Scott syndrome, characterized by impaired transmembrane migration of procoagulant phosphatidylserine and hemorrhagic complications, is an inherited disorder

Blood

Volumn 87, Issue 4, 1996, Pages 1409-1415

  Toti, Florence ^b   Satta, Nathalie ^b   Fressinaud, Edith ^b   Meyer, Dominique ^b   Freyssinet, Jean Marie ^a  

^a UNIVERSITÉ DE STRASBOURG   (France)

^b NONE

Author keywords

[No Author keywords available]

Indexed keywords

PHOSPHATIDYLSERINE; PROCOAGULANT; PROTHROMBIN;

ADULT; AGED; ARTICLE; AUTOSOMAL RECESSIVE INHERITANCE; B LYMPHOCYTE; BLEEDING DISORDER; BLEEDING TENDENCY; CELL MEMBRANE TRANSPORT; CLINICAL ARTICLE; CONTROLLED STUDY; EPISTAXIS; FEMALE; GENE EXPRESSION; GENETIC DISORDER; HEMATOMA; HETEROZYGOSITY; HOMOZYGOSITY; HUMAN; HUMAN CELL; MALE; PRIORITY JOURNAL; PROTHROMBIN TIME; THROMBOCYTE ACTIVATION;

EID: 0030059544     PISSN: 00064971     EISSN: None     Source Type: Journal    
DOI: 10.1182/blood.v87.4.1409.bloodjournal8741409     Document Type: Article

References (30)

1. Furie B, Furie BC: Molecular and cellular biology of blood coagulation. N Engl J Med 326:800, 1992
2. Zwaal RFA, Comfurius P, Bevers EM: Mechanisms and function of changes in membrane-phospholipid asymmetry in platelets and erythrocytes. Biochem Soc Trans 21:248, 1993
3. Mann KG, Nesheim ME, Church WR, Haley P, Krishnaswamy S: Surface-dependent reactions of the vitamin K-dependent enzyme complexes. Blood 76:1, 1990
4. Devaux PF: Static and dynamic lipid asymmetry in cell membranes. Biochemistry 30:1163, 1991
5. Hoekstra D, Kok JW: Entry mechanisms of enveloped viruses. Implications for fusion of intracellular membranes. Biosci Rep 9:273, 1989
6. Yamada S, Ohnishi S: Vesicular stomatitis virus binds and fuses with phospholipid domain on target cell membrane. Biochemistry 25:3703, 1986
7. Thompson CB: Apoptosis in the pathogenesis and treatment of disease. Science 267:1456, 1995
8. Savill J, Fadok V, Henson P, Haslett C: Phagocyte recognition of cells undergoing apoptosis. Immunol Today 14:131, 1993
9. Koopman G, Reutelingsperger CPM, Kuijten GAM, Keehnen RMJ, Pals ST, van Oers MHJ: Annexin V for flow cytometric detection of phosphatidylserine expression on B cells undergoing apoptosis. Blood 84:1415, 1994
10. Allen TM, Williamson P, Schlegel R: Phosphatidylserine as a determinant of reticuloendothelial recognition of liposome models of the erythrocyte surface. Proc Natl Acad Sci USA 85:8067, 1988
11. Galli M, Bevers EM, Comfurius P, Barbui T, Zwaal RFA: Effect of antiphospholipid antibodies on procoagulant activity of activated platelets and platelet-derived microvesicles. Br J Haematol 83:466, 1993
12. Santoro SA: Antiphospholipid antibodies and thrombotic predisposition: Underlying pathogenic mechanisms Blood 83:2389, 1994
13. Weiss HJ, Vicic W, Lages B, Rogers J: Isolated deficiency of platelet procoagulant activity. Am J Med 67:206, 1979
14. Miletich JP, Kane WH, Hofmann SL, Stanford N, Majerus PW: Deficiency of factor Xa-factor Va binding sites on the platelets of a patient with a bleeding disorder. Blood 54:1015, 1979
15. Rosing J, Bevers EM, Comfurius P, Hemker HC, van Dieijen G, Weiss HJ, Zwaal RFA: Impaired factor X and prothrombin activation associated with decreased phospholipid exposure in platelets from a patient with a bleeding disorder. Blood 65:1557, 1985
16. Ahmad SS, Rawala-Sheikh R, Ashby B, Walsh PN: Platelet receptor-mediated factor X activation by factor IXa. High-affinity factor IXa receptors induced by factor VIII are deficient on platelets in Scott syndrome. J Clin Invest 84.824, 1989
17. Weiss HJ: Scott syndrome: A disorder of platelet procoagulant activity. Semin Hematol 31:312, 1994
18. Sims PJ, Wiedmer T, Esmon CT, Weiss HJ, Shattil SJ: Assembly of the prothrombinase complex is linked to the vesiculation of the platelet plasma membrane. Studies in Scott sydrome: An isolated defect in platelet procoagulant activity. J Biol Chem 264:17049, 1989
19. 2+-induced microvesiculation and deficient expression of procoagulant activity in erythrocytes of a patient with a bleeding disorder: A study of the red blood cells of Scott syndrome. Blood 79:380, 1992
20. Kojima H, Newton-Nash D, Weiss HJ, Zhao J, Sims PJ, Wiedmer T: Production and characterization of transformed B-lymphocytes expressing the membrane defect of Scott syndrome. J Clin Invest 94:2237, 1994
21. Dachary-Prigent J, Freyssinet JM, Pasquet JM, Carron JC, Nurden AT: Annexin V as a probe of aminophospholipid exposure and platelet membrane vesiculation: A flow cytometric study showing a role for free sulfhydryl groups. Blood 81:2554, 1993
22. Connor J, Bucana C, Fidler IJ, Schroit AJ: Differentiation-dependent expression of phosphatidylserine in mammalian plasma membranes: Quantitative assessment of outer-leaflet lipid in prothrombinase complex formation. Proc Natl Acad Sci USA 86:3184, 1989
23. Satta N, Toti F, Feugeas O, Bohbot A, Dachary-Prigent J, Eschwège V, Hedman H, Freyssinet JM: Monocyte vesiculation: A mechanism for dissemination of membrane-associated procoagulant activities and adhesion molecules following stimulation by lipopolysaccharide. J Immunol 153:3245, 1994
24. Freyssinet JM, Gauchy J, Cazenave JP: The effect of phospholipids on the activation of protein C by the human thrombiothrombomodulin complex. Biochem J 238:151, 1986
25. Thiagarajan P, Tait JF: Binding of annexin V/placental anticoagulant protein I to platelets. Evidence for phosphatidylserine exposure in the procoagulant response of activated platelets. J Biol Chem 265:17420, 1990
26. Ravanat C, Archipoff G, Beretz A, Freund G, Cazenave JP, Freyssinet JM: Use of annexin V to demonstrate the role of phosphatidylserine exposure in the maintenance of haemostatic balance by endothelial cells. Biochem J 282:7, 1992
27. Auland ME, Roufogalis BD, Devaux PF, Zachowski A: Reconstitution of ATP-dependent aminophospholipid translocation in proteoliposomes. Proc Natl Acad Sci USA 91:10938, 1994
28. Higgins CF: Flip-Flop: The transmembrane translocation of lipids. Cell 79:393, 1994
29. Smit JJM, Schinkel AH, Oude Elferink RPJ, Groen AK, Wagenaar E, van Deemter L, Mol CAAM, Ottenhoff R, van der Lugt NMT, van Roon, MA, van der Valk MA, Offerhaus GJA, Berns AJM, Borst P: Homozygous disruption of the murine mdr2 P-glycoprotein gene leads to a complete absence of phospholipid from bile and to liver disease. Cell 75:451, 1993
30. Hanada K, Pagano RE: A Chinese hamster ovary cell mutant defective in the non-endocytic uptake of fluorescent analogues of phosphatidylserine: Isolation using a cytosol acidification procedure. J Cell Biol 195:793, 1994