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Department of Internal Medicine

Hematology, Oncology and Blood & 
Marrow Transplantation Faculty

Steven R. Lentz, M.D., Ph.D.

Additional Publications

Peer-reviewed Publications

  1. Uhing, R.J., Lentz, S.R., and Graves, D.J. Effects of 1,2-dimethoxyethane on the catalytic and coenzyme properties of glycogen phosphorylase. (1981) Biochemistry 20:2537-2544.
  2. Fernley, R.T., Lentz, S.R., and Bradshaw, R.A. Malate dehydrogenase: isolation from E. coli and comparison with the eucaryotic mitochondrial and cytoplasmic forms. (1981) Bioscience Reports 1:497-507.
  3. Gordon, J.I., Simms, H.F., Lentz, S.R., Edelstein, C., Scanu, A.M., and Strauss, A.W. Proteolytic processing of human preproapolipoprotein A-I. A proposed defect in the conversion of pro A-I to A-I in Tangier's disease. (1983) J. Biol. Chem . 258:4037-40444.
  4. Lentz, S.R., Birken, S., Lustbader, J., and Boime, I. Posttranslational modification of the carboxy terminal region of the subunit of human chorionic gonadotropin. (1984) Biochemistry 23:5330-5337.
  5. Lentz, S.R., Raish, R.J., Orlowski, E.P., and Marion, J.M. Squamous cell carcinoma in epidermolysis bullosa: treatment with systemic chemotherapy. (1990) Cancer 66:1276-1278.
  6. Tsiang, M., Lentz, S.R., Dittman, W.A., Wen, D., Scarpati, E.M., and Sadler, J.E. Equilibrium binding of thrombin to recombinant human thrombomodulin: effect of hirudin, fibrinogen, factor Va, and peptide analogues. (1990) Biochemistry 29:10602-10612.
  7. Lentz, S.R., Tsiang, M., and Sadler, J.E. Regulation of thrombomodulin by tumor necrosis factor- : comparison of transcriptional and posttranscriptional mechanisms. (1991) Blood 77:542-550.
  8. Wu, Q., Sheehan, J.P., Tsiang, M., Lentz, S.R., Birktoft, J.J., and Sadler, J.E. Single amino acid substitutions dissociate fibrinogen-clotting and thrombomodulin-binding activities of human thrombin. (1991) Proc. Natl. Acad. Sci. USA 88:6775-6779.
  9. Lentz, S.R. and Sadler, J.E. Inhibition of thrombomodulin surface expression and protein C activation by the thrombogenic agent homocysteine. (1991) J. Clin. Invest. 88:1906-1914.
  10. Tsiang, M., Lentz, S.R., and Sadler, J.E. Functional domains of membrane-bound human thrombomodulin: EGF-like domains four to six and the serine/threonine-rich domain are required for cofactor activity. (1992) J. Biol. Chem. 267:6164-6170.
  11. Wu, Q., Tsiang, M., Lentz, S.R., and Sadler, J.E. Ligand specificity of human thrombomodulin: equilibrium binding of human thrombin, meizothrombin and factor Xa to recombinant thrombomodulin. (1992) J. Biol. Chem. 267:7083-7088.
  12. Lentz, S.R., Krewson, L, and Zutter, M.M. Recurrent neuroendocrine (Merkel cell) carcinoma of the skin presenting as marrow failure in a man with systemic lupus erythematosus. (1993) Med. Pediatr. Oncol. 21:137-141.
  13. Lentz, S.R. and Sadler, J.E. Homocysteine inhibits von Willebrand factor processing and secretion by preventing transport from the endoplasmic reticulum. (1993) Blood 81:683-689.
  14. Lentz, S.R., Chen. Y., and Sadler, J.E. Sequences required for thrombomodulin cofactor activity within the fourth epidermal growth factor-like domain of human thrombomodulin. (1993) J. Biol. Chem. 268:15312-15317.
  15. Raife, T.J., Lager, D.J., Madison, K.C., Piette, W.W., Howard, E.J., Sturm, M.T., Chen, Y. and Lentz, S.R. Thrombomodulin expression by human keratinocytes. Induction of cofactor activity during epidermal differentiation. (1994) J. Clin. Invest. 93:1846-1851.
  16. Lager, D.J., Callaghan, E.J., Worth, S.F., Raife, T.J., and Lentz, S.R. Cellular localization of thrombomodulin in human epithelium and squamous malignancies. (1995) Am. J. Pathol. 146:933-943.
  17. Schlueter, A.J., Al-Jurf, A.S., and Lentz, S.R. Kung fu phlebitis: an unusual presentation of Mondor's disease. (1996) Am. J. Hematol. 52:66-67.
  18. Lentz, S.R., Sobey, C.G., Piegors, D.J., Bhopatkar, M.Y., Faraci, F.M., Malinow, M.R., and Heistad, D.D. Vascular dysfunction in monkeys with diet-induced hyperhomocyst(e)inemia. (1996) J. Clin. Invest. 98:24-29.
  19. Raife, T.J., Demetroulis, E.M., and Lentz, S.R. Regulation of thrombomodulin expression by all-trans retinoic acid and tumor necrosis factor- a : differential responses in keratinocytes and endothelial cells. (1996) Blood 88:2043-2049.
  20. Newell, A.A, Raife, T.J., and Lentz, S.R. Interference in protein C activation assays from endogenous serpins in mammalian host cells. (1996) Thromb. Res. 84:373-377.
  21. Raife, T. J., Olson, J.D, and Lentz, S.R. Platelet antibody testing in idiopathic thrombocytopenic purpura. (1997) Blood 89:1112-1113.
  22. Joyce, D.E., Chen, Y., Erger, R.A., Koretzky, G.A., and Lentz, S.R. Functional interactions between the thrombin receptor and the T cell antigen receptor in human T cell lines. Blood (1997) 90:1893-1901.
  23. Lentz, S.R., Malinow, M.R., Piegors, D.J., Bhopatkar-Teredesai, M., Faraci, F.M., and Heistad, D.D. Consequences of hyperhomocyst(e)inemia on vascular function in atherosclerotic monkeys. Arterioscler. Thromb. Vasc. Biol. (1997) 17:2930-2934.
  24. Raife, T.J., Lager, D.J., Peterson, J.J., Erger, R.A., and Lentz, S.R. Keratinocyte-specific expression of human thrombomodulin in transgenic mice. Effects on epidermal differentiation and cutaneous wound healing. J. Investigat. Med. (1998) 46:127-133.
  25. Raife, T. J., McArthur, J., Peters, C., Kisker, C. T., and Lentz, S.R. Remission after 13-cis retinoic acid treatment in a case of refractory thrombotic thrombocytopenic purpura. Lancet (1998) 352:454-455.
  26. Clements, J.L., Lee, J.R., Yang, B., Olson, J.D., Sandra, A., Lentz, S.R., and Koretzky, G.A. Fetal hemorrhage and platelet dysfunction in SLP-76 deficient mice . J. Clin. Invest. (1999) 103:19-25.
  27. Lentz, S.R., Fernandez, J.A., Griffin, J.H., Piegors, D.J., Erger, R.A., Malinow, M.R., and Heistad, D.D. Impaired anticoagulant response to infusion of thrombin in atherosclerotic monkeys associated with acquired defects in the protein C system. Arterioscler. Thromb. Vasc. Biol. (1999) 19:1744-1750.
  28. Peterson, J.J., Rayburn, H.B., Lager, D.J., Raife, T.J., Kealey, G.P., Rosenberg, R.D., and Lentz, S.R. Expression of thrombomodulin and consequences of thrombomodulin deficiency during healing of cutaneous wounds. Am. J. Pathol. (1999) 155:1569-1575.
  29. Böger, R.H., Bode-Böger, S.M., Heistad, D.D., Frolich, J.C. and Lentz, S.R. Plasma concentration of asymmetric dimethylarginine, an endogenous inhibitor of nitric oxide synthase, is elevated in monkeys with hyperhomocyst(e)inemia. Arterioscler. Thromb. Vasc. Biol. (2000) 20:1557-1564.
  30. Mudd, S.H, Finkelstein, J.D., Refsum, H., Ueland, P.M., Lentz, S.R., Jacobsen, D.W., Brattström, L., Wilcken, B., Wilcken, D.E.L., Blom, H.J., Stabler, S.P., Allen, R.H., Selhub, J., and Rosenberg, I.H. Homocysteine and its disulfide derivatives: a suggested consensus terminology. Arterioscler. Thromb. Vasc. Biol. (2000) 20:1704-1706.
  31. Lentz, S.R., Erger, R.A., Dayal, S., Maeda, N., Malinow, M.R., Heistad, D.D., and Faraci, F.M. Folate dependence of hyperhomocysteinemia and vascular dysfunction in cystathionine b -synthase-deficient mice. Am. J. Physiol. Heart. Circ. Physiol. (2000) 279:H970-H975.
  32. Joyce, D.E, Piette, W.W., Stone, M.S., Carlisle, T.L., and Lentz, S.R. Severe acquired protein S deficiency and skin necrosis in a patient with ANCA-associated vasculitis. Thromb. Haemost. (2000) 84:929-930.
  33. Böger, R.H., Lentz, S.R., Bode-Böger, S.M., Knapp, H.R., and Haynes, W.G. Elevation of asymmetric dimethylarginine may mediate endothelial dysfunction during experimental hyperhomocyst(e)inemia in humans. Clin. Sci. (2001) 100:161-167.
  34. Lentz, S. R. Piegors, D.J., Malinow, M.R., and Heistad, D.D. Supplementation of atherogenic diet with B vitamins does not prevent atherosclerosis or vascular dysfunction in monkeys. Circulation (2001) 103:1006-1011.
  35. Werstuck, G.H., Lentz, S.R., Dayal, S., Hossain, G.S., Sood, S.K., Shi, Y.Y., Zhou, J., Maeda, N., Krisans, S.K., Malinow, M.R., and Austin, R.C. Homocysteine-induced endoplasmic reticulum stress causes dysregulation of the cholesterol and fatty acid biosynthesis pathways . J. Clin. Invest. (2001) 107:1263-1273.
  36. Dayal, S., Bottiglieri, T., Arning, E., Maeda, N., Malinow, M.R., Sigmund, C.D., Heistad, D.D., Faraci, F.M., and Lentz, S.R. Endothelial dysfunction and elevation of S-adenosylhomocysteine in cystathionine b-synthase-deficient mice. Circ. Res. (2001) 88:1203-1209.
  37. Piette, W.W. and Lentz, S.R. Endothelial protein C activation in meningococcal sepsis (Letter). N. Eng. J. Med. (2001) 345:1776.
  38. Raife, T.J., Lentz, S.R., Atkinson, B., Vesely, S.K., and Hessner, M.J. Factor V Leiden : a genetic risk factor for thrombotic microangiopathy in patients with normal von Willebrand factor-cleaving protease activity. Blood (2002) 99:437-442.
  39. Rosenfeld, S.B., Watkinson, K.K., Thompson, B.H., Macfarlane, D.E., and Lentz, S.R. Pulmonary embolism after sequential use of recombinant factor VIIa and activated prothrombin complex concentrate in a factor VIII inhibitor patient. Thromb. Haemost. (2002) 87:925-926.
  40. Lentz, S.R., Miller, F.J., Jr., Piegors, D.J., Erger, R.A., Fernández, J.A., Griffin, J.H., and Heistad, D.D. Anticoagulant responses to thrombin are enhanced during regression of atherosclerosis in monkeys. Circulation (2002) 106:842-846.
  41. Lentz, S.R., Piegors, D.J., Fernández, J.A., Erger, R.A., Arning, E., Malinow, M.R., Griffin, J.H., Bottiglieri, T., Haynes, W.G., and Heistad, D.D. Effect of hyperhomocysteinemia on protein C activation and activity. Blood (2002) 100:2108-2112.
  42. Leo, L., Di Paola, J., Judd, B.A., Koretzky, G.A., and Lentz, S.R. Role of the adapter protein SLP-76 in GPVI-dependent platelet procoagulant responses to collagen. Blood (2002) 100:2839-2844.
  43. Baumbach, G.L., Sigmund, C.D., Bottiglieri, T., and Lentz, S.R. Structure of cerebral arterioles in cystathionine b -synthase-deficient mice. Circ. Res. (2002) 91:931-937.
  44. Dayal, S., Brown, K.L, Weydert, C.J., Oberley, L.W., Arling, E., Bottiglieri, T., Faraci, F.M., and Lentz, S.R. Deficiency of glutathione peroxidase-1 sensitizes hyperhomocysteinemic mice to endothelial dysfunction. Arterioscler. Thromb. Vasc. Biol. (2002) 22:1996-2002.
  45. Slungaard, A., Fernández, J.A., Griffin, J.H., Key, N.L., Long, J.L., Piegors, D.J., and Lentz, S.R. Platelet factor 4 enhances generation of activated protein C in vitro and in vivo. Blood (2003) 102:146-151.
  46. Elzey, B.D., Tian, J., Jensen, R.J., Swanson, A.K., Lees, J.R., Lentz, S.R., Stein, C., Nieswandt, B., Wang, Y., Davidson, B., and Ratliff, T.L. Platelet-mediated modulation of adaptive immunity: a communication link between innate and adaptive immune compartments. Immunity (2003) 19:9-19.
  47. Wilson, K.M., Lynch, C.M., Faraci, F.M., and Lentz, S.R. Effect of mechanical ventilation on carotid artery thrombosis induced by photochemical injury in mice. J. Thromb. Haemost . (2003) 1:2669-2674.
  48. Devlin, A.M., Arning, E., Bottiglieri, T., Faraci, F.M., Rozen, R., and Lentz, S.R. Effect of MTHFR genotype on diet-induced hyperhomocysteinemia and vascular dysfunction in mice. Blood (2004) 103:2624-2629.
  49. Vitvitsky, V., Dayal, S., Stabler, S., Wang, H., Lentz, S.R., and Banerjee, R. Perturbations in homocysteine-linked redox homeostasis in a murine model for hyperhomocysteinemia. Am. J. Physiol. (2004) 287:R39-R46.
  50. Zhou, J., Werstuck, G.H., Lhoták, S., de Koning, A.B.L., Sood, S.K., Hossain, G.S., Møller, J., Ritskes-Hoitinga, M., Falk, E., Dayal, S., Lentz, S.R., and Austin, R.C. Association of multiple cellular stress pathways with accelerated atherosclerosis in hyperhomocysteinemic apoE-deficient mice. Circulation (2004) 110:207-213.
  51. Dayal, S., Arning, E., Bottiglieri, T., Böger, R.H., Sigmund, C.D., Faraci, F.M., and Lentz, S.R. Cerebral vascular dysfunction mediated by superoxide in hyperhomocysteinemic mice. Stroke (2004) 35:1957-1962.
  52. Crist, S.A., Elzey, B.D., Ludwig, A.T., Griffith, T.S., Staack, J.B., Lentz, S.R., and Ratliff, T.L. Expression of TNF-related apoptosis-inducing ligand (TRAIL) in megakaryocytes and platelets. Exp. Hematol. (2004) 32:1073-1081.
  53. Wilson, K.M. and Lentz, S.R. Need for mechanical ventilation with murine thrombosis models (Letter). Thromb. Haemost. (2005) 95:389.
  54. Devlin, A.M., Bottiglieri, T., Domann, F.E., and Lentz, S.R. Tissue-specific changes in H19 methylation and expression in mice with hyperhomocysteinemia. J. Biol. Chem. (2005) 280:25506-25511.
  55. Dayal, S., Devlin, A.M., McCaw, R.B., Liu, M.L., Arning, E., Bottiglieri, T., Shane, B., Faraci, F.M., and Lentz, S.R. Cerebral vascular dysfunction in methionine synthase-deficient mice. Circulation (2005) 112:737-744.
  56. Jobe, S.M., Leo, L., Eastvold, J., Dickneite, G., Ratliff, T.L., Lentz, S.R., and Di Paola, J. Role of FcR? and factor XIIIA in coated platelet formation. Blood (in press).
  57. Crist, S.A., Elzey, B.D., Staack, J.B., Sinn, H.W., Lentz, S.R., and Ratliff, T.L. Regulation of Fas ligand expression in megakaryocytes. (submitted, in revision).
  58. Kitayama, J., Faraci, F.M., Lentz, S.R., and Heistad, D.D. Cerebral vascular dysfunction in hypercholesterolemia. (submitted).
  59. Symons, J.D., Zaid, U.B., Athanassious, C.N., Mullick, A.E., Lentz, S.R., and Rutledge, J.C. Influence of folate on arterial permeability and stiffness in the absence or presence of hyperhomocysteinemia. (in preparation).
  60. Dayal, S., Wilson, K.M., Leo, L., Arning, E., Bottiglieri, T., and Lentz, S.R. Hyperhomocysteinemia accelerates carotid artery thrombosis in mice. (in preparation).
  61. Wilson, K.M., McCaw, R.B., Leo, L., Arning, E., Lhoták, S., Bottiglieri, Austin, R.C., and Lentz, S.R. Prothrombotic effect of mild hyperhomocysteinemia in apolipoprotein E-deficient mice. (in preparation).
  62. Raife, T.J., Dwyre, D.M., Erger, R.A., Wilson, K.M., Fernández, J.A., Altenburg, J., Kim, H-S., Griffin, J.H., Maeda, N., and Lentz, S. R. Human thrombomodulin knockin mice exhibit decreased protein C activation and accelerated thrombosis but have unaltered systemic response to endotoxin. (in preparation).

Chapters, Reviews, and Editorials

  1. Lentz, S.R. Disorders of hemostasis. In: Manual of Medical Therapeutics, 27th ed. Woodley, M. and Whelan, A., editors. Little, Brown and Company, Boston. (1992) 323-340.
  2. Sadler, J.E., Lentz, S.R., Sheehan, J.P., Tsiang, M., Wu, Q. Structure-function relationships of the thrombin-thrombomodulin interaction. (1993) Haemostasis 23
    (suppl 1):183-193.
  3. Lentz, S.R. and Sadler, J.E. The molecular basis of thrombomodulin function. In: Thrombin, thrombomodulin, and the control of hemostasis. Giddings, J.C., editor. R.G. Landes Company, Austin, TX. (1994) 91-120.
  4. Heistad, D.D, Lentz, S.R., and Rios, C.D. Atherosclerotic vascular disease: will folate or gene therapy be useful? Trans. Am. Clin. Climatol. Assoc. (1996) 108:69-77.
  5. Heistad, D.D, Lentz, S.R., Hayreh, S.S., and Faraci, F.M. Cerebral vascular effects of atherosclerosis and homocysteine: potential target for gene therapy. In: Proceedings of the 20 th Princeton Conference. Futura. (1997).
  6. Lentz, S.R. Hypercoagulable states and cerebrovascular disease. In: Neuroba se. Gilman, S., Goldstein, G.W, and Waxman, S.G., editors. Arbor Publishing Corp, La Jolla, CA, 1997 ed.
  7. Lentz, S.R. Homocysteine and vascular dysfunction. (1997) Life Sciences 61:1205-1215.
  8. Lentz, S.R. Hyperhomocysteinemia. In: Neuroba se. Gilman, S., Goldstein, G.W, and Waxman, S.G., editors. Arbor Publishing Corp, La Jolla, CA, 1998 ed.
  9. Lentz, S.R. Hypercoagulable states and cerebrovascular disease. In: Neuroba se. Gilman, S., Goldstein, G.W, and Waxman, S.G., editors. Arbor Publishing Corp, La Jolla, CA, 1998 ed.
  10. Lentz, S.R. Mechanisms of thrombosis in hyperhomocysteinemia. (1998) Current Opinion in Hematology 5:343-349.
  11. Lentz, S.R. Hyperhomocysteinemia. In: Neuroba se. Gilman, S., Goldstein, G.W, and Waxman, S.G., editors. Arbor Publishing Corp, La Jolla, CA, 1999 ed.
  12. Lentz, S.R. Hypercoagulable states and cerebrovascular disease. In: Neuroba se. Gilman, S., Goldstein, G.W, and Waxman, S.G., editors. Arbor Publishing Corp, La Jolla, CA, 1999 ed.
  13. Lentz, S.R. Hyperhomocysteinemia. In: Neuroba se. Gilman, S., Goldstein, G.W, and Waxman, S.G., editors. Arbor Publishing Corp, La Jolla, CA, 2000 ed.
  14. Lentz, S.R. Hypercoagulable states and cerebrovascular disease. In: Neuroba se. Gilman, S., Goldstein, G.W, and Waxman, S.G., editors. Arbor Publishing Corp, La Jolla, CA, 2000 ed.
  15. Shrader, M. and Lentz, S.R. Electronic communication at Arteriosclerosis, Thrombosis, and Vascular Biology. Arterioscler. Thromb. Vasc. Biol. (2000) 20:1851.
  16. Lentz, S.R. Homocysteine and cardiovascular physiology. In: Homocysteine in Health and Disease. Carmel, R. and Jacobsen, D.W., editors. R.G. Cambridge University Press, New York. (2001) 441-450.
  17. Lentz, S.R. Does homocysteine promote atherosclerosis? Arterioscler. Thromb. Vasc. Biol. (2001) 21:1385-1386.
  18. Raife, T.J., Rosenfeld, S.B., and Lentz, S.R. Bleeding from acquired coagulation defects and fibrinolytic therapy. In: Rossi's Principles of Transfusion Medicine, 3rd ed. Simon, T.L., Dzik, W.H., Snyder, E., Stowell, C.P., and Strauss, R.G., editors. R.G. Lippincott Williams & Wilkens, Philadelphia. (2002) 399-414
  19. Lentz, S.R. ATVB In Focus: a new series of focused brief reviews in Arteriosclerosis, Thrombosis, & Vascular Biology. Arterioscler. Thromb. Vasc. Biol. (2002) 22:1064.
  20. Hiatt, B.K and Lentz, S. R. Prothrombotic states that predispose to stroke. Current Treatment Options in Neurology (2002) 4:417-425.
  21. Lentz, S.R. Thrombosis of vein grafts: wall tension restrains thrombomodulin expression. Circ. Res. (2003) 92:12-13.
  22. Lentz, S.R. Homocysteine. In: McGraw-Hill Yearbook of Science & Technology . McGraw-Hill Companies, New York. (2003) 180-182.
  23. Lentz, S.R., Rodionov, R.N., and Dayal, S. Hyperhomocysteinemia, endothelial dysfunction, and cardiovascular risk: the potential role of ADMA. Atherosclerosis Suppl . (2003) 4: 61-65.
  24. Faraci, F.M. and Lentz, S.R. Hyperhomocysteinemia, oxidative stress, and cerebral vascular dysfunction. Stroke (2004) 35:345-347.
  25. Lentz, S.R. and Haynes, W.G. Homocysteine: Is it a clinically important cardiovascular risk factor? Cleveland Clinic J. Med . (2004) 71:729-734.
  26. Austin, R.C., Lentz, S.R., and Werstuck, G.H. Role of hyperhomocysteinemia in endothelial dysfunction and atherothrombotic disease. Cell Death and Differ. (2004) 11:S56-S64.
  27. Lentz, S.R., Devlin, A.M., and Dayal, S. Endothelial dysfunction & homocysteine: studies in genetic models of moderate hyperhomocysteinemia in mice. (2005) In: Homocysteine Metabolism. Symposia Proceedings, 4 th International Conference on Homocysteine Metabolism. Fowler, B. Ed. SPS Publications.
  28. Wilson, K.M. and Lentz, S.R. Mechanisms of the atherogenic effects of elevated homocysteine in experimental models. Sem. Vasc. Med. (2005) 5: 163-171.
  29. Dayal, S. and Lentz, S.R. ADMA and hyperhomocysteinemia. Vascular Medicine (2005) 10:S27-33.
  30. Jacobs, B.S. and Lentz, S.R. Hyperhomocysteinemia. In: Medlink Neurology . Gilman, S., editor. 2004 ed.
  31. Lentz, S.R. Another lesson from the factor V Leiden mouse: thrombin generation drives arterial disease. Circulation (2005) 111:1733-1734.
  32. Raife, T.J. and Lentz, S.R. The benefits of excess EPCR. J. Thromb. Haemost. (2005) 3:1349-1350.
  33. Lentz, S.R. Mechanisms of homocysteine-induced atherothrombosis. J. Thromb. Haemost. (2005) 3:1646-1654
  34. Lentz, S.R. Homocysteine. In: McGraw-Hill Encyclopedia of Science & Technology, 10 th Edition. (in press).
  35. Lentz, S.R. and Faraci, F.M. Circulatory System. In: Russo, A.F., Sigmund, C.D., et al. Human Physiology: Genomics to Systems. McGraw-Hill (in press).
  36. Devlin, A.M. and Lentz, S.R. Hyperhomocysteinemia. In: Clinical Studies in Medical Biochemistry, 3 rd Edition. Oxford University Press (in press).

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