Adamts13

Summary

Gene Symbol: Adamts13
Description: a disintegrin-like and metallopeptidase (reprolysin type) with thrombospondin type 1 motif, 13
Alias: ADAM-TS13, ADAMTS-13, Gm710, vWF-CP, A disintegrin and metalloproteinase with thrombospondin motifs 13, a disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 13, vWF-CP mRNA for von Willebrand factor-cleaving, vWF-cleaving protease, von Willebrand factor-cleaving protease
Species: mouse

Top Publications

  1. pmc The combined roles of ADAMTS13 and VWF in murine models of TTP, endotoxemia, and thrombosis
    Anil K Chauhan
    CBR Institute for Biomedical Research, Department of Pathology, Harvard Medical School, Boston, MA, USA
    Blood 111:3452-7. 2008
  2. pmc Shiga toxin B subunits induce VWF secretion by human endothelial cells and thrombotic microangiopathy in ADAMTS13-deficient mice
    Jing Huang
    Departments of Medicine, Biochemistry, and Molecular Biophysics, Washington University School of Medicine, St Louis, MO 63110, USA
    Blood 116:3653-9. 2010
  3. 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
  4. pmc Systemic antithrombotic effects of ADAMTS13
    Anil K Chauhan
    CBR Institute for Biomedical Research and Department of Pathology, Harvard Medical School, Boston, MA 02115, USA
    J Exp Med 203:767-76. 2006
  5. pmc Shigatoxin triggers thrombotic thrombocytopenic purpura in genetically susceptible ADAMTS13-deficient mice
    David G Motto
    Department of Pediatrics, University of Michigan, Ann Arbor, Michigan 48109, USA
    J Clin Invest 115:2752-61. 2005
  6. ncbi Complete deficiency in ADAMTS13 is prothrombotic, but it alone is not sufficient to cause thrombotic thrombocytopenic purpura
    Fumiaki Banno
    National Cardiovascular Center Research Institute, 5 7 1 Fujishirodai, Suita, Osaka 565 8565, Japan
    Blood 107:3161-6. 2006
  7. pmc ADAMTS13 is expressed in hepatic stellate cells
    Wenhua Zhou
    Division of Hematology, Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, NY 10467, USA
    Lab Invest 85:780-8. 2005
  8. pmc Probing ADAMTS13 substrate specificity using phage display
    Karl C Desch
    Department of Pediatrics, University of Michigan, Ann Arbor, Michigan, United States of America
    PLoS ONE 10:e0122931. 2015
  9. pmc Nxf1 natural variant E610G is a semi-dominant suppressor of IAP-induced RNA processing defects
    Dorothy Concepcion
    Department of Cellular and Molecular Medicine, Moores UCSD Cancer Center and Institute for Genomic Medicine, University of California, San Diego School of Medicine, La Jolla, California, United States of America Department of Medicine, University of California, San Diego School of Medicine, La Jolla, California, United States of America
    PLoS Genet 11:e1005123. 2015
  10. doi ADAMTS13 deficiency in mice does not affect adipose tissue development
    Lotte Geys
    Center for Molecular and Vascular Biology, KU Leuven, Leuven, Belgium
    Biochim Biophys Acta 1850:1368-74. 2015

Scientific Experts

  • Masayuki Fujioka
  • T Raife
  • Jing Fei Dong
  • Toshiyuki Miyata
  • Jun Mimuro
  • Fumiaki Banno
  • Anil K Chauhan
  • Friedrich Scheiflinger
  • X Long Zheng
  • David G Motto
  • Sheng Yu Jin
  • Denisa D Wagner
  • Juan Xiao
  • Chintan Gandhi
  • Ramesh Tati
  • David Ginsburg
  • Jialing Bao
  • Simon F De Meyer
  • Hanspeter Rottensteiner
  • Alexandra Schiviz
  • Karl C Desch
  • B De Maeyer
  • Wenhua Zhou
  • Frederik Denorme
  • Gungor Cagdas Dincel
  • Karen Vanhoorelbeke
  • E De Cock
  • Johanne Dubail
  • Lotte Geys
  • Brandy Pickens
  • Dorothy Concepcion
  • Alexander T Bauer
  • Yingying Mao
  • C Gandhi
  • Yoko Morioka
  • Ayse Metin
  • Han Mou Tsai
  • A K Chauhan
  • N Vandeputte
  • S F De Meyer
  • K Vanhoorelbeke
  • H Deckmyn
  • Diana Karpman
  • Barbara Dietrich
  • Cecile V Denis
  • Atsuko Igari
  • Tania N Petruzziello-Pellegrini
  • M M Khan
  • Olivier D Christophe
  • Steven R Lentz
  • David Motto
  • Klilah Hershko
  • Peter J Lenting
  • Anne lie Ståhl
  • Ann Charlotte Kristoffersson
  • Miroslaw Rybaltowski
  • Barbara Plaimauer
  • Junmei Chen
  • Suneel S Apte
  • Jing Huang
  • Julie Rayes
  • Pablo Laje
  • Bing Qiao Zhao
  • D G Motto
  • Myung Chul Chung
  • Meghan T Walsh
  • Michael Dockal
  • Bon Hun Koo
  • Guojing Zhu
  • Eric E Bouhassira
  • Colin B Lamb
  • K Bruno
  • Aline Vandenbulcke
  • Olivier Francois
  • Christoph Kleinschnitz
  • Hasan Tarik Atmaca
  • Hans Deckmyn
  • Tommy Andersson
  • Linda Desender
  • Friederike Langhauser
  • Philipp Beckhove
  • Kristin Metzger
  • J De Raeymaecker
  • Markus Hecker
  • Nidhi Agrawal
  • Henri Roger Lijnen
  • Kathrin Frank
  • Elien Roose
  • Ilse Scroyen
  • Mortimer Poncz

Detail Information

Publications56

  1. pmc The combined roles of ADAMTS13 and VWF in murine models of TTP, endotoxemia, and thrombosis
    Anil K Chauhan
    CBR Institute for Biomedical Research, Department of Pathology, Harvard Medical School, Boston, MA, USA
    Blood 111:3452-7. 2008
    ..Therefore, to examine directly the requirement for VWF in TTP pathogenesis, we generated ADAMTS13-deficient mice on a TTP-susceptible genetic background that were also either haploinsufficient (Vwf+/-) or ..
  2. pmc Shiga toxin B subunits induce VWF secretion by human endothelial cells and thrombotic microangiopathy in ADAMTS13-deficient mice
    Jing Huang
    Departments of Medicine, Biochemistry, and Molecular Biophysics, Washington University School of Medicine, St Louis, MO 63110, USA
    Blood 116:3653-9. 2010
    ..human endothelial cells and causes thrombotic thrombocytopenic purpura, a disease with similarities to D+HUS, in Adamts13(-/-) mice...
  3. 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 ..
  4. pmc Systemic antithrombotic effects of ADAMTS13
    Anil K Chauhan
    CBR Institute for Biomedical Research and Department of Pathology, Harvard Medical School, Boston, MA 02115, USA
    J Exp Med 203:767-76. 2006
    The metalloprotease ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type I repeats 13) cleaves highly adhesive large von Willebrand factor (VWF) multimers after their release from the endothelium...
  5. pmc Shigatoxin triggers thrombotic thrombocytopenic purpura in genetically susceptible ADAMTS13-deficient mice
    David G Motto
    Department of Pediatrics, University of Michigan, Ann Arbor, Michigan 48109, USA
    J Clin Invest 115:2752-61. 2005
    ..thrombocytopenic purpura (TTP) is a life-threatening illness caused by deficiency of the vWF-cleaving protease ADAMTS13. Here we show that ADAMTS13-deficient mice are viable and exhibit normal survival, although vWF-mediated platelet-..
  6. ncbi Complete deficiency in ADAMTS13 is prothrombotic, but it alone is not sufficient to cause thrombotic thrombocytopenic purpura
    Fumiaki Banno
    National Cardiovascular Center Research Institute, 5 7 1 Fujishirodai, Suita, Osaka 565 8565, Japan
    Blood 107:3161-6. 2006
    b>ADAMTS13 is a plasma metalloproteinase that regulates platelet adhesion and aggregation through cleavage of von Willebrand factor (VWF) multimers...
  7. pmc ADAMTS13 is expressed in hepatic stellate cells
    Wenhua Zhou
    Division of Hematology, Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, NY 10467, USA
    Lab Invest 85:780-8. 2005
    b>ADAMTS13 is a circulating zinc metalloprotease that cleaves the hemostatic glycoprotein von Willebrand factor (VWF) in a shear-dependent manner...
  8. pmc Probing ADAMTS13 substrate specificity using phage display
    Karl C Desch
    Department of Pediatrics, University of Michigan, Ann Arbor, Michigan, United States of America
    PLoS ONE 10:e0122931. 2015
    ..VWF correlates with the length of VWF multimers, which is proteolytically controlled by the metalloprotease ADAMTS13. To probe ADAMTS13 substrate specificity, we created phage display libraries containing randomly mutated residues ..
  9. pmc Nxf1 natural variant E610G is a semi-dominant suppressor of IAP-induced RNA processing defects
    Dorothy Concepcion
    Department of Cellular and Molecular Medicine, Moores UCSD Cancer Center and Institute for Genomic Medicine, University of California, San Diego School of Medicine, La Jolla, California, United States of America Department of Medicine, University of California, San Diego School of Medicine, La Jolla, California, United States of America
    PLoS Genet 11:e1005123. 2015
    ..Strikingly, CRISPR/Cas9-mediated genome editing demonstrates that a single amino acid substitution in Nxf1, E610G, is sufficient to recreate a quantitative genetic modifier in a co-isogenic background. ..
  10. doi ADAMTS13 deficiency in mice does not affect adipose tissue development
    Lotte Geys
    Center for Molecular and Vascular Biology, KU Leuven, Leuven, Belgium
    Biochim Biophys Acta 1850:1368-74. 2015
    BMI and ADAMTS13 levels are positively correlated in man. Development of obesity is associated with angiogenesis and inflammation, and increased ADAMTS13 synthesis in the liver.
  11. doi ADAMTS13-mediated thrombolysis of t-PA-resistant occlusions in ischemic stroke in mice
    Frederik Denorme
    Laboratory for Thrombosis Research, KU Leuven Campus Kulak Kortrijk, Kortrijk, Belgium
    Blood 127:2337-45. 2016
    ..3% ± 10.1% VWF, and this was inversely correlated with thrombus red blood cell content. We hypothesized that ADAMTS13 can exert a thrombolytic effect in VWF-containing thrombi in the setting of stroke...
  12. pmc Platelet-delivered ADAMTS13 inhibits arterial thrombosis and prevents thrombotic thrombocytopenic purpura in murine models
    Brandy Pickens
    Department of Pathology and Department of Laboratory Medicine, The University of Pennsylvania, Philadelphia, PA
    Blood 125:3326-34. 2015
    b>ADAMTS13 metalloprotease cleaves von Willebrand factor (VWF), thereby inhibiting platelet aggregation and arterial thrombosis...
  13. doi Insights on ADAMTS proteases and ADAMTS-like proteins from mammalian genetics
    Johanne Dubail
    Cleveland Clinic Lerner Research Institute, Cleveland, OH, USA
    Matrix Biol 44:24-37. 2015
    ..The clinical manifestations of Mendelian disorders resulting from mutations in ADAMTS2, ADAMTS10, ADAMTS13, ADAMTS17, ADAMTSL2 and ADAMTSL4 identified essential roles for each gene, but also suggested potential ..
  14. doi The novel ADAMTS13-p.D187H mutation impairs ADAMTS13 activity and secretion and contributes to thrombotic thrombocytopenic purpura in mice
    E De Cock
    Laboratory for Thrombosis Research, KU Leuven Kulak, Kortrijk, Belgium
    J Thromb Haemost 13:283-92. 2015
    Congenital thrombotic thrombocytopenic purpura (TTP) is characterized by mutations in the ADAMTS13 gene, which either impair protein secretion or influence ADAMTS13 (A Disintegrin-like And Metalloprotease domain with ThromboSpondin type-1 ..
  15. doi Expression of a structurally constrained von Willebrand factor variant triggers acute thrombotic thrombocytopenic purpura in mice
    Yoko Morioka
    Kyoto University, Career Path Unit for Young Life Scientists, Kyoto, Japan
    Blood 123:3344-53. 2014
    ..deficiency in plasma protease a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13), the enzyme responsible for cleaving ultra-large von Willebrand factor (VWF) multimers into nonthrombogenic ..
  16. doi Increased expressions of ADAMTS-13 and apoptosis contribute to neuropathology during Toxoplasma gondii encephalitis in mice
    Gungor Cagdas Dincel
    Gumushane University, Siran Mustafa Beyaz Vocational High School, Gümüşhane, Turkey
    Neuropathology 36:211-26. 2016
    ..To the best of the authors' knowledge, this is the first report on ADAMTS-13 expression in the CNS of T. gondii-infected mice. ..
  17. pmc Carboxyl terminus of ADAMTS13 directly inhibits platelet aggregation and ultra large von Willebrand factor string formation under flow in a free-thiol-dependent manner
    Jialing Bao
    From the Department of Pathology and Laboratory Medicine, The Children s Hospital of Philadelphia J B, J X, Y M, X L Z The University of Pennsylvania Perelman School of Medicine, Philadelphia X L Z and the Department of Obstetrics and Gynecology, Tongji Hospital, Huazhong University of Science and Technology, Wuhan, China J X, X L Z
    Arterioscler Thromb Vasc Biol 34:397-407. 2014
    b>ADAMTS13 (A Disintegrin And Metalloprotease with Thrombospondin type 1 repeats, 13) cleaves von Willebrand factor (VWF), thereby inhibiting thrombus formation...
  18. doi ADAMTS13 modulates atherosclerotic plaque progression in mice via a VWF-dependent mechanism
    C Gandhi
    Department of Internal Medicine, University of Iowa, Iowa City, IA, USA
    J Thromb Haemost 12:255-60. 2014
    b>ADAMTS13 reduces the adhesiveness of hyperactive ultra-large von Willebrand factor (ULVWF) multimers by cleaving them into smaller, less active multimers...
  19. doi Congenital thrombotic thrombocytopenic purpura with novel mutations in three unrelated Turkish children
    Ayse Metin
    Division of Pediatric Immunology and Allergy, Ankara Children s Hematology Oncology Training and Research Hospital, Ministry of Health, Ankara, Turkey
    Pediatr Blood Cancer 61:558-61. 2014
    Congenital thrombotic thrombocytopenic purpura (TTP) is an inherited disease caused by mutations in the ADAMTS 13 gene and has been reported to have diverse ages of presentation, ranging from the newborn period to adulthood...
  20. pmc The CXCR4/CXCR7/SDF-1 pathway contributes to the pathogenesis of Shiga toxin-associated hemolytic uremic syndrome in humans and mice
    Tania N Petruzziello-Pellegrini
    Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada
    J Clin Invest 122:759-76. 2012
    ..Collectively, these data implicate the CXCR4/CXCR7/SDF-1 pathway in Stx-mediated pathogenesis and suggest novel therapeutic strategies for prevention and/or treatment of complications associated with E. coli O157:H7 infection...
  21. pmc von Willebrand factor fibers promote cancer-associated platelet aggregation in malignant melanoma of mice and humans
    Alexander T Bauer
    Experimental Dermatology, Medical Faculty Mannheim, University of Heidelberg, Mannheim, Germany
    Blood 125:3153-63. 2015
    ..that a promoted VWF release combined with a local inhibition of proteolytic activity and protein expression of ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type I repeats 13) accounts for this procoagulatory ..
  22. pmc Complement activation associated with ADAMTS13 deficiency in human and murine thrombotic microangiopathy
    Ramesh Tati
    Department of Pediatrics, Clinical Sciences Lund, Lund University, 22185 Lund, Sweden
    J Immunol 191:2184-93. 2013
    This study addressed the contribution of ADAMTS13 deficiency to complement activation in thrombotic thrombocytopenic purpura (TTP). Renal tissue and blood samples were available from 12 TTP patients...
  23. pmc AAV-mediated expression of an ADAMTS13 variant prevents shigatoxin-induced thrombotic thrombocytopenic purpura
    Sheng Yu Jin
    Department of Pathology and Laboratory Medicine, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Blood 121:3825-9, S1-3. 2013
    Severe deficiency of plasma ADAMTS13 activity causes thrombotic thrombocytopenic purpura (TTP), a life-threatening syndrome for which plasma is the only effective therapy currently available...
  24. pmc Cyclosporin A impairs the secretion and activity of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeat)
    Klilah Hershko
    Center for Biologics Evaluation and Research, Food and Drug Administration, Bethesda, Maryland 20982, USA
    J Biol Chem 287:44361-71. 2012
    The protease ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeat) cleaves multimers of von Willebrand factor, thus regulating platelet aggregation...
  25. pmc ADAMTS13 deficiency exacerbates VWF-dependent acute myocardial ischemia/reperfusion injury in mice
    Chintan Gandhi
    Department of Internal Medicine, University of Iowa, Iowa City, IA 52242, USA
    Blood 120:5224-30. 2012
    Epidemiologic studies suggest that elevated VWF levels and reduced ADAMTS13 activity in the plasma are risk factors for myocardial infarction...
  26. 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 ..
  27. doi Identification of epitopes on ADAMTS13 recognized by a panel of monoclonal antibodies with functional or non-functional effects on catalytic activity
    Atsuko Igari
    Laboratory Medicine, Keio University School of Medicine, Tokyo, Japan
    Thromb Res 130:e79-83. 2012
    von Willebrand factor (VWF) cleavage by ADAMTS13 is mediated by multi-step interactions between their multi-domain structures...
  28. pmc ADAMTS13 reduces VWF-mediated acute inflammation following focal cerebral ischemia in mice
    M M Khan
    Department of Internal Medicine, University of Iowa, Iowa City, IA 52242, USA
    J Thromb Haemost 10:1665-71. 2012
    b>ADAMTS13 cleaves hyperactive ultra-large von Willebrand factor (ULVWF) multimers into smaller and less active forms...
  29. pmc Genetic ablation of Adamts13 gene dramatically accelerates the formation of early atherosclerosis in a murine model
    Sheng Yu Jin
    Department of Pathology and Laboratory Medicine, The Children s Hospital of Philadelphia, 34th St and Civic Center Blvd, 816G ARC, Philadelphia, PA 19104, USA
    Arterioscler Thromb Vasc Biol 32:1817-23. 2012
    b>ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats-13) cleaves von Willebrand factor, thereby modulating thrombosis and inflammation...
  30. doi A new mouse model mimicking thrombotic thrombocytopenic purpura: correction of symptoms by recombinant human ADAMTS13
    Alexandra Schiviz
    Department of Preclinical Pharmacology and Toxicology, Baxter Innovations GmbH, Industriestrasse 67, Vienna, Austria
    Blood 119:6128-35. 2012
    Deficiency of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13), a VWF-cleaving protease, is the key factor in the pathogenesis of thrombotic thrombocytopenic purpura (TTP), a life-threatening ..
  31. doi ADAMTS13 gene deletion enhances plasma high-mobility group box1 elevation and neuroinflammation in brain ischemia-reperfusion injury
    Masayuki Fujioka
    Department of Neuropharmacology, Faculty of Pharmaceutical Sciences, Fukuoka University, Fukuoka, Japan
    Neurol Sci 33:1107-15. 2012
    ..A disintegrin and metalloproteinase with thrombospondin type-1 motifs 13 (ADAMTS13) cleaves the VWF multimer strand and reduces its prothrombotic and proinflammatory functions...
  32. pmc ADAMTS13 reduces vascular inflammation and the development of early atherosclerosis in mice
    Chintan Gandhi
    Department of Internal Medicine, University of Iowa, Iowa City, USA
    Blood 119:2385-91. 2012
    b>ADAMTS13, a metalloprotease, plays a pivotal role in preventing spontaneous microvascular thrombosis by cleaving hyperactive ultra large von Willebrand factor multimers into smaller, less active multimers...
  33. pmc The distal carboxyl-terminal domains of ADAMTS13 are required for regulation of in vivo thrombus formation
    Fumiaki Banno
    Research Institute, National Cardiovascular Center, Suita, Japan
    Blood 113:5323-9. 2009
    b>ADAMTS13 is a multidomain protease that limits platelet thrombogenesis through the cleavage of von Willebrand factor (VWF)...
  34. 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.
  35. doi Degradation of circulating von Willebrand factor and its regulator ADAMTS13 implicates secreted Bacillus anthracis metalloproteases in anthrax consumptive coagulopathy
    Myung Chul Chung
    National Center for Biodefense and Infectious Diseases, College of Sciences, George Mason University, 10900 University Boulevard, Manassas, VA 20110, USA
    J Biol Chem 283:9531-42. 2008
    ..that anthrax infection modulates the activity of von Willebrand factor (VWF) and its endogenous regulator ADAMTS13, which play important roles in hemostasis and thrombosis, including interaction of endothelial cells with ..
  36. pmc ADAMTS13: a new link between thrombosis and inflammation
    Anil K Chauhan
    Immune Disease Institute, Harvard Medical School, Boston, MA 02115, USA
    J Exp Med 205:2065-74. 2008
    ..VWF) levels are elevated and a disintegrin-like and metalloprotease with thrombospondin type I repeats-13 (ADAMTS13) activity is decreased in both acute and chronic inflammation...
  37. ncbi Identification of strain-specific variants of mouse Adamts13 gene encoding von Willebrand factor-cleaving protease
    Fumiaki Banno
    National Cardiovascular Center Research Institute, Osaka 565 8565, Japan
    J Biol Chem 279:30896-903. 2004
    Human ADAMTS13 was recently identified as a gene encoding von Willebrand factor-cleaving protease, hADAMTS13. Both congenital and acquired defects in this enzyme can cause thrombotic thrombocytopenic purpura...
  38. ncbi ADAMTS-like 2 (ADAMTSL2) is a secreted glycoprotein that is widely expressed during mouse embryogenesis and is regulated during skeletal myogenesis
    Bon Hun Koo
    Department of Biomedical Engineering and Orthopaedic Research Center, Lerner Research Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195, USA
    Matrix Biol 26:431-41. 2007
    ..Furthermore, activation of the key myogenic determinant MyoD in 10T1/2 fibroblasts also triggered expression of Adamtsl2 mRNA. Collectively, the data suggest that induction of Adamtsl2 mRNA is an integral feature of myogenesis...
  39. pmc An IAP retrotransposon in the mouse ADAMTS13 gene creates ADAMTS13 variant proteins that are less effective in cleaving von Willebrand factor multimers
    Wenhua Zhou
    Division of Hematology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY 10467, USA
    Blood 110:886-93. 2007
    Severe deficiency of ADAMTS13, a von Willebrand factor (VWF)-cleaving metalloprotease, causes thrombotic thrombocytopenic purpura...
  40. ncbi ADAMTS13 assays and ADAMTS13-deficient mice
    Toshiyuki Miyata
    National Cardiovascular Center Research Institute, Fujishirodai, Suita, Osaka, Japan
    Curr Opin Hematol 14:277-83. 2007
    ..can be induced by acquired or congenital deficiency of the plasma von Willebrand factor-cleaving protease, ADAMTS13. Measurement of ADAMTS13 activity is important for the diagnosis and treatment of microangiopathies including ..
  41. ncbi Structural and functional correlation of ADAMTS13
    Jing Fei Dong
    Thrombosis Research Section, Baylor College of Medicine, Houston, TX 77030, USA
    Curr Opin Hematol 14:270-6. 2007
    b>ADAMTS13 represents a landmark in a journey that began over 80 years ago with a single clinical case. Thrombotic thrombocytopenic purpura exemplifies how von Willebrand factor can be responsible for life-threatening thrombosis...
  42. ncbi Cloning, expression and functional characterization of the full-length murine ADAMTS13
    K Bruno
    Baxter BioScience, Vienna, Austria
    J Thromb Haemost 3:1064-73. 2005
    ..deficiency or absence of the human von Willebrand factor (VWF)-cleaving protease (VWF-cp), recently termed ADAMTS13, has been shown to cause acquired and congenital thrombotic thrombocytopenic purpura (TTP), respectively...
  43. doi The distal carboxyterminal domains of murine ADAMTS13 influence proteolysis of platelet-decorated VWF strings in vivo
    B De Maeyer
    Laboratory for Thrombosis Research, K U Leuven Campus Kortrijk, Kortrijk, Belgium
    J Thromb Haemost 8:2305-12. 2010
    The multidomain metalloprotease ADAMTS13 regulates the size of von Willebrand factor (VWF) multimers upon their release from endothelial cells...
  44. pmc Correction of murine ADAMTS13 deficiency by hematopoietic progenitor cell-mediated gene therapy
    Pablo Laje
    Department of General Surgery, The Children s Hospital of Philadelphia and The University of Pennsylvania Medical Center, Philadelphia, PA 19104, USA
    Blood 113:2172-80. 2009
    b>ADAMTS13, a metalloprotease primarily synthesized in liver and endothelial cells, cleaves von Willebrand factor (VWF) at the central A2 domain, thereby reducing the sizes of circulating VWF multimers...
  45. pmc von Willebrand factor-cleaving protease ADAMTS13 reduces ischemic brain injury in experimental stroke
    Bing Qiao Zhao
    Immune Disease Institute, Boston, MA 02115, USA
    Blood 114:3329-34. 2009
    ..The activity of VWF is modulated by ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type I repeats-13) that cleaves VWF to smaller less-..
  46. doi ADAMTS13 gene deletion aggravates ischemic brain damage: a possible neuroprotective role of ADAMTS13 by ameliorating postischemic hypoperfusion
    Masayuki Fujioka
    Department of Emergency and Critical Care Medicine, Nara Medical University, 840 Shijo Cho, Kashihara, Nara, Japan
    Blood 115:1650-3. 2010
    ..Because ADAMTS13 cleaves VWF and limits platelet-dependent thrombus growth, ADAMTS13 may ameliorate ischemic brain damage in acute ..
  47. doi The function of ADAMTS13 in thrombogenesis in vivo: insights from mutant mice
    Fumiaki Banno
    National Cardiovascular Center Research Institute, 5 7 1 Fujishirodai, Suita, Osaka, 565 8565, Japan
    Int J Hematol 91:30-5. 2010
    Recently, two independent groups have established ADAMTS13-deficient mice using gene-targeting techniques...
  48. doi Mutation and ADAMTS13-dependent modulation of disease severity in a mouse model for von Willebrand disease type 2B
    Julie Rayes
    Inserm U770 and Universite Paris Sud, Le Kremlin Bicetre, France
    Blood 115:4870-7. 2010
    ..Loss of large multimers was observed in 50% of the mice. The role of ADAMTS13 was investigated by expressing both mutants in VWF/ADAMTS13 double-deficient mice...
  49. pmc Essential domains of a disintegrin and metalloprotease with thrombospondin type 1 repeats-13 metalloprotease required for modulation of arterial thrombosis
    Juan Xiao
    Department of Pathology and Laboratory Medicine, Children s Hospital of Philadelphia, PA 19104, USA
    Arterioscler Thromb Vasc Biol 31:2261-9. 2011
    A disintegrin and metalloprotease with thrombospondin type 1 repeats-13 (ADAMTS13) inhibits platelet aggregation and arterial thrombosis by cleavage of von Willebrand factor...
  50. pmc Phenotypic expression of ADAMTS13 in glomerular endothelial cells
    Ramesh Tati
    Department of Pediatrics, Clinical Sciences Lund, Lund University, Lund, Sweden
    PLoS ONE 6:e21587. 2011
    b>ADAMTS13 is the physiological von Willebrand factor (VWF)-cleaving protease...
  51. doi In vivo imaging analysis of the interaction between unusually large von Willebrand factor multimers and platelets on the surface of vascular wall
    Miroslaw Rybaltowski
    Department of Physiology, Hamamatsu University School of Medicine, Handa yama, Higashi ku, Hamamatsu, Japan
    Pflugers Arch 461:623-33. 2011
    ..the protease that cleaves UL-VWF, a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13-/-)...
  52. pmc N-acetylcysteine reduces the size and activity of von Willebrand factor in human plasma and mice
    Junmei Chen
    Puget Sound Blood Center, 921 Terry Ave, Seattle, Washington 98104, USA
    J Clin Invest 121:593-603. 2011
    ..These multimers accumulate because of a deficiency of the processing enzyme ADAMTS13. vWF protein forms long multimers from homodimers that first form through C-terminal disulfide bonds and then ..