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Adjuvant low-dose rituximab and plasma exchange for acquired TTP.

Zwicker JI, Muia J, Dolatshahi L, Westfield LA, Nieters P, Rodrigues A, Hamdan A, Antun AG, Metjian A, Sadler JEE.

Blood. 2019 Jul 22. pii: blood.2019000795. doi: 10.1182/blood.2019000795. [Epub ahead of print] No abstract available.


Phylogenetic and functional analysis of ADAMTS13 identifies highly conserved domains essential for allosteric regulation.

Muia J, Zhu J, Greco SC, Vanhoorelbeke K, Gupta G, Westfield LA, Sadler JE.

Blood. 2019 Apr 25;133(17):1899-1908. doi: 10.1182/blood-2018-11-886275. Epub 2019 Jan 30.


Exploring the "minimal" structure of a functional ADAMTS13 by mutagenesis and small-angle X-ray scattering.

Zhu J, Muia J, Gupta G, Westfield LA, Vanhoorelbeke K, Tolia NH, Sadler JE.

Blood. 2019 Apr 25;133(17):1909-1918. doi: 10.1182/blood-2018-11-886309. Epub 2019 Jan 28.


Pathophysiology of thrombotic thrombocytopenic purpura.

Sadler JE.

Blood. 2017 Sep 7;130(10):1181-1188. doi: 10.1182/blood-2017-04-636431. Epub 2017 Aug 2. Review.


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. doi: 10.1038/nsmb.3333. Epub 2016 Dec 5.


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):1725-35. doi: 10.1111/jth.13398. Epub 2016 Aug 20.


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. doi: 10.1160/TH15-09-0700. Epub 2016 Apr 7.


What's new in the diagnosis and pathophysiology of thrombotic thrombocytopenic purpura.

Sadler JE.

Hematology Am Soc Hematol Educ Program. 2015;2015:631-6. doi: 10.1182/asheducation-2015.1.631.


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):2063-75. doi: 10.1111/jth.13149. Epub 2015 Oct 20.


Atypical HUS may become a diagnosis of inclusion.

Sadler JE.

Blood. 2015 Jun 4;125(23):3525-6. doi: 10.1182/blood-2015-04-640656.


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. doi: 10.1073/pnas.1413282112. Epub 2014 Dec 15.


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. doi: 10.1074/jbc.M113.535963. Epub 2014 Feb 18.


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. doi: 10.1021/mp400210q. Epub 2013 Oct 10.


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. doi: 10.1111/jth.12319.


von Willebrand factor in its native environment.

Sadler JE.

Blood. 2013 Apr 4;121(14):2583-4. doi: 10.1182/blood-2013-02-482000. No abstract available.


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. doi: 10.1182/blood-2012-01-408096. Epub 2012 Jun 20.


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. doi: 10.1074/jbc.M112.380535. Epub 2012 Jun 15.


Weibel-Palade bodies.

Sadler JE.

Clin Adv Hematol Oncol. 2011 Sep;9(9):673-4. No abstract available.


Should all adult patients with diarrhoea-associated HUS receive plasma exchange?

Tarr PI, Sadler JE, Chandler WL, George JN, Tsai HM.

Lancet. 2012 Feb 11;379(9815):516; author reply 516-7. doi: 10.1016/S0140-6736(12)60225-6. No abstract available.


Surface functionalization of electro-deposited nickel.

Sadler JE, Szumski DS, Kierzkowska A, Catarelli SR, Stella K, Nichols RJ, Fonticelli MH, Benitez G, Blum B, Salvarezza RC, Schwarzacher W.

Phys Chem Chem Phys. 2011 Oct 28;13(40):17987-93. doi: 10.1039/c1cp22203a. Epub 2011 Sep 15.


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. doi: 10.1182/blood-2011-06-363648. Epub 2011 Aug 3.


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. doi: 10.1074/jbc.M111.249151. Epub 2011 May 17.


Functional architecture of Weibel-Palade bodies.

Valentijn KM, Sadler JE, Valentijn JA, Voorberg J, Eikenboom J.

Blood. 2011 May 12;117(19):5033-43. doi: 10.1182/blood-2010-09-267492. Epub 2011 Jan 25. Review.


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. doi: 10.1182/blood-2010-02-271957. Epub 2010 Jul 19.


Redeeming ristocetin.

Sadler JE.

Blood. 2010 Jul 15;116(2):155-6. doi: 10.1182/blood-2010-04-276394. No abstract available.


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. doi: 10.1016/j.bpj.2009.12.4324.


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. doi: 10.1111/j.1538-7836.2010.03753.x. Epub 2010 Jan 17.


Low von Willebrand factor: sometimes a risk factor and sometimes a disease.

Sadler JE.

Hematology Am Soc Hematol Educ Program. 2009:106-12. doi: 10.1182/asheducation-2009.1.106. Review.


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. doi: 10.1016/j.str.2009.09.011.


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. doi: 10.1111/j.1538-7836.2009.03620.x. Epub 2009 Sep 18.


von Willebrand factor assembly and secretion.

Sadler JE.

J Thromb Haemost. 2009 Jul;7 Suppl 1:24-7. doi: 10.1111/j.1538-7836.2009.03375.x. Review.


Enteropeptidase, a type II transmembrane serine protease.

Zheng XL, Kitamoto Y, Sadler JE.

Front Biosci (Elite Ed). 2009 Jun 1;1:242-9. Review.


Clinical and laboratory diagnosis of von Willebrand disease: a synopsis of the 2008 NHLBI/NIH guidelines.

Nichols WL, Rick ME, Ortel TL, Montgomery RR, Sadler JE, Yawn BP, James AH, Hultin MB, Manco-Johnson MJ, Weinstein M.

Am J Hematol. 2009 Jun;84(6):366-70. doi: 10.1002/ajh.21405.


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. doi: 10.1182/blood-2008-05-158584. Epub 2008 Oct 16.


Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura.

Sadler JE.

Blood. 2008 Jul 1;112(1):11-8. doi: 10.1182/blood-2008-02-078170. Review.


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. doi: 10.1182/blood-2008-04-148759. Epub 2008 May 20.


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):363-370. doi: 10.1007/s12185-008-0065-8.


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. doi: 10.1111/j.1365-2516.2007.01643.x.


Pathogenesis of thrombotic microangiopathies.

Zheng XL, Sadler JE.

Annu Rev Pathol. 2008;3:249-77. doi: 10.1146/annurev.pathmechdis.3.121806.154311. Review.


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. doi: 10.1073/pnas.0710079105. Epub 2008 Jan 8.


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. Epub 2007 Sep 27.


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. Epub 2007 Sep 25.


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; SERF-TTP Research Group.

J Am Coll Cardiol. 2007 Sep 18;50(12):1138-43. Epub 2007 Sep 4.


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. Epub 2006 Dec 4.


Thrombotic thrombocytopenic purpura: a moving target.

Sadler JE.

Hematology Am Soc Hematol Educ Program. 2006:415-20. Review.


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; Working Party on von Willebrand Disease Classification.

J Thromb Haemost. 2006 Oct;4(10):2103-14. Epub 2006 Aug 2. Review.


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. Epub 2005 Dec 5.


Zinc and calcium ions cooperatively modulate ADAMTS13 activity.

Anderson PJ, Kokame K, Sadler JE.

J Biol Chem. 2006 Jan 13;281(2):850-7. Epub 2005 Nov 11.


von Willebrand factor: two sides of a coin.

Sadler JE.

J Thromb Haemost. 2005 Aug;3(8):1702-9. Review.

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