109, USA
    Nature 413:488-94. 2001
    ..of a much larger transcript, identifying a new member of the ADAMTS family of zinc metalloproteinase genes (ADAMTS13)...
  39. ncbi Cloning, expression analysis, and structural characterization of seven novel human ADAMTSs, a family of metalloproteinases with disintegrin and thrombospondin-1 domains
    Santiago Cal
    Departamento de Bioquimica y Biologia Molecular, Facultad de Medicina, Instituto Universitario de Oncologia, Universidad de Oviedo, 33006, Oviedo, Spain
    Gene 283:49-62. 2002
    ..ADAMTS14, ADAMTS16, ADAMTS17, ADAMTS18, and ADAMTS19), kidney (ADAMTS14, ADAMTS15, and ADAMTS16), and liver (ADAMTS13, ADAMTS15 and ADAMTS18)...
  40. ncbi Cloning, expression, and functional characterization of the von Willebrand factor-cleaving protease (ADAMTS13)
    Barbara Plaimauer
    Baxter BioScience, Biomedical Research Center, Orth, Austria
    Blood 100:3626-32. 2002
    ..gene encoding for VWF-cp has recently been identified as a member of the metalloprotease family and designated ADAMTS13, but the functional activity of the ADAMTS13 gene product has not been verified...
  41. ncbi von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP
    Reinhard Schneppenheim
    Children s University Hospital, Hamburg Eppendorf Lab Association Prof Arndt and Partners, Coagulation Laboratory, Hamburg, Germany
    Blood 101:1845-50. 2003
    ..multimers of von Willebrand factor (VWF) due to deficiency of the specific VWF-cleaving protease (VWF-CP) ADAMTS13, resulting in microangiopathic disease...
  42. ncbi Genetic defects leading to hereditary thrombotic thrombocytopenic purpura
    Koichi Kokame
    National Cardiovascular Center Research Institute, Suita, Osaka, Japan
    Semin Hematol 41:34-40. 2004
    ..23 patients with hereditary TTP and their families has been analyzed and 33 causative mutations identified in the ADAMTS13 gene: 19 missense, five nonsense, five frameshift, and four splice mutations...
  43. ncbi Identification of novel mutations in ADAMTS13 in an adult patient with congenital thrombotic thrombocytopenic purpura
    Toshihiro Uchida
    New Product Laboratories II, Daiichi Pharmaceutical, Tokyo, Japan
    Blood 104:2081-3. 2004
    ..uremic syndrome (TTP/HUS) is associated with an inherited von Willebrand factor-cleaving protease (ADAMTS13 [a disintegrin and metalloprotease with thrombospondin type I domains 13]) deficiency...
  44. ncbi The frequency of P475S polymorphism in von Willebrand factor-cleaving protease in the Chinese population and its relevance to arterial thrombotic disorders
    Changgeng Ruan
    Thromb Haemost 91:1257-8. 2004
  45. ncbi The spacer domain of ADAMTS13 contains a major binding site for antibodies in patients with thrombotic thrombocytopenic purpura
    Brenda M Luken
    Department of Plasma Proteins, Sanquin Research at CLB, Amsterdam, The Netherlands
    Thromb Haemost 93:267-74. 2005
    ..thrombocytopenic purpura (TTP) is a microangiopathy often associated with a severely decreased activity of ADAMTS13. In plasma of the majority of patients with TTP, antibodies are present that inhibit the von Willebrand factor (..
  46. ncbi ADAMTS13 turns 3
    Gallia G Levy
    Cell and Molecular Biology Program and Department of Pediatrics, University of Michigan, Ann Arbor, MI, USA
    Blood 106:11-7. 2005
    ..factor (VWF)-cleaving protease implicated in thrombocytopenic purpura (TTP) pathogenesis was identified as ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13)...
  47. ncbi Binding of ADAMTS13 to von Willebrand factor
    Elaine M Majerus
    Department of Medicine and Howard Hughes Medical Institute, Washington University School of Medicine, 660 S Euclid Avenue, St Louis, MO 63110, USA
    J Biol Chem 280:21773-8. 2005
    b>ADAMTS13, a metalloprotease, cleaves von Willebrand factor (VWF) in plasma to generate smaller, less thrombogenic fragments...
  48. ncbi Absence of Pro475Ser polymorphism in ADAMTS-13 in Caucasians
    T N Bongers
    J Thromb Haemost 3:805. 2005
  49. pmc Enzymatically active ADAMTS13 variants are not inhibited by anti-ADAMTS13 autoantibodies: a novel therapeutic strategy?
    Wenhua Zhou
    Division of Hematology, Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, New York 10467, USA
    J Biol Chem 280:39934-41. 2005
    b>ADAMTS13 (a disintegrin and metalloprotease with thrombospondin motifs), a circulating multidomain zinc metalloprotease of the reprolysin subfamily, is critical for preventing von Willebrand factor-platelet interaction under high shear ..
  50. ncbi Novel compound heterozygote mutations (H234Q/R1206X) of the ADAMTS13 gene in an adult patient with Upshaw-Schulman syndrome showing predominant episodes of repeated acute renal failure
    Yugo Shibagaki
    Department of Nephrology and Endocrinology, Graduate School of Medicine, University of Tokyo, Japan
    Nephrol Dial Transplant 21:1289-92. 2006
    ..patients with Upshaw-Schulman syndrome (USS)--a congenital deficiency of von Willebrand factor-cleaving protease (ADAMTS13) activity--responds very well to plasma infusion and does not even require plasma exchange...
  51. ncbi [Identification of two novel mutations in ADAMTS13 gene in a patient with hereditary thrombotic thrombocytopenic purpura]
    Fang Liu
    Jiangsu Institute of Hematology, The First Affliated Hospital of Soochow University, Suzhou, China
    Zhonghua Xue Ye Xue Za Zhi 26:521-4. 2005
    To investigate the gene mutations of ADAMTS13 in a highly suspected hereditary thrombocytopenic purpura (TTP) patient, and then make a progressive diagnosis and adjust the plan of therapy.
  52. ncbi Novel ADAMTS-13 mutations in an adult with delayed onset thrombotic thrombocytopenic purpura
    Z Tao
    Section of Thrombosis Research, Baylor College of Medicine, Houston, TX 77030, USA
    J Thromb Haemost 4:1931-5. 2006
    ..Mutations throughout the ADAMTS13 gene have been identified in congenital TTP patients, most of whom have initial episodes during infancy or in ..
  53. ncbi Effect of von Willebrand disease type 2B and type 2M mutations on the susceptibility of von Willebrand factor to ADAMTS-13
    J Rayes
    Inserm U770 and Universite Paris Sud, Faculté de Médecine IFR93, Le Kremlin Bicetre, France
    J Thromb Haemost 5:321-8. 2007
    ..While increased proteolysis by a disintegrin-like and metalloprotease with thrombospondin type 1 motifs-13 (ADAMTS-13) has been clearly established for VWF type 2A, only little is known about VWF types 2B and 2M in this regard...
  54. ncbi Functional imaging of shear-dependent activity of ADAMTS13 in regulating mural thrombus growth under whole blood flow conditions
    Yasuaki Shida
    Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan
    Blood 111:1295-8. 2008
    The metalloprotease ADAMTS13 is assumed to regulate the functional levels of von Willebrand factor (VWF) appropriate for normal hemostasis in vivo by reducing VWF multimer size, which directly represents the thrombogenic activity of this ..
  55. pmc Frequency of Pro475Ser polymorphism of ADAMTS13 gene and its association with ADAMTS-13 activity in the Korean population
    Mun Ju Jang
    Department of Internal Medicine, College of Medicine, Pochon CHA University, 351 Yatap dong, Bundang gu, Sungnam 463 712, Korea
    Yonsei Med J 49:405-8. 2008
    ..In this study, the frequency of the Pro475Ser polymorphism in Koreans was studied and plasma ADAMTS-13 activity was measured to find out whether this polymorphism contributes to decreased ADAMTS-13 activity in Koreans...
  56. pmc A novel binding site for ADAMTS13 constitutively exposed on the surface of globular VWF
    Sara Zanardelli
    Department of Haematology, Imperial College London, Hammersmith Hospital Campus, London, United Kingdom
    Blood 114:2819-28. 2009
    b>ADAMTS13 metalloprotease regulates the multimeric size of von Willebrand factor (VWF) by cleaving the Tyr1605-Met1606 bond in the VWF A2 domain. The mechanisms of VWF recognition by ADAMTS13 have yet to be fully resolved...
  57. pmc Characterization of conformation-sensitive antibodies to ADAMTS13, the von Willebrand cleavage protease
    Zuben E Sauna
    Laboratory of Hemostasis, Division of Hematology, Center for Biologics Evaluation and Research, Food and Drug Administration, Bethesda, Maryland, United States of America
    PLoS ONE 4:e6506. 2009
    The zinc metalloprotease ADAMTS13 is a multidomain protein that cleaves von Willebrand Factor (VWF) and is implicated in Thrombotic Thrombocytopenic Purpura (TTP) pathogenesis...
  58. pmc ADAMTS13 bound to endothelial cells exhibits enhanced cleavage of von Willebrand factor
    Anthony N Vomund
    Division of Hematology, Department of Internal Medicine, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 284:30925-32. 2009
    b>ADAMTS13 is a plasma metalloprotease that cleaves ultralarge von Willebrand factor multimers to generate less thrombogenic fragments...
  59. doi Severe malaria is associated with a deficiency of von Willebrand factor cleaving protease, ADAMTS13
    Ester C Löwenberg
    Department of Vascular Medicine, Academic Medical Center, University of Amsterdam, The Netherlands
    Thromb Haemost 103:181-7. 2010
    ..disease strongly associated with a deficiency of the von Willebrand factor (VWF) cleaving protease, ADAMTS13. We measured plasma ADAMTS13 activity, VWF antigen and VWF propeptide levels in 30 patients with severe ..
  60. pmc A splice variant of ADAMTS13 is expressed in human hepatic stellate cells and cancerous tissues
    Noam Shomron
    Department of Cell and Developmental Biology, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
    Thromb Haemost 104:531-5. 2010
    Although ADAMTS13, the von Willebrand factor (VWF)-cleaving protease, is expressed in a range of tissues, the physiological significance of tissue-specific ADAMTS13 alternative splicing isoforms have yet to be clarified...
  61. doi VWF and ADAMTS13 behavior in estradiol-treated HUVEC
    Yanina Powazniak
    FONCyT CONICET, Instituto de Investigaciones Hematologicas, Academia Nacional de Medicina, Buenos Aires, Argentina
    Eur J Haematol 86:140-7. 2011
    In this study, the role of 17β-estradiol (E2) in the regulation of von Willebrand factor (VWF) and ADAMTS13 synthesis, storage, and secretion was investigated in cultured human umbilical vein endothelial cells (HUVEC).
  62. pmc Mechanism of von Willebrand factor scissile bond cleavage by a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13)
    Yaozu Xiang
    Centre for Haematology, Department of Medicine, Imperial College London, Hammersmith Hospital Campus, London W12 ONN, United Kingdom
    Proc Natl Acad Sci U S A 108:11602-7. 2011
    The platelet-tethering function of von Willebrand factor (VWF) is proteolytically regulated by ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), which cleaves the Tyr1605-Met1606 (P1-P1') bond ..
  63. ncbi Nonneutralizing IgM and IgG antibodies to von Willebrand factor-cleaving protease (ADAMTS-13) in a patient with thrombotic thrombocytopenic purpura
    Friedrich Scheiflinger
    Baxter BioScience, Biomedical Research Center, Uferstrasse 15, 2304 Orth Donau, Austria
    Blood 102:3241-3. 2003
    ..Given that ADAMTS-13 may interact physiologically with various receptors or ligands, the occurrence, distribution, and the epitope mapping of nonneutralizing antibodies will be an important area for future research...
  64. ncbi Cleavage of the ADAMTS13 propeptide is not required for protease activity
    Elaine M Majerus
    Department of Medicine, Washington University School of Medicine, 660 South Euclid Avenue, St Louis, MO 63110, USA
    J Biol Chem 278:46643-8. 2003
    b>ADAMTS13 belongs to the "a disintegrin and metalloprotease with thrombospondin repeats" family, and cleaves von Willebrand factor multimers into smaller forms...
  65. ncbi Thrombotic microangiopathies: thrombotic thrombocytopenic purpura / hemolytic uremic syndrome
    Maria Goretti Polito
    Universidade Federal de Sao Paulo, Brazil
    J Bras Nefrol 32:303-15. 2010
    ..Recent studies have demonstrated that deficiency in the von Willebrand factor cleaving protease ADAMTS13, due to deficiency of ADAMTS13 can be genetic or more common, acquired, resulting from ..
  66. ncbi Thrombotic microangiopathy in malignant hypertension and hemolytic uremic syndrome (HUS)/ thrombotic thrombocytopenic purpura (TTP): can we differentiate one from the other?
    Yugo Shibagaki
    Department of Nephrology and Endocrinology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
    Hypertens Res 28:89-95. 2005
    ..a disintegrin and metalloprotease domain, with thrombospondin type 1 motif 13), which is a von Willebrand Factor cleaving protease, has been shown to be very low in familial or some of the sporadic cases of TTP, and a low level ..
  67. doi Interventions for hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: a systematic review of randomized controlled trials
    Mini Michael
    Renal Section Department of Pediatrics, Texas Children s Hospital Baylor College of Medicine, Houston, TX, USA
    Am J Kidney Dis 53:259-72. 2009
    ..The objective of this systematic review is to evaluate the benefits and harms of available interventions for HUS and TTP...
  68. ncbi High-dose plasma infusion versus plasma exchange as early treatment of thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome
    Paul Coppo
    Laboratoire Hématopoïèse et Cellules souches, Institut Gustave Roussy, Villejuif, France
    Medicine (Baltimore) 82:27-38. 2003
    ..However, the large volumes of plasma required to reach complete remission may result in fluid overload, which may necessitate subsequent therapeutic plasma exchange...
  69. pmc Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS): a 24-year clinical experience with 178 patients
    Mark Levandovsky
    Division of Hematology and Oncology, UC Davis School of Medicine, Sacramento, CA, USA
    J Hematol Oncol 1:23. 2008
    ..We performed a retrospective cohort study of 178 consecutively treated patients with TTP-HUS and analyzed whether clinical or laboratory characteristics could predict for important short- and long-term outcome measures...
  70. ncbi [Evaluation of antibiotics used for enterohemorrhagic Escherichia coli O157 enteritis--effect of various antibiotics on extracellular release of verotoxin]
    T Ito
    Department of Bacteriology, Faculty of Medicine, Juntendo University
    Kansenshogaku Zasshi 71:130-5. 1997
    ....
  71. doi Lessons learned from the Oklahoma thrombotic thrombocytopenic purpura-hemolytic uremic syndrome registry
    James N George
    College of Public Health, The University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma 73126 0901, USA
    J Clin Apher 23:129-37. 2008
    ..related to their associated conditions and clinically apparent etiologies, and by the presence of severe ADAMTS13 deficiency. ADAMTS13 activity has been measured on 235 (93%) of 254 patients since 1995...
  72. ncbi Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome following allogeneic HPC transplantation: a diagnostic dilemma
    James N George
    Department of Medicine, College of Medicine, The University of Oklahoma Health Sciences Center, Oklahoma City 73190, USA
    Transfusion 44:294-304. 2004
    ..Nevertheless, because multiple transplant-related sequela can cause the characteristic clinical features of TTP-HUS, the diagnosis is difficult...
  73. ncbi Increased von Willebrand factor over decreased ADAMTS13 activity may contribute to the development of liver disturbance and multiorgan failure in patients with alcoholic hepatitis
    Tomomi Matsuyama
    Third Department of Internal Medicine, Nara Medical University, Kashihara, Nara, Japan
    Alcohol Clin Exp Res 31:S27-35. 2007
    ..The deficiency of ADAMTS13 results in an increase of the plasma unusually large von Willebrand factor multimer (UL-VWFM) and finally causes ..
  74. ncbi [Thrombotic microangiopathies: HUS/TTP. Physiopathological aspects]
    J C Sanchez Avalos
    Departamento de Hematologia, Hospital de Clinicas Jose de San Martin, Facultad de Medicina, Universidad de Buenos Aires
    Medicina (B Aires) 60:46-58. 2000
    ..Better knowledge of the endothelial physiology and the genetic polymorphism of the endothelial cell, the clonation of vWF-cleavage protease, etc., will provide valuable tools for the understanding of these fascinating entities...
  75. ncbi Thrombotic thrombocytopenic purpura associated with ticlopidine in the setting of coronary artery stents and stroke prevention
    C L Bennett
    Institute for Health Services Research and Policy Studies, Robert H Lurie Comprehensive Cancer Center, Northwestern University, Chicago, Ill, USA
    Arch Intern Med 159:2524-8. 1999
    ..Recently, Hoffman-LaRoche Pharmaceuticals, following reports of 20 deaths from ticlopidine-associated TTP, updated the information about the hematologic adverse effects of the drug...
  76. doi Plasma exchange therapy for thrombotic thrombocytopenic purpura in pediatric patients with liver transplantation
    C H Lee
    Division of Nephrology, Liver Transplant Program, Chang Gung Memorial Hosptial Kaohsiung Medical Center, Chang Gung University College of Medicine, Taiwan
    Transplant Proc 40:2554-6. 2008
    ..We reported our experience with TTP using plasma exchange (PE) therapy in patients with liver transplantation (OLT)...
  77. doi Potential role of enhanced cytokinemia and plasma inhibitor on the decreased activity of plasma ADAMTS13 in patients with alcoholic hepatitis: relationship to endotoxemia
    Masatoshi Ishikawa
    Third Department of Internal Medicine, Nara Medical University, Kashihara, Nara, Japan
    Alcohol Clin Exp Res 34:S25-33. 2010
    Deficiency of ADAMTS13 (adisintegrin-like and metalloproteinase with thrombospondin type-1 motifs 13) results in an increase in unusually large von Willebrand factor multimer (UL-VWFM) of the plasma and finally causes microcirculatory ..
  78. ncbi Significance of plasma von Willebrand factor level and von Willebrand factor-cleaving protease activity in patients with chronic renal diseases
    Guo Yuan Lu
    Department of Nephrology Jiangsu Institute of Haematology, First Affiliated Hospital of Soochow University, Suzhou, Jiangsu 215006, China
    Chin Med J (Engl) 121:133-6. 2008
    ..On release from endothelial cells, vWF is rapidly cleaved by ADAMTS13/vWF-cleaving protease (vWF-CP)...
  79. ncbi Incidence and clinical course of thrombotic thrombocytopenic purpura due to ticlopidine following coronary stenting. EPISTENT Investigators. Evaluation of Platelet IIb/IIIa Inhibitor for Stenting
    S R Steinhubl
    Department of Cardiology, Wilford Hall Medical Center, Lackland Air Force Base, San Antonio, Tex 78236 5300, USA
    JAMA 281:806-10. 1999
    ....
  80. ncbi Posttransplantation thrombotic thrombocytopenic purpura: a single-center experience and a contemporary review
    Michelle A Elliott
    Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, Minn 55905, USA
    Mayo Clin Proc 78:421-30. 2003
    ..To assess the activity of von Willebrand factor-cleaving protease (vWF-CP) in patients with thrombotic thrombocytopenic purpura (TTP) complicating bone marrow transplantation (BMT) and peripheral blood stem cell transplantation (PBSCT)...
  81. ncbi Therapeutic plasma exchange for the treatment of thrombotic thrombocytopenic purpura: a retrospective multicenter study
    Fevzi Altuntas
    Erciyes University, Department of Hematology and Hemapheresis Unit, 38039 Kayseri, Turkey
    Transfus Apher Sci 36:57-67. 2007
    ..Thrombotic thrombocytopenic purpura (TTP) is a rare disease that is fatal if it is not treated. Therapeutic plasma exchange (TPE) has resulted in excellent remission and survival rates in TTP patients...
  82. pmc Two mechanistic pathways for thienopyridine-associated thrombotic thrombocytopenic purpura: a report from the SERF-TTP Research Group and the RADAR Project
    Charles L Bennett
    VA Center for Management of Complex Chronic Care at Jesse Brown VA Medical Center, Division of Hematology Oncology, Department of Medicine, Feinberg School of Medicine, Northwestern University, Chicago, Illinois 60611, USA
    J Am Coll Cardiol 50:1138-43. 2007
    ..We sought to describe clinical and laboratory findings for a large cohort of patients with thienopyridine-associated thrombotic thrombocytopenic purpura (TTP)...
  83. doi Acute myocardial infarction as a systemic prothrombotic condition evidenced by increased von Willebrand factor protein over ADAMTS13 activity in coronary and systemic circulation
    Manabu Horii
    First Department of Internal Medicine, Nara Medical University, 840 Shijo Cho, Kashihara, 634 8521, Japan
    Heart Vessels 23:301-7. 2008
    The aim of the present study is to clarify the roles of circulating ADAMTS13 and von Willebrand factor (VWF) in the formation of coronary artery thrombi in acute myocardial infarction (AMI)...
  84. ncbi Von Willebrand factor cleaving protease activity in the physiopathology of microangiopathic disorders
    Maria Marta Amaral
    Instituto de Investigaciones Hematologicas, Academia Nacional de Medicina, Pacheco de Melo 3081, 1425 Buenos Aires, Argentina
    Medicina (B Aires) 63:130-6. 2003
    The von Willebrand factor cleaving protease (VWFCP) modulates the von Willebrand factor (VWF) multimeric size in normal plasma. VWFCP activity levels are decreased in different physiological and pathologic situations...
  85. ncbi Plasma ADAMTS13 activity may predict early adverse events in living donor liver transplantation: observations in 3 cases
    Saiho Ko
    Department of Surgery, Nara Medical University, Kashihara, Nara, Japan
    Liver Transpl 12:859-69. 2006
    A disintegrin-like and metalloproteinase with thrombospondin type-1 motifs 13 (ADAMTS13) is a metalloproteinase that specifically cleaves the multimeric von Willebrand factor (VWF)...
  86. ncbi Unbalanced expression of ADAMTS13 and von Willebrand factor in mouse endotoxinemia
    Jun Mimuro
    Division of Cell and Molecular Medicine, Center for Molecular Medicine, Jichi Medical University, 3311 1 Yakushiji, Shimotsuke, 329 0498, Japan
    Thromb Res 122:91-7. 2008
    Secondary ADAMTS13 deficiency may occur in septic patients. The expression of ADAMTS13 in mouse endotoxinemia was studied.
  87. ncbi Therapeutic plasma exchange plus corticosteroid for the treatment of the thrombotic thrombocytopenic purpura: a single institutional experience in the southern Marmara region of Turkey
    Fahir Ozkalemkas
    Department of Hematology, Uludag University, 16059 Gorukle, Bursa, Turkey
    Transfus Apher Sci 36:109-15. 2007
    ..It suggests that considerable number of patients escaped notice. We hope that, demonstrating the successful outcome, this article serves to urge primary physicians to keep in mind the diagnosis of TTP and refer suspected cases quickly...
  88. ncbi Pancreatitis preceding acute episodes of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: report of five patients with a systematic review of published reports
    Karen K Swisher
    Hematology Oncology Section, Department of Medicine, College of Medicine, The University of Oklahoma Health Science Center, Oklahoma City, OK 73190, USA
    Haematologica 92:936-43. 2007
    ..We describe the occurrence of TTP-HUS following pancreatitis and consider whether pancreatitis may be a triggering event for acute episodes of TTP-HUS...
  89. pmc Different disparities of gender and race among the thrombotic thrombocytopenic purpura and hemolytic-uremic syndromes
    Deirdra R Terrell
    Department of Biostatistics and Epidemiology, College of Public Health, The University of Oklahoma Health Sciences Center, Oklahoma City, OK 73126 0901, USA
    Am J Hematol 85:844-7. 2010
    ..The relative frequency of women and black race among patients with idiopathic TTP and TTP-associated with severe ADAMTS13 deficiency was significantly greater than their control populations...
  90. ncbi Thrombotic thrombocytopenic purpura
    B Lammle
    Department of Hematology and Central Hematology Laboratory, Inselspital, University Hospital, Bern, Switzerland
    J Thromb Haemost 3:1663-75. 2005
    ..There remains a large research agenda to improve diagnosis of TMA, gain further insight into the pathophysiology of the various TMA and to improve and possibly tailor the management of affected patients...
  91. ncbi Treatment of thrombotic thrombocytopenic purpura
    S Fontana
    Department of Haematology and Central Haematology Laboratory, University Hospital, Inselspital, Bern, Switzerland
    Vox Sang 90:245-54. 2006
    ..Recent findings may explain its effectiveness, which may include, in particular, the removal of anti-ADAMTS13 autoantibodies and unusually large von Willebrand factor multimers and/or supply of ADAMTS13 in acquired ..
  92. ncbi Disseminated malignancy misdiagnosed as thrombotic thrombocytopenic purpura: A report of 10 patients and a systematic review of published cases
    Kristin K Francis
    Hematology Oncology Section, Department of Medicine, The University of Oklahoma Health Sciences Center, Room CHB 358, PO Box 26901, Oklahoma City, Oklahoma 73190, USA
    Oncologist 12:11-9. 2007
    ....
  93. doi Frequency and significance of HIV infection among patients diagnosed with thrombotic thrombocytopenic purpura
    Melody Benjamin
    Department of Medicine, College of Medicine, The University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma 73126 0901, USA
    Clin Infect Dis 48:1129-37. 2009
    ..Case series of patients with a diagnosis of thrombotic thrombocytopenic purpura (TTP) have reported different frequencies of human immunodeficiency virus (HIV) infection; some series suggest that HIV infection may cause TTP...
  94. ncbi Thrombotic thrombocytopenic purpura associated with von Willebrand factor-cleaving protease (ADAMTS13) deficiency in children
    Chantal Loirat
    Service de Nephrologie Pediatrique, Hopital Robert Debre, Paris, France
    Semin Thromb Hemost 32:90-7. 2006
    ..TTP in adults was most often associated with an acquired deficiency of von Willebrand factor-cleaving protease (ADAMTS13) due to autoantibodies, whereas TTP in children was most often associated with a hereditary autosomal recessive ..
  95. ncbi Outcomes in the treatment of thrombotic thrombocytopenic purpura with splenectomy: a retrospective cohort study
    Ubaldo Martinez Outschoorn
    Cardeza Foundation for Hematological Research and Department of Medicine, Thomas Jefferson University, Philadephia, PA 19107, USA
    Am J Hematol 81:895-900. 2006
    ..13-90 months). In conclusion, relapses in TTP can be managed successfully with additional PE or with splenectomy. PE dependent or refractory TTP can be successfully treated with splenectomy...
  96. doi Thrombotic thrombocytopenic purpura in systemic lupus erythematosus: risk factors and clinical outcome: a single centre study
    S K Kwok
    Division of Rheumatology, Department of Internal Medicine, School of Medicine, The Catholic University of Korea, South Korea
    Lupus 18:16-21. 2009
    ..Development of TTP in patients with SLE can be fatal. Therefore, intensive therapy will be needed especially in the presence of infection...
  97. doi Cognitive deficits after recovery from thrombotic thrombocytopenic purpura
    April S Kennedy
    Department of Medicine, College of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City, OK 73126 0901, USA
    Transfusion 49:1092-101. 2009
    ..Patients with apparent complete recovery from thrombotic thrombocytopenic purpura (TTP) often complain of problems with memory, concentration, and fatigue...
  98. doi Thrombotic thrombocytopenic purpura at the Johns Hopkins Hospital from 1992 to 2008: clinical outcomes and risk factors for relapse
    Huichun Zhan
    Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Ross Research 1032, 720 Rutland Avenue, Baltimore, MD 21205, USA
    Transfusion 50:868-74. 2010
    ..We aimed to describe the clinical spectrum of TTP, to determine the valid endpoint for plasma exchange cessation, and to explore the risk factors for disease relapse...
  99. doi Effect of blood group on idiopathic thrombotic thrombocytopenic purpura
    Lara Zuberi
    Department of Hematology Oncology, Henry Ford Hospital, Detroit, Michigan 48202, USA
    J Clin Apher 24:131-3. 2009
    Thrombotic thrombocytopenic purpura (TTP) is a condition caused by deficiency of ADAMTS13 resulting in accumulation of ultra large Von Willebrand factor multimers (ULVWF), leading to micro thrombi in multiple organs...
  100. ncbi Minimal evidence of platelet and endothelial cell reactive antibodies in thrombotic thrombocytopenic purpura
    T J Raife
    The Blood Center of Southeastern Wisconsin, Milwaukee, Wisconsin, USA
    Am J Hematol 62:82-7. 1999
    ..Our findings suggest that autoantibodies against platelets or endothelial cells are not important in the pathogenesis of this syndrome...
  101. ncbi [Paediatric case report of an acquired autoimmune thrombotic thrombocytopenic purpura]
    C Curtillet
    Service Hématologie Pédiatrique du Professeur Michel, Hôpital Timone Enfants Marseille, 264, rue Saint Pierre, 13385 Marseille Cedex 05, France
    Arch Pediatr 13:1521-4. 2006
    ..The two types of TTP are distinguishable by the presence or absence of antiprotease inhibitors, which are, respectively, either acquired or constitutional. The acquired autoimmune form is most frequently observed in adults...

Research Grants64

  1. A platelet ligand mimetic for neurothrombotic disorders
    Catherine Woods; Fiscal Year: 2006
    ..unreadable] [unreadable] [unreadable] [unreadable] [unreadable]..
  2. "Development of a Laboratory Test for TTP"
    Christopher Hillyer; Fiscal Year: 2003
    ..Recently, a metalloprotease (MP [ADAMTS13]) has been "identified" that cleaves large von Willebrand factor (vWF) protein multimers...
  3. VON WILLEBRAND FACTOR AND THE PATHOPHYSIOLOGY OF TTP
    Joel Moake; Fiscal Year: 1990
    ..We also expect that the results of our study will make possible molecular level on TTP and related disorders...
  4. X Long Zheng; Fiscal Year: 2016
    ..Acquired TTP is mainly caused by autoantibodies that inhibit ADAMTS13 enzyme. Plasma exchange is the only effective therapy available to date...
  5. Ph 3 of Cyclosporine or Corticosteroids Adjunct to Plasma Exhcange in Tx of TTP
    Haifeng M Wu; Fiscal Year: 2013
    ..investigations of TTP: 1) To determine the effect of corticosteroids and cyclosporine given individually on ADAMTS13 antigen, activity, and ADAMTS13 antibody concentration, and inhibitor titer over time using serial measurements;2)..
  6. Timothy A Springer; Fiscal Year: 2015
    ..The A1 domain in VWF binds to glycoprotein Ib1 on platelets to form a hemostatic plug. ADAMTS13 is an enzyme that cleaves the A2 domain of VWF, and by cleaving only after unfolding of the A2 domain by ..
  7. David Ginsburg; Fiscal Year: 2016
    ..In addition, the genes for 2 related disorders of VWF and FVIII were identified: ADAMTS13 as the cause of familial thrombotic thrombocytopenic purpura (TTP) and MCFD2/LMAN1 as the cause of combined ..
  8. Xiaoping Du; Fiscal Year: 2016
    ....
  9. Von Willebrand Factor Multimer Assembly and Structure
    J Evan Sadler; Fiscal Year: 2012
    ..Conversely, ADAMTS13, a metalloprotease, cleaves VWF multimers at sites of thrombosis, and ADAMTS13 deficiency causes thrombotic ..
  10. BIOMECHANICS OF UNFOLDING THE A2 DOMAIN OF VON WILLEBRAND FACTOR
    Jin Yu Shao; Fiscal Year: 2012
    ..Normal VWF function depends on its size distribution in plasma, regulated via proteolytic cleavage by ADAMTS13. On the one hand, insufficient VWF cleavage leads to thrombotic thrombocytopenic purpura, a disease characterized ..
  11. Regulation of von Willebrand Factor Reactivity
    JOSE ARON LOPEZ; Fiscal Year: 2012
    ..The studies we propose will help us learn how the new VWF is different from that VWF that has been processes and we will learn how it is processed. In this way, we will be able to understand and treat disorders of blood clotting. ..
  12. Functional analysis of ADAMTS13
    David G Motto; Fiscal Year: 2010
    ..additional environmental and/or genetic factors are required to induce TTP in the setting of murine ADAMTS 13 deficiency...
  13. WEIBEL PALADE BODY FORMATION AND ROLE IN HEMOSTASIS
    DENISA WAGNER; Fiscal Year: 2007
    ..In the third aim we will examine the role of Weibel-Palade[unreadable] bodies and ADAMTS13 in stroke and stroke recovery...
  14. Mechanical Regulation of Binding and Cleavage of VWF by ADAMTS-13
    Cheng Zhu; Fiscal Year: 2012
    ..abstract_text> ..
  15. David G Motto; Fiscal Year: 2015
    ..PHS 398/2590 (Rev. 06/09) Page Continuation Format Page ..
  16. J Evan Sadler; Fiscal Year: 2016
    ..Project 1 will characterize the molecular basis of ADAMTS13 substrate specificity, develop optimized assays to investigate the role of ADAMTS13 deficiency in thrombotic ..
  17. PLATELET AGGLUTINATION IN THROMBOTIC MICROANGIOPATHY
    ERIC LIAN; Fiscal Year: 1993
    ....
  18. MECHANISMS OF ENDOTHELIAL CELL INJURY IN TTP/HUS
    Jerome Gottschall; Fiscal Year: 1999
    ..In the process, the study will attempt to determine if there are differences in the pathogenesis of HIV-associated TTP vs. more typical TTP/HUS. ..
  19. ENDOTHELIAL GB3 SPECIES INVOLVED IN HUS PATHOGENESIS
    DAVID NEWBURG; Fiscal Year: 2003
    ..The detailed description of the expression of these endothelial cell Stx receptors will potentially lead to development of precisely targeted therapies that can reduce Stx toxicity and these devastating effects of HUS/TTP. ..
  20. Judith A James; Fiscal Year: 2016
    ..With our rich Native American heritage and large rural populations, the patients provided by the Oklahoma ACE will be previously understudied and provide unique insights for therapeutic trials. ..
  21. MOLECULAR GENETICS OF COAGULATION DISORDERS
    David Ginsburg; Fiscal Year: 2013
    ..This Project will identify key genes in this system that should provide valuable new diagnostic tools as well as suggest novel approaches to treatment. ..
  22. Transfusion Medicine/Hemostasis Core Clinical Center
    Thomas L Ortel; Fiscal Year: 2012
    ..These studies are not possible at single institutions and require the multicenter approach of the Transfusion Medicine and Hemostasis Clinical Trials Network. ..
  23. MGH Transfusion Medicine and Hemostasis Network Clinical Core Center
    David J Kuter; Fiscal Year: 2012
    ..They will also establish for the first time the appropriate treatment for uncommon diseases such as HIT, TTP, and chronic thrombocytopenia. ..
  24. Forecasters of futute and progressive CDK in patients with microvascular glomerul
    Haifeng M Wu; Fiscal Year: 2013
    ..Using these markers, we will identify patients at risk for CKD early, and treat them aggressively to prevent the CKD from occurring. ..
  25. Transfusion Medicine/Hemostasis Clinical Research Network
    Keith R McCrae; Fiscal Year: 2012
    ....
  26. Study of TTP: Incidence Rates and Risk Factors
    Charles Bennett; Fiscal Year: 2005
    ..Our study is unique and will advance scientific knowledge in this area. The findings of this study would facilitate health care professional for future clinical actions and public policy decisions. ..
  27. TTP PATHOGENESIS IN MODEL SYSTEMS IN VITRO AND IN VIVO
    David Acheson; Fiscal Year: 1999
    ..This will provide insights for the development of diagnostic, therapeutic and preventative regimens. ..
  28. PHYSICAL FORCES EFFECTS ON PLATELETS
    J Hellums; Fiscal Year: 2000
    ....
  29. THE ENDOTHELIAL CELL IN PATHOGENESIS OF TTP
    Jeffrey Laurence; Fiscal Year: 2004
    ..The model presented could also enable exploration of new therapeutic modalities for TTP, based upon modulation of specific apoptotic pathways. ..
  30. VW Factor Cleaving Prostease and Thrombotic Diseases
    DOMINIC CHUNG; Fiscal Year: 2005
    ..This protease, which has been named vWF cleaving protease (VWFCP), is a metalloprotease and specifically cleaves the Tyrl 605-MetI 606 bond in the P2 domain of the ..
  31. ASDAMTS-13 and Inflammation
    TRUNG CHANH NGUYEN; Fiscal Year: 2012
    ....
  32. Weibel-Palade Bodies: roles in thrombosis, inflammation and stroke
    Denisa D Wagner; Fiscal Year: 2013
    ..The project will consist of two interrelated Specific Aims: 1. New roles of VWF and its processing enzyme ADAMTS13 In this first aim, we propose to develop our recent observation that VWF and ADAMTS13 regulate leukocyte adhesion ..
  33. Fluorescence Fluctuation Spectroscopy for von Willebrand Factor Multimer Analysis
    Michael J Levene; Fiscal Year: 2010
    ....
  34. Chemistry and Biology of Coagulation
    Sriram Krishnaswamy; Fiscal Year: 2013
    ..investigates the link between coagulation and von Willebrand Factor processing by investigating the regulation of ADAMTS13 function by factor VIII and shear stress...
  35. CHAR MARIE WITMER; Fiscal Year: 2014
    ..Proteolytic cleavage of these UL-VWF multimers by ADAMTS13 metalloprotease decreases VWF function...
  36. FASEB SRC on Proteases in Hemostasis and Vascular Biology
    JAMES ANDREW HUNTINGTON; Fiscal Year: 2013
    ....
  37. JOSE ARON LOPEZ; Fiscal Year: 2016
    ..that VWF oxidation by neutrophil oxidants increases its platelet binding functions and renders it resistant to ADAMTS13 cleavage, and c) a study demonstrating that N-acetylcysteine (NAC), an antioxidant drug, decreases VWF size and ..
  38. Regulation of von Willebrand factor processing
    Trisha N Davis; Fiscal Year: 2011
    ..are proteolytically processed to a less polymerized form in a shear-dependent manner by the metalloprotease ADAMTS13 in plasma...
  39. Blood Dyscrasias and Transfusion Medicine
    Gilbert C White; Fiscal Year: 2010
    ..Topics will include immunohematology, integrin biology, von Willebrand factor/ADAMTS13, transfusion medicine, and stem cell biology...
  40. Inhibitors of ADAMTS313 and Outcome of TTP
    Han Mou Tsai; Fiscal Year: 2004
    ..advances in biochemical and genetic studies have demonstrated that deficiency of a novel zinc metalloprotease, ADAMTS13, due to autoimmune inhibitors or genetic mutations, causes TTP...
  41. FASL IN PREECLAMPSIA AND IMMUNOTOLERANCE TO THE FETUS
    Scott Kauma; Fiscal Year: 2002
    ..These studies will significantly advance our understanding of the mechanisms involved in the pathogenesis of preeclampsia and provide new rationales for the treatment of this disease. ..
  42. Keith L Kirkwood; Fiscal Year: 2015
    ....
  43. Intensive Smoking Cessation Invervention for Head and Neck Cancer Patients
    JANICE ANITA BLALOCK; Fiscal Year: 2012
    ..If ACT is found to be more effective, it could be further tested in larger studies. ..
  44. Folate Status, Genomic Uracil, and the Balance of Base Excision Repair Activity
    Michael D Wyatt; Fiscal Year: 2011
    ..We will investigate the links between BER and folic acid that define the seemingly contradictory pro- and anticancer effects of each. ..
  45. Robert Paine; Fiscal Year: 2016
    ..Thus, they have the potential to provide new therapeutic approaches to restore alveolar epithelial integrity while limiting unintended adverse effects. ..
  46. Elizabeth E Hatch; Fiscal Year: 2014
    ..Identifying modifiable risk factors for infertility and miscarriage is an important public health goal, especially because the effectiveness of treatments for these conditions is relatively poor. ..
  47. Bonnie B Blomberg; Fiscal Year: 2014
    ....
  48. A role for miRNAs in adenosine-dependent alternative macrophage activation
    Samuel Joseph Leibovich; Fiscal Year: 2013
    ..These studies should provide novel insights into differential gene regulation in macrophages under conditions that induce "M2d" activation. ..
  49. Post-Transcriptional Regulation in Colorectal Cancer
    DAN ALAN DIXON; Fiscal Year: 2013
    ....
  50. Jianguo Liu; Fiscal Year: 2016
    ....
  51. Determinants of Efficacy to the Akt Inhibitor Perifosine in Colorectal Cancer
    Cathy Eng; Fiscal Year: 2012
    ..Endpoint: Pharma- codynamic biomarker changes will correlate with efficacy of perifosine therapy. ..
  52. Post-transcriptional control of inflammatory gene expression
    Thomas A Hamilton; Fiscal Year: 2013
    ..This proposal is focused upon understanding the mechanisms through which IL-17 (and other stimuli) controls degradation of specific inflammation related messenger RNAs and thereby modulates aspects of inflammatory response. ..
  53. Mechanisms of normal and abnormal platelet homeostasis
    Joel S Bennett; Fiscal Year: 2010
    ..The projects are supported by a Bioinformatics Core to store and analyze clinical data and by a Core for Administration. ..
  54. SILVIA BEATRIZ RAMOS; Fiscal Year: 2016
    ..Ramos's research and career development. The research environment will provide a productive, collegial, and collaborative atmosphere ideal for pursuing the above research and training goals. ..
  55. Ionizing Radiation Control of Inflammatory Cytokine mRNA Stability and Expression
    Robert Schneider; Fiscal Year: 2013
    ..abstract_text> ..
  56. Mitochondrial Toxicity of Antiviral Nucleosides
    Edward E McKee; Fiscal Year: 2010
    ..This information is very important in developing strategies for treating these toxicities and in the rational design of new drugs with less toxic side-effects. ..
  57. Lauren A Wise; Fiscal Year: 2014
    ..However, the feasibility of these methods remains to be tested in the United States. Such a design could have far-ranging implications for the practice of epidemiology. ..
  58. FAOUD TERRENCE ISHMAEL; Fiscal Year: 2014
    ..This work will promote the scientific development of the candidate and increase the understanding of PTR in the pathogenesis of asthmatic inflammation and GC-mechanisms, opening the door for discovery of new anti- inflammatory targets. ..
  59. Functions of Tocopherol Transfer Protein
    Danny Manor; Fiscal Year: 2012
    ..TTP is the only known protein that specifically regulates the levels of vitamin E in the body. We propose to determine what exactly TTP does in liver cells, and how mutations in TTP that are found in humans affect its activity. ..
  60. Novel HBV Therapeutic with Potential for Treatment of Drug-Resistant Infection
    Andrea Cuconati; Fiscal Year: 2013
    ..Concurrent with the above aims, we will 7) attempt to identify the precise molecular target of the TTPs through affinity studies. At the end of Phase 2, a TTP compound will be ready for IND filing and eventual clinical trials. ..
  61. Histopathologic and Immunohistochemical Features of Tissue Adherent to the Electr
    CONSTANTINOS THASOS SOFOCLEOUS; Fiscal Year: 2011
    ..This information is vital in the treatment of a large population with CRHM and may impact the overall population of cancer patients treated with RFA. ..
  62. The role of tristetraprolin in head and neck cancer progression
    ELIZABETH ANNE VAN TUBERGEN; Fiscal Year: 2012
    ....
  63. LISA HELENE BUTTERFIELD; Fiscal Year: 2014
    ..Rational design of vaccines, based on this new information should allow for more rational design of the immune activation to be achieved by newer vaccines. ..