Genomes and Genes
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
- The combined roles of ADAMTS13 and VWF in murine models of TTP, endotoxemia, and thrombosisAnil 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 ..
- Shiga toxin B subunits induce VWF secretion by human endothelial cells and thrombotic microangiopathy in ADAMTS13-deficient miceJing 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...
- Leukocyte proteases cleave von Willebrand factor at or near the ADAMTS13 cleavage siteThomas 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 ..
- Systemic antithrombotic effects of ADAMTS13Anil K Chauhan
CBR Institute for Biomedical Research and Department of Pathology, Harvard Medical School, Boston, MA 02115, USA
J Exp Med 203:767-76. 2006The 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...
- Shigatoxin triggers thrombotic thrombocytopenic purpura in genetically susceptible ADAMTS13-deficient miceDavid 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-..
- Complete deficiency in ADAMTS13 is prothrombotic, but it alone is not sufficient to cause thrombotic thrombocytopenic purpuraFumiaki Banno
National Cardiovascular Center Research Institute, 5 7 1 Fujishirodai, Suita, Osaka 565 8565, Japan
Blood 107:3161-6. 2006b>ADAMTS13 is a plasma metalloproteinase that regulates platelet adhesion and aggregation through cleavage of von Willebrand factor (VWF) multimers...
- ADAMTS13 is expressed in hepatic stellate cellsWenhua Zhou
Division of Hematology, Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, NY 10467, USA
Lab Invest 85:780-8. 2005b>ADAMTS13 is a circulating zinc metalloprotease that cleaves the hemostatic glycoprotein von Willebrand factor (VWF) in a shear-dependent manner...
- Probing ADAMTS13 substrate specificity using phage displayKarl 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 ..
- Nxf1 natural variant E610G is a semi-dominant suppressor of IAP-induced RNA processing defectsDorothy 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. ..
- ADAMTS13 deficiency in mice does not affect adipose tissue developmentLotte Geys
Center for Molecular and Vascular Biology, KU Leuven, Leuven, Belgium
Biochim Biophys Acta 1850:1368-74. 2015BMI and ADAMTS13 levels are positively correlated in man. Development of obesity is associated with angiogenesis and inflammation, and increased ADAMTS13 synthesis in the liver.
- ADAMTS13-mediated thrombolysis of t-PA-resistant occlusions in ischemic stroke in miceFrederik 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...
- Platelet-delivered ADAMTS13 inhibits arterial thrombosis and prevents thrombotic thrombocytopenic purpura in murine modelsBrandy Pickens
Department of Pathology and Department of Laboratory Medicine, The University of Pennsylvania, Philadelphia, PA
Blood 125:3326-34. 2015b>ADAMTS13 metalloprotease cleaves von Willebrand factor (VWF), thereby inhibiting platelet aggregation and arterial thrombosis...
- Insights on ADAMTS proteases and ADAMTS-like proteins from mammalian geneticsJohanne 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 ..
- The novel ADAMTS13-p.D187H mutation impairs ADAMTS13 activity and secretion and contributes to thrombotic thrombocytopenic purpura in miceE De Cock
Laboratory for Thrombosis Research, KU Leuven Kulak, Kortrijk, Belgium
J Thromb Haemost 13:283-92. 2015Congenital 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 ..
- Expression of a structurally constrained von Willebrand factor variant triggers acute thrombotic thrombocytopenic purpura in miceYoko 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 ..
- Increased expressions of ADAMTS-13 and apoptosis contribute to neuropathology during Toxoplasma gondii encephalitis in miceGungor 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. ..
- Carboxyl terminus of ADAMTS13 directly inhibits platelet aggregation and ultra large von Willebrand factor string formation under flow in a free-thiol-dependent mannerJialing 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. 2014b>ADAMTS13 (A Disintegrin And Metalloprotease with Thrombospondin type 1 repeats, 13) cleaves von Willebrand factor (VWF), thereby inhibiting thrombus formation...
- ADAMTS13 modulates atherosclerotic plaque progression in mice via a VWF-dependent mechanismC Gandhi
Department of Internal Medicine, University of Iowa, Iowa City, IA, USA
J Thromb Haemost 12:255-60. 2014b>ADAMTS13 reduces the adhesiveness of hyperactive ultra-large von Willebrand factor (ULVWF) multimers by cleaving them into smaller, less active multimers...
- Congenital thrombotic thrombocytopenic purpura with novel mutations in three unrelated Turkish childrenAyse 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. 2014Congenital 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...
- The CXCR4/CXCR7/SDF-1 pathway contributes to the pathogenesis of Shiga toxin-associated hemolytic uremic syndrome in humans and miceTania 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...
- von Willebrand factor fibers promote cancer-associated platelet aggregation in malignant melanoma of mice and humansAlexander 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 ..
- Complement activation associated with ADAMTS13 deficiency in human and murine thrombotic microangiopathyRamesh Tati
Department of Pediatrics, Clinical Sciences Lund, Lund University, 22185 Lund, Sweden
J Immunol 191:2184-93. 2013This 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...
- AAV-mediated expression of an ADAMTS13 variant prevents shigatoxin-induced thrombotic thrombocytopenic purpuraSheng Yu Jin
Department of Pathology and Laboratory Medicine, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
Blood 121:3825-9, S1-3. 2013Severe deficiency of plasma ADAMTS13 activity causes thrombotic thrombocytopenic purpura (TTP), a life-threatening syndrome for which plasma is the only effective therapy currently available...
- 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. 2012The protease ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeat) cleaves multimers of von Willebrand factor, thus regulating platelet aggregation...
- ADAMTS13 deficiency exacerbates VWF-dependent acute myocardial ischemia/reperfusion injury in miceChintan Gandhi
Department of Internal Medicine, University of Iowa, Iowa City, IA 52242, USA
Blood 120:5224-30. 2012Epidemiologic studies suggest that elevated VWF levels and reduced ADAMTS13 activity in the plasma are risk factors for myocardial infarction...
- Protective anti-inflammatory effect of ADAMTS13 on myocardial ischemia/reperfusion injury in miceSimon 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 ..
- Identification of epitopes on ADAMTS13 recognized by a panel of monoclonal antibodies with functional or non-functional effects on catalytic activityAtsuko Igari
Laboratory Medicine, Keio University School of Medicine, Tokyo, Japan
Thromb Res 130:e79-83. 2012von Willebrand factor (VWF) cleavage by ADAMTS13 is mediated by multi-step interactions between their multi-domain structures...
- ADAMTS13 reduces VWF-mediated acute inflammation following focal cerebral ischemia in miceM M Khan
Department of Internal Medicine, University of Iowa, Iowa City, IA 52242, USA
J Thromb Haemost 10:1665-71. 2012b>ADAMTS13 cleaves hyperactive ultra-large von Willebrand factor (ULVWF) multimers into smaller and less active forms...
- Genetic ablation of Adamts13 gene dramatically accelerates the formation of early atherosclerosis in a murine modelSheng 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. 2012b>ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats-13) cleaves von Willebrand factor, thereby modulating thrombosis and inflammation...
- A new mouse model mimicking thrombotic thrombocytopenic purpura: correction of symptoms by recombinant human ADAMTS13Alexandra Schiviz
Department of Preclinical Pharmacology and Toxicology, Baxter Innovations GmbH, Industriestrasse 67, Vienna, Austria
Blood 119:6128-35. 2012Deficiency 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 ..
- ADAMTS13 gene deletion enhances plasma high-mobility group box1 elevation and neuroinflammation in brain ischemia-reperfusion injuryMasayuki 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...
- ADAMTS13 reduces vascular inflammation and the development of early atherosclerosis in miceChintan Gandhi
Department of Internal Medicine, University of Iowa, Iowa City, USA
Blood 119:2385-91. 2012b>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...
- The distal carboxyl-terminal domains of ADAMTS13 are required for regulation of in vivo thrombus formationFumiaki Banno
Research Institute, National Cardiovascular Center, Suita, Japan
Blood 113:5323-9. 2009b>ADAMTS13 is a multidomain protease that limits platelet thrombogenesis through the cleavage of von Willebrand factor (VWF)...
- Unbalanced expression of ADAMTS13 and von Willebrand factor in mouse endotoxinemiaJun 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. 2008Secondary ADAMTS13 deficiency may occur in septic patients. The expression of ADAMTS13 in mouse endotoxinemia was studied.
- Degradation of circulating von Willebrand factor and its regulator ADAMTS13 implicates secreted Bacillus anthracis metalloproteases in anthrax consumptive coagulopathyMyung 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 ..
- ADAMTS13: a new link between thrombosis and inflammationAnil 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...
- Identification of strain-specific variants of mouse Adamts13 gene encoding von Willebrand factor-cleaving proteaseFumiaki Banno
National Cardiovascular Center Research Institute, Osaka 565 8565, Japan
J Biol Chem 279:30896-903. 2004Human 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...
- ADAMTS-like 2 (ADAMTSL2) is a secreted glycoprotein that is widely expressed during mouse embryogenesis and is regulated during skeletal myogenesisBon 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...
- An IAP retrotransposon in the mouse ADAMTS13 gene creates ADAMTS13 variant proteins that are less effective in cleaving von Willebrand factor multimersWenhua Zhou
Division of Hematology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY 10467, USA
Blood 110:886-93. 2007Severe deficiency of ADAMTS13, a von Willebrand factor (VWF)-cleaving metalloprotease, causes thrombotic thrombocytopenic purpura...
- ADAMTS13 assays and ADAMTS13-deficient miceToshiyuki 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 ..
- Structural and functional correlation of ADAMTS13Jing Fei Dong
Thrombosis Research Section, Baylor College of Medicine, Houston, TX 77030, USA
Curr Opin Hematol 14:270-6. 2007b>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...
- Cloning, expression and functional characterization of the full-length murine ADAMTS13K 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...
- The distal carboxyterminal domains of murine ADAMTS13 influence proteolysis of platelet-decorated VWF strings in vivoB De Maeyer
Laboratory for Thrombosis Research, K U Leuven Campus Kortrijk, Kortrijk, Belgium
J Thromb Haemost 8:2305-12. 2010The multidomain metalloprotease ADAMTS13 regulates the size of von Willebrand factor (VWF) multimers upon their release from endothelial cells...
- Correction of murine ADAMTS13 deficiency by hematopoietic progenitor cell-mediated gene therapyPablo 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. 2009b>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...
- von Willebrand factor-cleaving protease ADAMTS13 reduces ischemic brain injury in experimental strokeBing 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-..
- ADAMTS13 gene deletion aggravates ischemic brain damage: a possible neuroprotective role of ADAMTS13 by ameliorating postischemic hypoperfusionMasayuki 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 ..
- The function of ADAMTS13 in thrombogenesis in vivo: insights from mutant miceFumiaki Banno
National Cardiovascular Center Research Institute, 5 7 1 Fujishirodai, Suita, Osaka, 565 8565, Japan
Int J Hematol 91:30-5. 2010Recently, two independent groups have established ADAMTS13-deficient mice using gene-targeting techniques...
- Mutation and ADAMTS13-dependent modulation of disease severity in a mouse model for von Willebrand disease type 2BJulie 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...
- Essential domains of a disintegrin and metalloprotease with thrombospondin type 1 repeats-13 metalloprotease required for modulation of arterial thrombosisJuan Xiao
Department of Pathology and Laboratory Medicine, Children s Hospital of Philadelphia, PA 19104, USA
Arterioscler Thromb Vasc Biol 31:2261-9. 2011A disintegrin and metalloprotease with thrombospondin type 1 repeats-13 (ADAMTS13) inhibits platelet aggregation and arterial thrombosis by cleavage of von Willebrand factor...
- Phenotypic expression of ADAMTS13 in glomerular endothelial cellsRamesh Tati
Department of Pediatrics, Clinical Sciences Lund, Lund University, Lund, Sweden
PLoS ONE 6:e21587. 2011b>ADAMTS13 is the physiological von Willebrand factor (VWF)-cleaving protease...
- In vivo imaging analysis of the interaction between unusually large von Willebrand factor multimers and platelets on the surface of vascular wallMiroslaw 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-/-)...
- N-acetylcysteine reduces the size and activity of von Willebrand factor in human plasma and miceJunmei 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 ..