J Evan Sadler

Summary

Affiliation: Washington University School of Medicine
Country: USA

Publications

  1. ncbi request reprint Structural biology. A ménage à trois in two configurations
    J Evan Sadler
    Howard Hughes Medical Institute, Department of Medicine, Washington University, St Louis, MO 63110, USA
    Science 301:177-9. 2003
  2. ncbi request reprint Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor
    J E Sadler
    Howard Hughes Medical Institute, Washington University, St Louis, MO 63110, USA
    J Thromb Haemost 4:2103-14. 2006
  3. ncbi request reprint Biochemistry and genetics of von Willebrand factor
    J E Sadler
    Howard Hughes Medical Institute, Department of Medicine, Washington University School of Medicine, Saint Louis, Missouri 63110, USA
    Annu Rev Biochem 67:395-424. 1998
  4. ncbi request reprint New concepts in von Willebrand disease
    J Evan Sadler
    Howard Hughes Medical Institute, Department of Medicine, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Annu Rev Med 56:173-91. 2005
  5. ncbi request reprint Slippery criteria for von Willebrand disease type 1
    J E Sadler
    Howard Hughes Medical Institute, Department of Medicine, Washington University School of Medicine, St Louis, MO 63110, USA
    J Thromb Haemost 2:1720-3. 2004
  6. ncbi request reprint Biomedicine. Contact--how platelets touch von Willebrand factor
    J Evan Sadler
    Department of Medicine, Washington University School of Medicine, St Louis, MO 63110, USA
    Science 297:1128-9. 2002
  7. pmc A new name in thrombosis, ADAMTS13
    J Evan Sadler
    Department of Medicine and Howard Hughes Medical Institute, Washington University School of Medicine, St Louis, MO 63110, USA
    Proc Natl Acad Sci U S A 99:11552-4. 2002
  8. pmc Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura
    J Evan Sadler
    Department of Medicine, Washington University School of Medicine, St Louis, MO 63110, USA
    Blood 112:11-8. 2008
  9. ncbi request reprint Von Willebrand disease type 1: a diagnosis in search of a disease
    J Evan Sadler
    Department of Medicine, Howard Hughes Medical Institute, Washington University School of Medicine, St Louis, MO 63110, USA
    Blood 101:2089-93. 2003
  10. ncbi request reprint von Willebrand factor: two sides of a coin
    J E Sadler
    Howard Hughes Medical Institute, Department of Medicine, Washington University School of Medicine, St Louis, MO 63110, USA
    J Thromb Haemost 3:1702-9. 2005

Detail Information

Publications60

  1. ncbi request reprint Structural biology. A ménage à trois in two configurations
    J Evan Sadler
    Howard Hughes Medical Institute, Department of Medicine, Washington University, St Louis, MO 63110, USA
    Science 301:177-9. 2003
  2. ncbi request reprint Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor
    J E Sadler
    Howard Hughes Medical Institute, Washington University, St Louis, MO 63110, USA
    J Thromb Haemost 4:2103-14. 2006
    ..The clinical significance of this heterogeneity is under investigation, which may support further subdivision of VWD type 1 or type 2A in the future...
  3. ncbi request reprint Biochemistry and genetics of von Willebrand factor
    J E Sadler
    Howard Hughes Medical Institute, Department of Medicine, Washington University School of Medicine, Saint Louis, Missouri 63110, USA
    Annu Rev Biochem 67:395-424. 1998
    ....
  4. ncbi request reprint New concepts in von Willebrand disease
    J Evan Sadler
    Howard Hughes Medical Institute, Department of Medicine, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Annu Rev Med 56:173-91. 2005
    ..Emerging data suggest that VWF level is a useful biomarker for the risk of either bleeding or thrombosis and could be incorporated into a comprehensive approach to treat or prevent these adverse events...
  5. ncbi request reprint Slippery criteria for von Willebrand disease type 1
    J E Sadler
    Howard Hughes Medical Institute, Department of Medicine, Washington University School of Medicine, St Louis, MO 63110, USA
    J Thromb Haemost 2:1720-3. 2004
  6. ncbi request reprint Biomedicine. Contact--how platelets touch von Willebrand factor
    J Evan Sadler
    Department of Medicine, Washington University School of Medicine, St Louis, MO 63110, USA
    Science 297:1128-9. 2002
  7. pmc A new name in thrombosis, ADAMTS13
    J Evan Sadler
    Department of Medicine and Howard Hughes Medical Institute, Washington University School of Medicine, St Louis, MO 63110, USA
    Proc Natl Acad Sci U S A 99:11552-4. 2002
  8. pmc Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura
    J Evan Sadler
    Department of Medicine, Washington University School of Medicine, St Louis, MO 63110, USA
    Blood 112:11-8. 2008
    ..A major unanswered question is whether plasma exchange is effective for the subset of patients with idiopathic TTP who do not have severe ADAMTS13 deficiency...
  9. ncbi request reprint Von Willebrand disease type 1: a diagnosis in search of a disease
    J Evan Sadler
    Department of Medicine, Howard Hughes Medical Institute, Washington University School of Medicine, St Louis, MO 63110, USA
    Blood 101:2089-93. 2003
    ..Such a risk management strategy could be generalized to include other hemorrhagic and thrombotic risk factors...
  10. ncbi request reprint von Willebrand factor: two sides of a coin
    J E Sadler
    Howard Hughes Medical Institute, Department of Medicine, Washington University School of Medicine, St Louis, MO 63110, USA
    J Thromb Haemost 3:1702-9. 2005
    ..More research is needed to understand how VWF function is regulated, and to enable physicians to use this knowledge for the benefit of their patients...
  11. ncbi request reprint Zinc and calcium ions cooperatively modulate ADAMTS13 activity
    Patricia J Anderson
    Howard Hughes Medical Institute, Department of Medicine, Washington University School of Medicine, St Louis, MO 63110, USA
    J Biol Chem 281:850-7. 2006
    ..The striking preference of ADAMTS13 for VWF suggests that substrate recognition depends on structural features or exosites on multimeric VWF that are missing from FRETS-VWF73...
  12. pmc Exosite interactions contribute to tension-induced cleavage of von Willebrand factor by the antithrombotic ADAMTS13 metalloprotease
    Weiqiang Gao
    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 103:19099-104. 2006
    ..The specificity of this unique mechanism depends on tension-induced unfolding of the VWF A2 domain, which exposes the scissile bond and exosite for interaction with complementary sites on ADAMTS13...
  13. ncbi request reprint 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
    ..The first thrombospondin repeat also affects binding, and the C-terminal thrombospondin type 1 and CUB domains of ADAMTS13 may modulate this interaction...
  14. ncbi request reprint Cleavage of von Willebrand factor requires the spacer domain of the metalloprotease ADAMTS13
    Xinglong Zheng
    Department of Pathology and Immunology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 278:30136-41. 2003
    ..Therefore, the spacer region is necessary for normal ADAMTS13 activity toward von Willebrand factor, and the more C-terminal TSP1 and CUB domains are dispensable in vitro...
  15. pmc Extensive contacts between ADAMTS13 exosites and von Willebrand factor domain A2 contribute to substrate specificity
    Weiqiang Gao
    Department of Medicine, Howard Hughes Medical Institute, Washington University School of Medicine, St Louis, MO 63110, USA
    Blood 112:1713-9. 2008
    ..Thus, specific recognition of VWF depends on cooperative, modular contacts between several ADAMTS13 domains and discrete segments of VWF domain A2...
  16. pmc Assembly of Weibel-Palade body-like tubules from N-terminal domains of von Willebrand factor
    Ren Huai Huang
    Howard Hughes Medical Institute, Washington University School of Medicine, St Louis, MO 63110, USA
    Proc Natl Acad Sci U S A 105:482-7. 2008
    ..2 units per turn. The symmetry and location of interdomain contacts suggest that decreasing pH along the secretory pathway coordinates the disulfide-linked assembly of VWF multimers with their tubular packaging...
  17. pmc Two Cys residues essential for von Willebrand factor multimer assembly in the Golgi
    Angie R Purvis
    Howard Hughes Medical Institute, Washington University School of Medicine, St Louis, MO 63110, USA
    Proc Natl Acad Sci U S A 104:15647-52. 2007
    ..This arrangement of intersubunit disulfide bonds implies that the dimeric N-terminal D'D3 domains of VWF subunits align in a parallel orientation within VWF multimers...
  18. 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
    ..Thus, binding of vWF to its major physiological ligands may promote the feedback inhibition of platelet adhesion by stimulating the cleavage of domain A2 by ADAMTS13 independent of fluid shear stress...
  19. 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
    ..This reaction is likely to account for a majority of VWF proteolysis after secretion and to determine the steady-state size distribution of circulating VWF multimers in vivo...
  20. pmc Integrin alpha(v)beta(3) on human endothelial cells binds von Willebrand factor strings under fluid shear stress
    Jing Huang
    Department of Medicine, Biochemistry and Molecular Biophysics, and Howard Hughes Medical Institute, Washington University School of Medicine, St Louis, MO 63110, USA
    Blood 113:1589-97. 2009
    ..These results indicate that VWF strings bind specifically to integrin alpha(v)beta(3) on human endothelial cells...
  21. pmc Rearranging exosites in noncatalytic domains can redirect the substrate specificity of ADAMTS proteases
    Weiqiang Gao
    Department of Medicine, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 287:26944-52. 2012
    ..Noncatalytic domains may perform similar functions in other ADAMTS family members...
  22. ncbi request reprint 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
    ..Therefore, the ADAMTS13 propeptide is not required for folding or secretion, and does not perform the common function of maintaining enzyme latency...
  23. pmc Phylogenetic and functional analysis of histidine residues essential for pH-dependent multimerization of von Willebrand factor
    Luke T Dang
    Department of Medicine, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 286:25763-9. 2011
    ..These results suggest that pH sensing by evolutionarily conserved His residues facilitates the assembly and packaging of VWF multimers upon arrival in the trans-Golgi...
  24. ncbi request reprint Identification of the regulatory elements of the human von Willebrand factor for binding to platelet GPIb. Importance of structural integrity of the regions flanked by the CYS1272-CYS1458 disulfide bond
    Takayuki Nakayama
    First Department of Internal Medicine, Nagoya University School of Medicine, Japan
    J Biol Chem 277:22063-72. 2002
    ..The crystallographic structure of the A1 domain suggests that GPIb binding is influenced by the molecular interface between the two regions and that the antibody binding to the interface inhibits binding...
  25. pmc The B subunits of Shiga-like toxins induce regulated VWF secretion in a phospholipase D1-dependent manner
    Jing Huang
    Department of Medicine, Washington University School of Medicine, St Louis, MO 63110, USA
    Blood 120:1143-9. 2012
    ..These data indicate that Stx1B and Stx2B induce acute VWF secretion in a PLD1-dependent manner but do so by differentially modulating PKCα, RhoA, and ADP-ribosylation factor 6...
  26. ncbi request reprint Remission of chronic thrombotic thrombocytopenic purpura after treatment with cyclophosphamide and rituximab
    Xinglong Zheng
    Washington University School of Medicine, 660 South Euclid Avenue, Box 8022, St Louis, Missouri 63110, USA
    Ann Intern Med 138:105-8. 2003
    ..Thrombotic thrombocytopenic purpura (TTP) in adults is usually caused by autoantibody inhibitors of ADAMTS13. Treatment with plasma exchange is often effective but does not address the underlying autoimmune process...
  27. pmc Shiga toxin (Stx)1B and Stx2B induce von Willebrand factor secretion from human umbilical vein endothelial cells through different signaling pathways
    Fang Liu
    Department of Medicine, Washington University School of Medicine, St Louis, MO, USA
    Blood 118:3392-8. 2011
    ..The activation of distinct signaling pathways may be relevant to understanding why E coli that express Stx2 are more likely to cause D(+)HUS than are E coli expressing only Stx1...
  28. pmc Cleavage of ultralarge multimers of von Willebrand factor by C-terminal-truncated mutants of ADAMTS-13 under flow
    Zhenyin Tao
    Thrombosis Research Section, Department of Medicine, BCM286, N1319, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
    Blood 106:141-3. 2005
    ..The wild type and the mutant without the spacer were more active in the presence of plasma, raising the possibility of ADAMTS-13 cofactors in plasma...
  29. ncbi request reprint Factor XI/ADAMTS13 complexes are quantitatively insignificant in human plasma
    Patricia J Anderson
    Department of Medicine, Washington University School of Medicine, 660 S Euclid, Box 8066, St Louis, MO 63110, USA
    Haematologica 92:1419-22. 2007
    ..These results suggest that ADAMTS13/FXI complexes are insignificant in plasma and unlikely to affect the function of either protein during normal hemostasis or in TTP...
  30. doi request reprint A structural explanation for the antithrombotic activity of ARC1172, a DNA aptamer that binds von Willebrand factor domain A1
    Ren Huai Huang
    Department of Medicine, Washington University School of Medicine, St Louis, MO 63110, USA
    Structure 17:1476-84. 2009
    ..The ARC1172 binding site on the A1 domain overlaps with that of botrocetin and clashes with glycoprotein Ibalpha binding at an adjacent site, which accounts for the antithrombotic activity of ARC1172 and related aptamers...
  31. 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
    ..These results demonstrate the existence of a novel Stx B-induced lipid raft-dependent signaling pathway in endothelial cells that may be responsible for some of the biological effects attributed previously to the cytotoxic Stx A subunit...
  32. pmc Unfolding the A2 domain of von Willebrand factor with the optical trap
    Junyi Ying
    Department of Biomedical Engineering, Washington University, St Louis, Missouri, USA
    Biophys J 98:1685-93. 2010
    ....
  33. ncbi request reprint ADAMTS13 and TTP
    Xinglong Zheng
    Department of Pathology and Immunology, Washington University School of Medicine, 660 South Euclid Avenue, St Louis, Missouri 63110, USA
    Curr Opin Hematol 9:389-94. 2002
    ..Further studies of ADAMTS13 seem likely to change our approach to the diagnosis and treatment of TTP and other thrombotic microangiopathies...
  34. ncbi request reprint A recombinant murine meizothrombin precursor, prothrombin R157A/R268A, inhibits thrombosis in a model of acute carotid artery injury
    Kyuhwan Shim
    Howard Hughes Medical Institute, Washington University School of Medicine, 660 S Euclid Ave, Box 8022, St Louis, MO 63110, USA
    Blood 104:415-9. 2004
    ..In this model, prothrombin R157A/R268A has anticoagulant activity that reflects its decreased fibrinogen-clotting activity and preserved protein C-activating activity and is consistent with dominant inhibition of fibrinogen clotting...
  35. ncbi request reprint Thrombomodulin structure and function
    J E Sadler
    Howard Hughes Medical Institute, Department of Medicine, Washington University School of Medicine, St Louis, MO 63110, USA
    Thromb Haemost 78:392-5. 1997
    ..These structures are small steps toward an understanding of how thrombomodulin regulates thrombin...
  36. doi request reprint von Willebrand factor assembly and secretion
    J E Sadler
    Department of Medicine, Washington University School of Medicine, St Louis, MO 63110, USA
    J Thromb Haemost 7:24-7. 2009
    ..Recent studies have identified some of the biochemical and structural properties that underlie these self-organizing behaviors...
  37. pmc Molecular basis of von Willebrand disease type IIB. Candidate mutations cluster in one disulfide loop between proposed platelet glycoprotein Ib binding sequences
    A M Randi
    Howard Hughes Medical Institute, Department of Medicine, Washington University School of Medicine, St Louis, Missouri 63110
    J Clin Invest 87:1220-6. 1991
    ....
  38. ncbi request reprint Type 1 von Willebrand disease mutation Cys1149Arg causes intracellular retention and degradation of heterodimers: a possible general mechanism for dominant mutations of oligomeric proteins
    I Bodo
    Howard Hughes Medical Institute and the Department of Medicine, Washington University School of Medicine, St Louis, MO 63110, USA
    Blood 98:2973-9. 2001
    ..A similar dominant negative mechanism could cause quantitative deficiencies of other multisubunit proteins...
  39. ncbi request reprint Crystal structure of von Willebrand factor A1 domain complexed with snake venom, bitiscetin: insight into glycoprotein Ibalpha binding mechanism induced by snake venom proteins
    Nobuo Maita
    Department of Biochemistry, National Institute of Agrobiological Sciences, Tsukuba, Ibaraki 305 8602, Japan
    J Biol Chem 278:37777-81. 2003
    ..These results suggest that snake venom proteins induce VWF A1-GPIbalpha binding by interacting with both proteins, and not by causing conformational changes in VWF A1...
  40. ncbi request reprint Identification of amino acid residues essential for heparin binding by the A1 domain of human von Willebrand factor
    Tatsuya Adachi
    Department of Hematology, Nagoya University Graduate School of Medicine, Nagoya, Japan
    Biochem Biophys Res Commun 339:1178-83. 2006
    ....
  41. ncbi request reprint Aortic stenosis, von Willebrand factor, and bleeding
    J Evan Sadler
    Howard Hughes Medical Institute, Department of Medicine, Washington University School of Medicine, St Louis, USA
    N Engl J Med 349:323-5. 2003
  42. pmc von Willebrand disease type B: a missense mutation selectively abolishes ristocetin-induced von Willebrand factor binding to platelet glycoprotein Ib
    I Rabinowitz
    Howard Hughes Medical Institute, Washington University School of Medicine, St Louis, MO
    Proc Natl Acad Sci U S A 89:9846-9. 1992
    ....
  43. pmc Expression of von Willebrand factor "Normandy": an autosomal mutation that mimics hemophilia A
    E A Tuley
    Howard Hughes Medical Institute, Department of Medicine, Jewish Hospital of St Louis, Washington, University School of Medicine, MO 63110
    Proc Natl Acad Sci U S A 88:6377-81. 1991
    ....
  44. ncbi request reprint Localization of disulfide bonds in the cystine knot domain of human von Willebrand factor
    A Katsumi
    Howard Hughes Medical Institute, Department of Medicine and Department of Biochemistry and Molecular Biophysics, Washington University School of Medicine, St Louis, MO 63110, USA
    J Biol Chem 275:25585-94. 2000
    ....
  45. ncbi request reprint Characterization of the residues involved in the human alpha-thrombin-haemadin complex: an exosite II-binding inhibitor
    John L Richardson
    Max Planck Institut fur Biochemie, D 82152 Martinsried, Germany
    Biochemistry 41:2535-42. 2002
    ....
  46. ncbi request reprint A covalent oxidoreductase intermediate in propeptide-dependent von Willebrand factor multimerization
    Angie R Purvis
    Howard Hughes Medical Institute, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 279:49982-8. 2004
    ..These results support a model in which the propeptide acts as an oxidoreductase to promote von Willebrand factor multimerization in the Golgi apparatus...
  47. doi request reprint Multi-step binding of ADAMTS-13 to von Willebrand factor
    H B Feys
    Department of Biochemistry and Molecular Biophysics, Department of Medicine, Washington University, School of Medicine, St Louis, MO, USA
    J Thromb Haemost 7:2088-95. 2009
    ..Substrate recognition is mediated through several non-catalytic domains in ADAMTS-13 distant from the active site...
  48. ncbi request reprint Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura
    X Long Zheng
    Department of Pathology and Laboratory Medicine, The Children s Hospital of Philadelphia, The University of Pennsylvania School of Medicine, 34th Street and Civic Center Boulevard, ARC 316, Philadelphia, PA 19104, USA
    Blood 103:4043-9. 2004
    ..02). We conclude that assays of ADAMTS13 activity and inhibitors in addition to the clinical categories (idiopathic TTP and nonidiopathic TTP) are predictive of outcome and may be useful to tailor patient treatment...
  49. 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)...
  50. ncbi request reprint Mucin-like domain of enteropeptidase directs apical targeting in Madin-Darby canine kidney cells
    Xinglong Zheng
    Department of Pathology and Immunology, Washington University School of Medicine, 660 S Euclid Avenue, St Louis, MO 63110, USA
    J Biol Chem 277:6858-63. 2002
    ..Density gradient centrifugation indicated that, unlike several other apically targeted membrane and soluble proteins, apical sorting of mucin-GFP chimeric proteins does not appear to utilize lipid rafts...
  51. ncbi request reprint Human von Willebrand factor gene and pseudogene: structural analysis and differentiation by polymerase chain reaction
    D J Mancuso
    Howard Hughes Medical Institute Laboratories, Department of Medicine, Washington University School of Medicine, St Louis, Missouri 63110
    Biochemistry 30:253-69. 1991
    ..This method is useful for the analysis of gene defects in patients with von Willebrand disease, without interference from homologous sequences in the pseudogene...
  52. ncbi request reprint Provisional criteria for the diagnosis of VWD type 1
    J E Sadler
    Howard Hughes Medical Institute, Chevy Chase, MD, USA
    J Thromb Haemost 3:775-7. 2005
  53. ncbi request reprint Structure of the gene for human von Willebrand factor
    D J Mancuso
    Department of Medicine, Howard Hughes Medical Institute Laboratories, St Louis, Missouri
    J Biol Chem 264:19514-27. 1989
    ..Regions of the gene that encode homologous domains have similar structures, supporting a model for their origin by gene segment duplication...
  54. ncbi request reprint The structure and function of mouse thrombomodulin. Phorbol myristate acetate stimulates degradation and synthesis of thrombomodulin without affecting mRNA levels in hemangioma cells
    W A Dittman
    Department of Internal Medicine, Washington University School of Medicine, St Louis, Missouri 63110
    J Biol Chem 263:15815-22. 1988
    ..We conclude that thrombomodulin is phosphorylated in response to treatment of hemangioma cells with PMA which leads to decreased protein C cofactor activity and both increased degradation and synthesis of thrombomodulin...
  55. ncbi request reprint 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
    ..Alternative splicing may have functional significance, producing proteins with distinct abilities to interact with cofactors, connective tissue, platelets, and von Willebrand factor...
  56. pmc Mutations in the proenteropeptidase gene are the molecular cause of congenital enteropeptidase deficiency
    Andreas Holzinger
    Department of Pediatrics, Division of Clinical Chemistry and Metabolism, Dr V Hauner Children s Hospital, Ludwig Maximilian University, Munich, Germany
    Am J Hum Genet 70:20-5. 2002
    ..These data provide first evidence that proenteropeptidase-gene mutations are the primary cause of congenital enteropeptidase deficiency...
  57. pmc Pathogenesis of thrombotic microangiopathies
    X Long Zheng
    Department of Pathology and Laboratory Medicine, The Children s Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Annu Rev Pathol 3:249-77. 2008
    ..The incomplete penetrance of mutations in either ADAMTS13 or complement regulatory genes suggests that precipitating events or triggers may be required to cause thrombotic microangiopathy in many patients...
  58. pmc Enterokinase, the initiator of intestinal digestion, is a mosaic protease composed of a distinctive assortment of domains
    Y Kitamoto
    Department of Medicine, Jewish Hospital of St Louis, Washington University School of Medicine, MO 63110
    Proc Natl Acad Sci U S A 91:7588-92. 1994
    ..The specificity of enterokinase for the DDDDK-I sequence of trypsinogen may be explained by complementary basic-amino acid residues clustered in potential S2-S5 subsites...
  59. pmc Incomplete embryonic lethality and fatal neonatal hemorrhage caused by prothrombin deficiency in mice
    J Xue
    Department of Medicine, Department of Biochemistry and Molecular Biophysics, Barnes Jewish Hospital and Howard Hughes Medical Institute, Washington University School of Medicine, St Louis, MO 63110, USA
    Proc Natl Acad Sci U S A 95:7603-7. 1998
    ..Therefore, the role of thrombin in development may be independent of its effects on blood coagulation and instead may involve signal transduction on cells other than platelets...
  60. ncbi request reprint Platelet glycoprotein Ib alpha binds to thrombin anion-binding exosite II inducing allosteric changes in the activity of thrombin
    C Q Li
    Department of Biochemistry and Molecular Biophysics, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 276:6161-8. 2001
    ....