Evan Sadler.jpg

J. Evan Sadler, MD, PhD

Department of Medicine
Hematology Division
Department of Biochemistry & Molecular Biophysics


  • 314-362-9029 (tel)
  • 314-454-3012 (fax)
  • Room 10027 Clinical Sciences Research Building (office)
  • Room 10034 Clinical Sciences Research Building (lab)
  • Hematology Division
    Campus Box 8125
    Washington University Medical School
    660 South Euclid Avenue
    St. Louis, MO 63110

Peer-reviewed Manuscripts

  • Pathophysiology of Thrombotic Thrombocytopenic Purpura
    Sadler JE
    Blood 2017 Sep 7;130(10):1181-1188
  • Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies
    Scully M, Cataland S, Coppo P, de la Rubia J, Friedman KD, Kremer Hovinga J, Lammle B, Matsumoto M, Pavenski K, Sadler E, Sarode R, Wu H
    J Thromb Haemost 2017 Feb;15(2):312-322
  • Warfarin traps human vitamin K epoxide reductase in an intermediate state during electron transfer
    Shen G, Cui W, Zhang H, Zhou F, Huang W, Liu Q, Yang Y, Li S, Bowman GR, Sadler JE, Gross ML, Li W
    Nat Struct Mol Biol 2017 Jan;24(1):69-76
  • Identification and characterization of the elusive mutation causing the historical von Willebrand disease type IIC Miami
    Obser T, Ledford-Kraemer M, Oyen F, Brehm MA, Denis CV, Marschalek R, Montgomery RR, Sadler JE, Schneppenheim S, Budde U, Schneppenheim R
    J Thromb Haemost 2016 Sep;14(9):1725-1735
  • The functions of the A1A2A3 domains in von Willebrand factor include multimerin 1 binding
    Parker DN, Tasneem S, Farndale RW, Bihan D, Sadler JE, Sebastian S, de Groot PG, Hayward CP
    Thromb Haemost 2016 Jul 4;116(1):87-95
  • Linker regions and flexibility around the metalloprotease domain account for conformational activation of ADAMTS-13
    Deforche L, Roose E, Vandenbulcke A, Vandeputte N, Feys HB, Springer TA, Mi LZ, Muia J, Sadler JE, Soejima K, Rottensteiner H, Deckmyn H, De Meyer SF, Vanhoorelbeke K
    J Thromb Haemost 2015 Nov;13(11):2063-2075
  • Allosteric activation of ADAMTS13 by von Willebrand factor
    Muia J, Zhu J, Gupta G, Haberichter SL, Friedman KD, Feys HB, Deforche L, Vanhoorelbeke K, Westfield LA, Roth R, Tolia NH, Heuser JE, Sadler JE
    Proc Natl Acad Sci U S A 2014 Dec 30;111(52):18584-9
  • Single particle tracking of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type-1 repeats) molecules on endothelial von Willebrand factor strings
    De Ceunynck K, Rocha S, De Meyer SF, Sadler JE, Uji-i H, Deckmyn H, Hofkens J, Vanhoorelbeke K
    J Biol Chem 2014 Mar 28;289(13):8903-15
  • Thrombin-targeted liposomes establish a sustained localized anticlotting barrier against acute thrombosis
    Palekar RU, Myerson JW, Schlesinger PH, Sadler JE, Pan H, Wickline SA
    Mol Pharm 2013 Nov 4;10(11):4168-75
  • An optimized fluorogenic ADAMTS13 assay with increased sensitivity for the investigation of patients with thrombotic thrombocytopenic purpura
    Muia J, Gao W, Haberichter SL, Dolatshahi L, Zhu J, Westfield LA, Covill SC, Friedman KD, Sadler JE
    J Thromb Haemost 2013 Aug;11(8):1511-8
  • Rearranging exosites in noncatalytic domains can redirect the substrate specificity of ADAMTS proteases
    Gao W, Zhu J, Westfield LA, Tuley EA, Anderson PJ, Sadler JE
    J Biol Chem 2012 Aug 3;287(32):26944-52
  • The B subunits of Shiga-like toxins induce regulated VWF secretion in a phospholipase D1-dependent manner
    Huang J, Haberichter SL, Sadler JE
    Blood 2012 Aug 2;120(5):1143-9
  • Shiga toxin (Stx)1B and Stx2B induce von Willebrand factor secretion from human umbilical vein endothelial cells through different signaling pathways
    Liu F, Huang J, Sadler JE
    Blood 2011 Sep 22;118(12):3392-8
  • Phylogenetic and functional analysis of histidine residues essential for pH-dependent multimerization of von Willebrand factor
    Dang LT, Purvis AR, Huang RH, Westfield LA, Sadler JE
    J Biol Chem 2011 Jul 22;286(29):25763-9
  • Shiga toxin B subunits induce VWF secretion by human endothelial cells and thrombotic microangiopathy in ADAMTS13-deficient mice
    Huang J, Motto DG, Bundle DR, Sadler JE
    Blood 2010 Nov 4;116(18):3653-9
  • Unfolding the A2 domain of von Willebrand factor with the optical trap
    Ying J, Ling Y, Westfield LA, Sadler JE, Shao JY
    Biophys J 2010 Apr 21;98(8):1685-93
  • Accelerated clearance alone explains ultra-large multimers in von Willebrand disease Vicenza
    Gézsi A, Budde U, Deák I, Nagy E, Mohl A, Schlammadinger A, Boda Z, Masszi T, Sadler JE, Bodó I
    J Thromb Haemost 2010 Jun;8(6):1273-80
  • A structural explanation for the antithrombotic activity of ARC1172, a DNA aptamer that binds von Willebrand factor domain A1
    Huang RH, Fremont DH, Diener JL, Schaub RG, Sadler JE
    Structure 2009 Nov 11;17(11):1476-84
  • Multi-step binding of ADAMTS-13 to von Willebrand factor
    Feys HB, Anderson PJ, Vanhoorelbeke K, Majerus EM, Sadler JE
    J Thromb Haemost 2009 Dec;7(12):2088-95
  • Integrin alpha(v)beta(3) on human endothelial cells binds von Willebrand factor strings under fluid shear stress
    Huang J, Roth R, Heuser JE, Sadler JE
    Blood 2009 Feb 12;113(7):1589-97
  • Extensive contacts between ADAMTS13 exosites and von Willebrand factor domain A2 contribute to substrate specificity
    Gao W, Anderson PJ, Sadler JE
    Blood 2008 Sep 1;112(5):1713-9
  • Identification of amino acid residues responsible for von Willebrand factor binding to sulfatide by charged-to-alanine-scanning mutagenesis
    Nakayama T, Matsushita T, Yamamoto K, Mutsuga N, Kojima T, Katsumi A, Nakao N, Sadler JE, Naoe T, Saito H
    Int J Hematol 2008 May;87(4):363-70
  • von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA)
    Nichols WL, Hultin MB, James AH, Manco-Johnson MJ, Montgomery RR, Ortel TL, Rick ME, Sadler JE, Weinstein M, Yawn BP
    Haemophilia 2008 Mar;14(2):171-232
  • Assembly of Weibel-Palade body-like tubules from N-terminal domains of von Willebrand factor
    Huang RH, Wang Y, Roth R, Yu X, Purvis AR, Heuser JE, Egelman EH, Sadler JE
    Proc Natl Acad Sci U S A 2008 Jan 15;105(2):482-7
  • Platelet-VWF complexes are preferred substrates of ADAMTS13 under fluid shear stress
    Shim K, Anderson PJ, Tuley EA, Wiswall E, Sadler JE
    Blood 2008 Jan 15;111(2):651-7
  • Two Cys residues essential for von Willebrand factor multimer assembly in the Golgi
    Purvis AR, Gross J, Dang LT, Huang RH, Kapadia M, Townsend RR, Sadler JE
    Proc Natl Acad Sci U S A 2007 Oct 2;104(40):15647-52
  • Two mechanistic pathways for thienopyridine-associated thrombotic thrombocytopenic purpura: a report from the SERF-TTP Research Group and the RADAR Project
    Bennett CL, Kim B, Zakarija A, Bandarenko N, Pandey DK, Buffie CG, McKoy JM, Tevar AD, Cursio JF, Yarnold PR, Kwaan HC, De Masi D, Sarode R, Raife TJ, Kiss JE, Raisch DW, Davidson C, Sadler JE, Ortel TL, Zheng XL, Kato S, Matsumoto M, Uemura M, Fujimura Y
    J Am Coll Cardiol 2007 Sep 18;50(12):1138-43
  • Factor XI/ADAMTS13 complexes are quantitatively insignificant in human plasma
    Anderson PJ, Gailani D, Feys HB, Gao W, Majerus EM, Vanhoorelbeke K, Sadler JE
    Haematologica 2007 Oct;92(10):1419-22
  • Exosite interactions contribute to tension-induced cleavage of von Willebrand factor by the antithrombotic ADAMTS13 metalloprotease
    Gao W, Anderson PJ, Majerus EM, Tuley EA, Sadler JE
    Proc Natl Acad Sci U S A 2006 Dec 12;103(50):19099-104
  • Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor
    Sadler JE, Budde U, Eikenboom JC, Favaloro EJ, Hill FG, Holmberg L, Ingerslev J, Lee CA, Lillicrap D, Mannucci PM, Mazurier C, Meyer D, Nichols WL, Nishino M, Peake IR, Rodeghiero F, Schneppenheim R, Ruggeri ZM, Srivastava A, Montgomery RR, Federici AB
    J Thromb Haemost 2006 Oct;4(10):2103-14
  • Identification of amino acid residues essential for heparin binding by the A1 domain of human von Willebrand factor
    Adachi T, Matsushita T, Dong Z, Katsumi A, Nakayama T, Kojima T, Saito H, Sadler JE, Naoe T
    Biochem Biophys Res Commun 2006 Jan 27;339(4):1178-83
  • Zinc and calcium ions cooperatively modulate ADAMTS13 activity
    Anderson PJ, Kokame K, Sadler JE
    J Biol Chem 2006 Jan 13;281(2):850-7
  • Binding of ADAMTS13 to von Willebrand factor
    Majerus EM, Anderson PJ, Sadler JE
    J Biol Chem 2005 Jun 10;280(23):21773-8
  • Cleavage of ultralarge multimers of von Willebrand factor by C-terminal-truncated mutants of ADAMTS-13 under flow
    Tao Z, Wang Y, Choi H, Bernardo A, Nishio K, Sadler JE, Lopez JA, Dong JF
    Blood 2005 Jul 1;106(1):141-3
  • Provisional criteria for the diagnosis of VWD type 1
    Sadler JE, Rodeghiero F
    J Thromb Haemost 2005 Apr;3(4):775-7
  • A covalent oxidoreductase intermediate in propeptide-dependent von Willebrand factor multimerization
    Purvis AR, Sadler JE
    J Biol Chem 2004 Nov 26;279(48):49982-8
  • Binding of platelet glycoprotein Ibalpha to von Willebrand factor domain A1 stimulates the cleavage of the adjacent domain A2 by ADAMTS13
    Nishio K, Anderson PJ, Zheng XL, Sadler JE
    Proc Natl Acad Sci U S A 2004 Jul 20;101(29):10578-83
  • A recombinant murine meizothrombin precursor, prothrombin R157A/R268A, inhibits thrombosis in a model of acute carotid artery injury
    Shim K, Zhu H, Westfield LA, Sadler JE
    Blood 2004 Jul 15;104(2):415-9
  • Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura
    Zheng XL, Kaufman RM, Goodnough LT, Sadler JE
    Blood 2004 Jun 1;103(11):4043-9
  • Cleavage of the ADAMTS13 propeptide is not required for protease activity
    Majerus EM, Zheng X, Tuley EA, Sadler JE
    J Biol Chem 2003 Nov 21;278(47):46643-8
  • Crystal structure of von Willebrand factor A1 domain complexed with snake venom, bitiscetIn: insight into glycoprotein Ibalpha binding mechanism induced by snake venom proteins
    Maita N, Nishio K, Nishimoto E, Matsui T, Shikamoto Y, Morita T, Sadler JE, Mizuno H
    J Biol Chem 2003 Sep 26;278(39):37777-81
  • Cleavage of von Willebrand factor requires the spacer domain of the metalloprotease ADAMTS13
    Zheng X, Nishio K, Majerus EM, Sadler JE
    J Biol Chem 2003 Aug 8;278(32):30136-41
  • Von Willebrand disease type 1: a diagnosis in search of a disease
    Sadler JE
    Blood 2003 Mar 15;101(6):2089-93
  • Remission of chronic thrombotic thrombocytopenic purpura after treatment with cyclophosphamide and rituximab
    Zheng X, Pallera AM, Goodnough LT, Sadler JE, Blinder MA
    Ann Intern Med 2003 Jan 21;138(2):105-8
  • 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
    Nakayama T, Matsushita T, Dong Z, Sadler JE, Jorieux S, Mazurier C, Meyer D, Kojima T, Saito H
    J Biol Chem 2002 Jun 14;277(24):22063-72
  • Characterization of the residues involved in the human alpha-thrombin-haemadin complex: an exosite II-binding inhibitor
    Richardson JL, Fuentes-Prior P, Sadler JE, Huber R, Bode W
    Biochemistry 2002 Feb 26;41(8):2535-42
  • Mutations in the proenteropeptidase gene are the molecular cause of congenital enteropeptidase deficiency
    Holzinger A, Maier EM, Buck C, Mayerhofer PU, Kappler M, Haworth JC, Moroz SP, Hadorn HB, Sadler JE, Roscher AA
    Am J Hum Genet 2002 Jan;70(1):20-5
  • Mucin-like domain of enteropeptidase directs apical targeting in Madin-Darby canine kidney cells
    Zheng X, Sadler JE
    J Biol Chem 2002 Mar 1;277(9):6858-63
  • Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura
    Zheng X, Chung D, Takayama TK, Majerus EM, Sadler JE, Fujikawa K
    J Biol Chem 2001 Nov 2;276(44):41059-63
  • Type 1 von Willebrand disease mutation Cys1149Arg causes intracellular retention and degradation of heterodimers: a possible general mechanism for dominant mutations of oligomeric proteins
    Bodo I, Katsumi A, Tuley EA, Eikenboom JC, Dong Z, Sadler JE
    Blood 2001 Nov 15;98(10):2973-9
  • A standard nomenclature for von Willebrand factor gene mutations and polymorphisms. On behalf of the ISTH SSC Subcommittee on von Willebrand factor
    Goodeve AC, Eikenboom JC, Ginsburg D, Hilbert L, Mazurier C, Peake IR, Sadler JE, Rodeghiero F
    Thromb Haemost 2001 May;85(5):929-31
  • Platelet glycoprotein Ib alpha binds to thrombin anion-binding exosite II inducing allosteric changes in the activity of thrombin
    Li CQ, Vindigni A, Sadler JE, Wardell MR
    J Biol Chem 2001 Mar 2;276(9):6161-8
  • Crystal structure of the human alpha-thrombin-haemadin complex: an exosite II-binding inhibitor
    Richardson JL, Kroger B, Hoeffken W, Sadler JE, Pereira P, Huber R, Bode W, Fuentes-Prior P
    EMBO J 2000 Nov 1;19(21):5650-60
  • Impact, diagnosis and treatment of von Willebrand disease
    Sadler JE, Mannucci PM, Berntorp E, Bochkov N, Boulyjenkov V, Ginsburg D, Meyer D, Peake I, Rodeghiero F, Srivastava A
    Thromb Haemost 2000 Aug;84(2):160-74
  • Acquired von Willebrand syndrome: data from an international registry
    Federici AB, Rand JH, Bucciarelli P, Budde U, van Genderen PJ, Mohri H, Meyer D, Rodeghiero F, Sadler JE
    Thromb Haemost 2000 Aug;84(2):345-9
  • Localization of disulfide bonds in the cystine knot domain of human von Willebrand factor
    Katsumi A, Tuley EA, Bodo I, Sadler JE
    J Biol Chem 2000 Aug 18;275(33):25585-25594
  • Activation of recombinant proenteropeptidase by duodenase
    Zamolodchikova TS, Sokolova EA, Lu D, Sadler JE
    FEBS Lett 2000 Jan 28;466(2-3):295-9
  • Localization of von willebrand factor-binding sites for platelet glycoprotein Ib and botrocetin by charged-to-alanine scanning mutagenesis
    Matsushita T, Meyer D, Sadler JE
    J Biol Chem 2000 Apr 14;275(15):11044-9
  • Structure of the Ser195Ala mutant of human alpha--thrombin complexed with fibrinopeptide A(7--16): evidence for residual catalytic activity
    Krishnan R, Sadler JE, Tulinsky A
    Acta Crystallogr D Biol Crystallogr 2000 Apr;56 ( Pt 4):406-10
  • Interaction of von Willebrand factor domain A1 with platelet glycoprotein Ibalpha-(1-289). Slow intrinsic binding kinetics mediate rapid platelet adhesion
    Miura S, Li CQ, Cao Z, Wang H, Wardell MR, Sadler JE
    J Biol Chem 2000 Mar 17;275(11):7539-46
  • Crystal structure of enteropeptidase light chain complexed with an analog of the trypsinogen activation peptide
    Lu D, Futterer K, Korolev S, Zheng X, Tan K, Waksman G, Sadler JE
    J Mol Biol 1999 Sep 17;292(2):361-73
  • Mechanism by which heparin proteoglycan modulates mast cell chymase activity
    Pejler G, Sadler JE
    Biochemistry 1999 Sep 14;38(37):12187-95
  • Apical sorting of bovine enteropeptidase does not involve detergent- resistant association with sphingolipid-cholesterol rafts
    Zheng X, Lu D, Sadler JE
    J Biol Chem 1999 Jan 15;274(3):1596-605
  • Incomplete embryonic lethality and fatal neonatal hemorrhage caused by prothrombin deficiency in mice
    Xue J, Wu Q, Westfield LA, Tuley EA, Lu D, Zhang Q, Shim K, Zheng X, Sadler JE
    Proc Natl Acad Sci U S A 1998 Jun 23;95(13):7603-7
  • Activation of thrombin-activable fibrinolysis inhibitor requires epidermal growth factor-like domain 3 of thrombomodulin and is inhibited competitively by protein C
    Kokame K, Zheng X, Sadler JE
    J Biol Chem 1998 May 15;273(20):12135-9
  • Type 2M von Willebrand disease: F606I and I662F mutations in the glycoprotein Ib binding domain selectively impair ristocetin- but not botrocetin-mediated binding of von Willebrand factor to platelets
    Hillery CA, Mancuso DJ, Sadler JE, Ponder JW, Jozwiak MA, Christopherson PA, Cox Gill J, Paul Scott J, Montgomery RR
    Blood 1998 Mar 1;91(5):1572-81
  • Structure of murine enterokinase (enteropeptidase) and expression in small intestine during development
    Yuan X, Zheng X, Lu D, Rubin DC, Pung CY, Sadler JE
    Am J Physiol 1998 Feb;274(2 Pt 1):G342-9
  • Generation and characterization of mice deficient in hepsin, a hepatic transmembrane serine protease
    Wu Q, Yu D, Post J, Halks-Miller M, Sadler JE, Morser J
    J Clin Invest 1998 Jan 15;101(2):321-6
  • Bovine proenteropeptidase is activated by trypsin, and the specificity of enteropeptidase depends on the heavy chain
    Lu D, Yuan X, Zheng X, Sadler JE
    J Biol Chem 1997 Dec 12;272(50):31293-300
  • Structure and localization of the thrombin receptor gene on mouse chromosome 13
    Xue J, Jenkins NA, Gilbert DJ, Copeland NG, Sadler JE
    Mamm Genome 1996 Aug;7(8):625-6
  • Dominant type 1 von Willebrand disease caused by mutated cysteine residues in the D3 domain of von Willebrand factor
    Eikenboom JC, Matsushita T, Reitsma PH, Tuley EA, Castaman G, Briet E, Sadler JE
    Blood 1996 Oct 1;88(7):2433-41
  • Analysis of Arg834Gln and Val902Glu type 2A von Willebrand disease mutations: studies with recombinant von Willebrand factor and correlation with patient characteristics
    Englender T, Lattuada A, Mannucci PM, Sadler JE, Inbal A
    Blood 1996 Apr 1;87(7):2788-94
  • Identification of amino acid residues essential for von Willebrand factor binding to platelet glycoprotein Ib. Charged-to-alanine scanning mutagenesis of the A1 domain of human von Willebrand factor
    Matsushita T, Sadler JE
    J Biol Chem 1995 Jun 2;270(22):13406-14
  • cDNA sequence and chromosomal localization of human enterokinase, the proteolytic activator of trypsinogen
    Kitamoto Y, Veile RA, Donis-Keller H, Sadler JE
    Biochemistry 1995 Apr 11;34(14):4562-8
  • Very low frequency of "normandy type" mutations among type 1 von Willebrand disease families [letter]
    Inbal A, Kornbrot N, Mannucci PM, Sadler JE
    Thromb Haemost 1995 Feb;73(2):324
  • Heparin cofactor II is regulated allosterically and not primarily by template effects. Studies with mutant thrombins and glycosaminoglycans
    Sheehan JP, Tollefsen DM, Sadler JE
    J Biol Chem 1994 Dec 30;269(52):32747-51
  • Structure of a nonadecapeptide of the fifth EGF domain of thrombomodulin complexed with thrombin
    Mathews II, Padmanabhan KP, Tulinksy A, Sadler JE
    Biochemistry 1994 Nov 22;33(46):13547-52
  • Characterization of partial gene deletions in type III von Willebrand disease with alloantibody inhibitors
    Mancuso DJ, Tuley EA, Castillo R, de Bosch N, Mannucci PM, Sadler JE
    Thromb Haemost 1994 Aug;72(2):180-5
  • Enterokinase, the initiator of intestinal digestion, is a mosaic protease composed of a distinctive assortment of domains
    Kitamoto Y, Yuan X, Wu Q, McCourt DW, Sadler JE
    Proc Natl Acad Sci U S A 1994 Aug 2;91(16):7588-92
  • Molecular mapping of the heparin-binding exosite of thrombin
    Sheehan JP, Sadler JE
    Proc Natl Acad Sci U S A 1994 Jun 7;91(12):5518-22
  • A revised classification of von Willebrand disease. For the Subcommittee on von Willebrand Factor of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis
    Sadler JE
    Thromb Haemost 1994 Apr;71(4):520-5
  • Disulfide bonds required to assemble functional von Willebrand factor multimers
    Dong Z, Thoma RS, Crimmins DL, McCourt DW, Tuley EA, Sadler JE
    J Biol Chem 1994 Mar 4;269(9):6753-8
  • Activation-induced exposure of the thrombin anion-binding exosite. Interactions of recombinant mutant prothrombins with thrombomodulin and a thrombin exosite-specific antibody
    Wu Q, Picard V, Aiach M, Sadler JE
    J Biol Chem 1994 Feb 4;269(5):3725-30
  • Effect of type IIB von Willebrand disease mutation Arg(545)Cys on platelet glycoprotein Ib binding--studies with recombinant von Willebrand factor
    Inbal A, Kornbrot N, Harrison P, Randi AM, Sadler JE
    Thromb Haemost 1993 Dec 20;70(6):1058-62
  • Type IIB mutation His-505-->Asp implicates a new segment in the control of von Willebrand factor binding to platelet glycoprotein Ib
    Rabinowitz I, Randi AM, Shindler KS, Tuley EA, Rustagi PK, Sadler JE
    J Biol Chem 1993 Sep 25;268(27):20497-501
  • The structure of alpha-thrombin inhibited by a 15-mer single-stranded DNA aptamer
    Padmanabhan K, Padmanabhan KP, Ferrara JD, Sadler JE, Tulinsky A
    J Biol Chem 1993 Aug 25;268(24):17651-4
  • Sequences required for thrombomodulin cofactor activity within the fourth epidermal growth factor-like domain of human thrombomodulin
    Lentz SR, Chen Y, Sadler JE
    J Biol Chem 1993 Jul 15;268(20):15312-7
  • Identification of three candidate mutations causing type IIA von Willebrand disease using a rapid, nonradioactive, allele-specific hybridization method
    Inbal A, Englender T, Kornbrot N, Randi AM, Castaman G, Mannucci PM, Sadler JE
    Blood 1993 Aug 1;82(3):830-6
  • New variant of von Willebrand disease type II with markedly increased levels of von Willebrand factor antigen and dominant mode of inheritance: von Willebrand disease type IIC Miami
    Ledford MR, Rabinowitz I, Sadler JE, Kent JW, Civantos F
    Blood 1993 Jul 1;82(1):169-75
  • Mutagenesis of thrombin selectively modulates inhibition by serpins heparin cofactor II and antithrombin III. Interaction with the anion- binding exosite determines heparin cofactor II specificity
    Sheehan JP, Wu Q, Tollefsen DM, Sadler JE
    J Biol Chem 1993 Feb 15;268(5):3639-45
  • Regulation of human tissue factor expression by mRNA turnover
    Ahern SM, Miyata T, Sadler JE
    J Biol Chem 1993 Jan 25;268(3):2154-9
  • Homocysteine inhibits von Willebrand factor processing and secretion by preventing transport from the endoplasmic reticulum
    Lentz SR, Sadler JE
    Blood 1993 Feb 1;81(3):683-9
  • A database of polymorphisms in the von Willebrand factor gene and pseudogene. For the Consortium on von Willebrand Factor Mutations and Polymorphisms and the Subcommittee on von Willebrand Factor of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis
    Sadler JE, Ginsburg D
    Thromb Haemost 1993 Feb 1;69(2):185-91
  • von Willebrand disease: a database of point mutations, insertions, and deletions. For the Consortium on von Willebrand Factor Mutations and Polymorphisms, and the Subcommittee on von Willebrand Factor of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis
    Ginsburg D, Sadler JE
    Thromb Haemost 1993 Feb 1;69(2):177-84
  • Localization of the single-stranded DNA binding site in the thrombin anion-binding exosite
    Wu Q, Tsiang M, Sadler JE
    J Biol Chem 1992 Dec 5;267(34):24408-12
  • Expression of human soluble tissue factor in yeast and enzymatic properties of its complex with factor VIIa
    Shigematsu Y, Miyata T, Higashi S, Miki T, Sadler JE, Iwanaga S
    J Biol Chem 1992 Oct 25;267(30):21329-37
  • von Willebrand disease type B: a missense mutation selectively abolishes ristocetin-induced von Willebrand factor binding to platelet glycoprotein Ib
    Rabinowitz I, Tuley EA, Mancuso DJ, Randi AM, Firkin BG, Howard MA, Sadler JE
    Proc Natl Acad Sci U S A 1992 Oct 15;89(20):9846-9
  • Recombinant von Willebrand factor Arg578-->Gln. A type IIB von Willebrand disease mutation affects binding to glycoprotein Ib but not to collagen or heparin
    Randi AM, Jorieux S, Tuley EA, Mazurier C, Sadler JE
    J Biol Chem 1992 Oct 15;267(29):21187-92
  • Characterization of three mutations causing von Willebrand disease type IIA in five unrelated families
    Inbal A, Seligsohn U, Kornbrot N, Brenner B, Harrison P, Randi A, Rabinowitz I, Sadler JE
    Thromb Haemost 1992 Jun 1;67(6):618-22
  • Functional domains of membrane-bound human thrombomodulin. EGF-like domains four to six and the serine/threonine-rich domain are required for cofactor activity
    Tsiang M, Lentz SR, Sadler JE
    J Biol Chem 1992 Mar 25;267(9):6164-70
  • Ligand specificity of human thrombomodulin. Equilibrium binding of human thrombin, meizothrombin, and factor Xa to recombinant thrombomodulin
    Wu Q, Tsiang M, Lentz SR, Sadler JE
    J Biol Chem 1992 Apr 5;267(10):7083-8
  • The mutation Arg (53)-->Trp causes von Willebrand disease Normandy by abolishing binding to factor VIII. Studies with recombinant von Willebrand factor
    Jorieux S, Tuley EA, Gaucher C, Mazurier C, Sadler JE
    Blood 1992 Feb 1;79(3):563-7
  • Refractory immune hemolytic anemia with a high thermal amplitude, low affinity IgG anti-Pra cold autoantibody
    Ramos RR, Curtis BR, Sadler JE, Eby CS, Chaplin H
    Autoimmunity 1992;12(2):149-54
  • Inhibition of thrombomodulin surface expression and protein C activation by the thrombogenic agent homocysteine
    Lentz SR, Sadler JE
    J Clin Invest 1991 Dec;88(6):1906-14
  • Single amino acid substitutions dissociate fibrinogen-clotting and thrombomodulin-binding activities of human thrombin
    Wu QY, Sheehan JP, Tsiang M, Lentz SR, Birktoft JJ, Sadler JE
    Proc Natl Acad Sci U S A 1991 Aug 1;88(15):6775-9
  • Expression of von Willebrand factor "Normandy": an autosomal mutation that mimics hemophilia A
    Tuley EA, Gaucher C, Jorieux S, Worrall NK, Sadler JE, Mazurier C
    Proc Natl Acad Sci U S A 1991 Jul 15;88(14):6377-81
  • Molecular basis of von Willebrand disease type IIB. Candidate mutations cluster in one disulfide loop between proposed platelet glycoprotein Ib binding sequences
    Randi AM, Rabinowitz I, Mancuso DJ, Mannucci PM, Sadler JE
    J Clin Invest 1991 Apr;87(4):1220-6
  • Induction of specific storage organelles by von Willebrand factor propolypeptide
    Wagner DD, Saffaripour S, Bonfanti R, Sadler JE, Cramer EM, Chapman B, Mayadas TN
    Cell 1991 Jan 25;64(2):403-13
  • Human von Willebrand factor gene and pseudogene: structural analysis and differentiation by polymerase chain reaction
    Mancuso DJ, Tuley EA, Westfield LA, Lester-Mancuso TL, Le Beau MM, Sorace JM, Sadler JE
    Biochemistry 1991 Jan 8;30(1):253-69
  • Regulation of thrombomodulin by tumor necrosis factor-alpha: comparison of transcriptional and posttranscriptional mechanisms
    Lentz SR, Tsiang M, Sadler JE
    Blood 1991 Feb 1;77(3):542-50
  • Synthetic RGDS-containing peptides of von Willebrand factor inhibit platelet adhesion to collagen
    Fressinaud E, Girma JP, Sadler JE, Baumgartner HR, Meyer D
    Thromb Haemost 1990 Dec 28;64(4):589-93
  • Family studies and prenatal diagnosis in severe von Willebrand disease by polymerase chain reaction amplification of a variable number tandem repeat region of the von Willebrand factor gene
    Peake IR, Bowen D, Bignell P, Liddell MB, Sadler JE, Standen G, Bloom AL
    Blood 1990 Aug 1;76(3):555-61
  • Equilibrium binding of thrombin to recombinant human thrombomodulIn: effect of hirudin, fibrinogen, factor Va, and peptide analogues
    Tsiang M, Lentz SR, Dittman WA, Wen D, Scarpati EM, Sadler JE
    Biochemistry 1990 Nov 27;29(47):10602-12
  • Severe type III von Willebrand's disease caused by deletion of exon 42 of the von Willebrand factor gene: family studies that identify carriers of the condition and a compound heterozygous individual
    Peake IR, Liddell MB, Moodie P, Standen G, Mancuso DJ, Tuley EA, Westfield LA, Sorace JM, Sadler JE, Verweij CL, et al
    Blood 1990 Feb 1;75(3):654-61
  • Regulation of endothelial cell coagulant properties. Modulation of tissue factor, plasminogen activator inhibitors, and thrombomodulin by phorbol 12-myristate 13-acetate and tumor necrosis factor [published erratum appears in J Biol Chem 1990 Aug 25;265(24):14696]
    Scarpati EM, Sadler JE
    J Biol Chem 1989 Dec 5;264(34):20705-13
  • Structure of the gene for human von Willebrand factor
    Mancuso DJ, Tuley EA, Westfield LA, Worrall NK, Shelton-Inloes BB, Sorace JM, Alevy YG, Sadler JE
    J Biol Chem 1989 Nov 25;264(33):19514-27
  • Use of yeast artificial chromosome clones for mapping and walking within human chromosome segment 18q21.3
    Silverman GA, Ye RD, Pollock KM, Sadler JE, Korsmeyer SJ
    Proc Natl Acad Sci U S A 1989 Oct;86(19):7485-9
  • Regional localization of the human thrombomodulin gene to 20p12-cen
    Espinosa R 3d, Sadler JE, Le Beau MM
    Genomics 1989 Oct;5(3):649-50
  • Structure of the gene for human plasminogen activator inhibitor-2. The nearest mammalian homologue of chicken ovalbumin
    Ye RD, Ahern SM, Le Beau MM, Lebo RV, Sadler JE
    J Biol Chem 1989 Apr 5;264(10):5495-502
  • Effects of cellular transformation on expression of plasminogen activator inhibitors 1 and 2. Evidence for independent regulation
    Cohen RL, Niclas J, Lee WM, Wun TC, Crowley CW, Levinson AD, Sadler JE, Shuman MA
    J Biol Chem 1989 May 15;264(14):8375-83
  • The structure and function of mouse thrombomodulin. Phorbol myristate acetate stimulates degradation and synthesis of thrombomodulin without affecting mRNA levels in hemangioma cells
    Dittman WA, Kumada T, Sadler JE, Majerus PW
    J Biol Chem 1988 Oct 25;263(30):15815-22
  • Mammalian protein secretion without signal peptide removal. Biosynthesis of plasminogen activator inhibitor-2 in U-937 cells
    Ye RD, Wun TC, Sadler JE
    J Biol Chem 1988 Apr 5;263(10):4869-75
  • Identification and mapping of RFLPs for human tissue factor (HTF) to chromosome 1p
    Scarpati EM, Sadler JE, O'Connell P, Nakamura Y, Leppert M, Ballard L, Lathrop GM, Lalouel JM, White R
    Nucleic Acids Res 1987 Nov 11;15(21):9098
  • A primary genetic linkage map for human chromosome 12
    O'Connell P, Lathrop GM, Law M, Leppert M, Nakamura Y, Hoff M, Kumlin E, Thomas W, Elsner T, Ballard L, Goodman P, Azen E, Sadler JE, Lai GY, Lalouel J-M, White R
    Genomics 1987; 1: 93-102
  • Human tissue factor: cDNA sequence and chromosome localization of the gene
    Scarpati EM, Wen D, Broze GJ Jr, Miletich JP, Flandermeyer RR, Siegel NR, Sadler JE
    Biochemistry 1987 Aug 25;26(17):5234-8
  • Human thrombomodulIn: complete cDNA sequence and chromosome localization of the gene
    Wen DZ, Dittman WA, Ye RD, Deaven LL, Majerus PW, Sadler JE
    Biochemistry 1987 Jul 14;26(14):4350-7
  • Evolution of human von Willebrand factor: cDNA sequence polymorphisms, repeated domains, and relationship to von Willebrand antigen II
    Shelton-Inloes BB, Broze GJ Jr, Miletich JP, Sadler JE
    Biochem Biophys Res Commun 1987 Apr 29;144(2):657-65
  • cDNA cloning and expression in Escherichia coli of a plasminogen activator inhibitor from human placenta
    Ye RD, Wun TC, Sadler JE
    J Biol Chem 1987 Mar 15;262(8):3718-25
  • Gene deletions correlate with the development of alloantibodies in von Willebrand disease
    Shelton-Inloes BB, Chehab FF, Mannucci PM, Federici AB, Sadler JE
    J Clin Invest 1987 May;79(5):1459-65
  • Linkage disequilibrium of plasminogen polymorphisms and assignment of the gene to human chromosome 6q26-6q27
    Murray JC, Buetow KH, Donovan M, Hornung S, Motulsky AG, Disteche C, Dyer K, Swisshelm K, Anderson J, Giblett E, Sadler E, Eddy R, Shows TB
    Am J Hum Genet 1987; 40: 338-350
  • Amino acid sequence of human von Willebrand factor
    Titani K, Kumar S, Takio K, Ericsson LH, Wade RD, Ashida K, Walsh KA, Chopek MW, Sadler JE, Fujikawa K
    Biochemistry 1986 Jun 3;25(11):3171-84
  • cDNA sequences for human von Willebrand factor reveal five types of repeated domains and five possible protein sequence polymorphisms
    Shelton-Inloes BB, Titani K, Sadler JE
    Biochemistry 1986 Jun 3;25(11):3164-71
  • Cloning and characterization of two cDNAs coding for human von Willebrand factor
    Sadler JE, Shelton-Inloes BB, Sorace JM, Harlan JM, Titani K, Davie EW
    Proc Natl Acad Sci U S A 1985 Oct;82(19):6394-8
  • Characterization of a complementary deoxyribonucleic acid coding for human and bovine plasminogen
    Malinowski DP, Sadler JE, Davie EW
    Biochemistry 1984 Aug 28;23(18):4243-50
  • Purification to homogeneity of H blood group beta-galactoside alpha 1 leads to 2 fucosyltransferase from porcine submaxillary gland
    Beyer TA, Sadler JE, Hill RL
    J Biol Chem 1980 Jun 10;255(11):5364-72
  • Biosynthesis of mammalian glycoproteins. Glycosylation pathways in the synthesis of the nonreducing terminal sequences
    Beyer TA, Rearick JI, Paulson JC, Prieels JP, Sadler JE, Hill RL
    J Biol Chem 1979 Dec 25;254(24):12531-4
  • Purification to homogeneity and enzymatic characterization of an alpha- N-acetylgalactosaminide alpha 2 leads to 6 sialyltransferase from porcine submaxillary glands
    Sadler JE, Rearick JI, Hill RL
    J Biol Chem 1979 Jul 10;254(13):5934-41
  • Enzymatic characterization of beta D-galactoside alpha2 leads to 3 sialyltransferase from porcine submaxillary gland
    Rearick JI, Sadler JE, Paulson JC, Hill RL
    J Biol Chem 1979 Jun 10;254(11):4444-51
  • The calculation of some physical parameters of proteins from sucrose density gradient centrifugation data
    Sadler JE
    J Biol Chem 1979 Jun 10;254(11):4443
  • Purification to homogeneity of a beta-galactoside alpha2 leads to 3 sialyltransferase and partial purification of an alpha-N- acetylgalactosaminide alpha2 leads to 6 sialyltransferase from porcine submaxillary glands
    Sadler JE, Rearick JI, Paulson JC, Hill RL
    J Biol Chem 1979 Jun 10;254(11):4434-42
  • Restoration of specific myxovirus receptors to asialoerythrocytes by incorporation of sialic acid with pure sialyltransferases
    Paulson JC, Sadler JE, Hill RL
    J Biol Chem 1979 Mar 25;254(6):2120-4
  • The role of sialic acid in the expression of human MN blood group antigens
    Sadler JE, Paulson JC, Hill RL
    J Biol Chem 1979 Mar 25;254(6):2112-9
  • Ring 'flipping' in bis(cyclo-octatetraene)titanium(II); an organometallic intramolecular redox reaction which involves only the geometrical deformation of organic ligands
    Schwartz J, Sadler JE
    JCS ChemCommun 1973; 172-173