|
|
 Previous Article | Table of Contents | Next Article 
Scott syndrome, characterized by impaired transmembrane migration of
procoagulant phosphatidylserine and hemorrhagic complications, is an
inherited disorder
F Toti, N Satta, E Fressinaud, D Meyer and JM Freyssinet
Institut d'Hematolgoie et Immunologie, Faculte de Medecine, Universite
Louis Pasteur, Strasbourg, France.
An as yet single family with a bleeding history is shown to present the
characteristic lack of membrane expression of procoagulant phospholipids
observed in Scott syndrome. Low prothrombin consumption in the serum of the
propositus, a 71-year-old woman, and two of her children was the sole
abnormal hemostasis parameter. The degree of exposure of procoagulant
phospholipids, chiefly phosphatidylserine, was reduced in stimulated
platelets, erythrocytes and Epstein-Barr virus- infected B lymphocytes. The
data are compatible with homozygous status of the propositus and
heterozygous status of her children. Scott syndrome appears to be
transmitted as an autosomal recessive trait reflecting the deletion or
mutation of a putative outward phosphatidylserine translocase. The detailed
knowledge of this transporter could have an impact in membrane physiology.
Volume 87,
Issue 4,
pp. 1409-1415,
02/15/1996
Copyright © 1996 by The American Society of Hematology
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
F. Toti and J.-M. Freyssinet
An ABC for Scott syndrome?
Blood,
July 15, 2005;
106(2):
396 - 397.
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. Albrecht, J. H. McVey, J. I. Elliott, A. Sardini, I. Kasza, A. D. Mumford, R. P. Naoumova, E. G. D. Tuddenham, K. Szabo, and C. F. Higgins
A novel missense mutation in ABCA1 results in altered protein trafficking and reduced phosphatidylserine translocation in a patient with Scott syndrome
Blood,
July 15, 2005;
106(2):
542 - 549.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. Hugel, M. C. Martinez, C. Kunzelmann, and J.-M. Freyssinet
Membrane Microparticles: Two Sides of the Coin
Physiology,
February 1, 2005;
20(1):
22 - 27.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
T. W. Kuijpers, N. A. Maianski, A. T. J. Tool, K. Becker, B. Plecko, F. Valianpour, R. J. A. Wanders, R. Pereira, J. Van Hove, A. J. Verhoeven, et al.
Neutrophils in Barth syndrome (BTHS) avidly bind annexin-V in the absence of apoptosis
Blood,
May 15, 2004;
103(10):
3915 - 3923.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. G. Mann and M. Kalafatis
Factor V: a combination of Dr Jekyll and Mr Hyde
Blood,
January 1, 2003;
101(1):
20 - 30.
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. B. Brooks, J. L. Catalfamo, H. A. Brown, P. Ivanova, and J. Lovaglio
A hereditary bleeding disorder of dogs caused by a lack of platelet procoagulant activity
Blood,
April 1, 2002;
99(7):
2434 - 2441.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. Presseizen, Z. Friedman, H. Shapiro, J. Radnay, and M. H. Ellis
Phosphatidylserine Expression on the Platelet Membrane of Patients with Myeloproliferative Disorders and its Effect on Platelet-Dependent Thrombin Formation
Clinical and Applied Thrombosis/Hemostasis,
January 1, 2002;
8(1):
33 - 39.
[Abstract]
[PDF]
|
|
|
|
|
|
|
N. O. Solum
Procoagulant Expression in Platelets and Defects Leading to Clinical Disorders
Arterioscler Thromb Vasc Biol,
December 1, 1999;
19(12):
2841 - 2846.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. Hugel, G. Socie, T. Vu, F. Toti, E. Gluckman, J.-M. Freyssinet, and M.-L. Scrobohaci
Elevated Levels of Circulating Procoagulant Microparticles in Patients With Paroxysmal Nocturnal Hemoglobinuria and Aplastic Anemia
Blood,
May 15, 1999;
93(10):
3451 - 3456.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
Q. Zhou, P. J. Sims, and T. Wiedmer
Expression of Proteins Controlling Transbilayer Movement of Plasma Membrane Phospholipids in the B Lymphocytes From a Patient With Scott Syndrome
Blood,
September 1, 1998;
92(5):
1707 - 1712.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. W.C. Dekkers, P. Comfurius, W. M.J. Vuist, J. T. Billheimer, I. Dicker, H. J. Weiss, R. F. A. Zwaal, and E. M. Bevers
Impaired Ca2+-Induced Tyrosine Phosphorylation and Defective Lipid Scrambling in Erythrocytes From a Patient With Scott Syndrome: A Study Using an Inhibitor for Scramblase That Mimics the Defect in Scott Syndrome
Blood,
March 15, 1998;
91(6):
2133 - 2138.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
E. Bevers, P. Comfurius, D. Dekkers, M. Harmsma, and R. Zwaal
Review : Regulatory mechanisms of transmembrane phospholipid distributions and pathophysiological implications of transbilayer lipid scrambling
Lupus,
January 1, 1998;
7(2_suppl):
S126 - S131.
[Abstract]
[PDF]
|
|
|
|
|
|
|
H. J. Weiss and B. Lages
Family Studies in Scott Syndrome
Blood,
July 1, 1997;
90(1):
475 - 476.
[Full Text]
[PDF]
|
|
|
|
|
|
|
H. J. Weiss and B. Lages
Platelet Prothrombinase Activity and Intracellular Calcium Responses in Patients With Storage Pool Deficiency, Glycoprotein IIb-IIIa Deficiency, or Impaired Platelet Coagulant Activity --- A Comparison With Scott Syndrome
Blood,
March 1, 1997;
89(5):
1599 - 1611.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. F.A. Zwaal and A. J. Schroit
Pathophysiologic Implications of Membrane Phospholipid Asymmetry in Blood Cells
Blood,
February 15, 1997;
89(4):
1121 - 1132.
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. Kunzelmann-Marche, J.-M. Freyssinet, and M. C. Martinez
Regulation of Phosphatidylserine Transbilayer Redistribution by Store-operated Ca2+ Entry. ROLE OF ACTIN CYTOSKELETON
J. Biol. Chem.,
February 9, 2001;
276(7):
5134 - 5139.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
